This document provides a summary of age dependent epileptic syndromes and focuses on primary generalized epilepsy and benign Rolandic epilepsy. It discusses the key characteristics of each condition including typical age of onset, common seizure types and EEG patterns. The summary concludes that these conditions are age determined and often seen in childhood, with seizures typically remitting by late adolescence.
This document summarizes different types of generalized epilepsies based on their EEG patterns and clinical presentations. It discusses primary generalized epilepsies, which have no identifiable cause and normal brain imaging. It also discusses more serious secondary generalized epilepsies, which are caused by diffuse brain injury and often associated with developmental delays. Specific syndromes like Lennox-Gastaut syndrome and West syndrome are examined in detail based on their characteristic EEG patterns including slow spike wave discharges, hypsarrhythmia, and independent multifocal spike discharges.
There are two main types of seizures - partial and generalized. Partial seizures originate in one area of the brain while generalized seizures affect the whole brain. Some specific childhood epilepsies discussed include benign Rolandic epilepsy, Rasmussen's syndrome, childhood absence epilepsy, myoclonic epilepsies, Lennox-Gastaut syndrome, West syndrome (infantile spasms), and Landau-Kleffner syndrome. Many childhood epilepsies have genetic components and vary in their symptoms, treatment response, and long-term prognosis.
This document summarizes infantile spasms (West syndrome), a rare and severe type of epilepsy that typically occurs in infancy. It describes the clinical features including characteristic spasms and EEG pattern called hypsarrhythmia. Etiologies can include brain malformations, tuberous sclerosis, perinatal insults, and cryptogenic cases. Optimal treatment aims to quickly control seizures to improve developmental outcomes, with vigabatrin found to be most effective but also carrying risks of visual field defects.
Abstract: Bening Rolandic Epilepsy 3 1.Abstract Benign rolandic epilepsy or Bening epilepsy of childhood with centro-temporal spikes (BECT) is the most widely recognized epilepsy disorder in the pediatric age group, with a beginning between age 3 and 13 years. The average introduction is a fractional seizure with parasthesias and tonic or clonic action of the lower face related with drooling and dysarthria. Seizures regularly happen around evening time and may turn out to be generalized. They are typically rare and may not require antiepileptic medicates in any case, whenever treated, they will in general be effectively controlled. Youngsters with BECT are neurologically and psychologically normal. The EEG shows trademark high-voltage sharp waves in the centro-temporal districts, which are enacted with sleepiness and rest. Right now, BECT is effectively perceived. Be that as it may, atypical cases are normal and the meaning of BECT can get obscured. Albeit further examinations are not required in cases with common clinical and EEG discoveries and typical neurologic assessments, neuro-imaging studies might be required in atypical cases to preclude other pathology. The long-term. medical and psychosocial forecast of BECT is magnificent, with basically all children entering long- term remission by mid-adolescene.
Neuropathology of epilepsy epilepsy related deaths and sudepAlejandro Palacio
This document discusses neuropathology findings related to epilepsy-related deaths and sudden unexpected death in epilepsy (SUDEP). It notes that pathologists will commonly encounter deaths in patients with epilepsy, including sudden deaths, and a systematic post-mortem examination is required. Macroscopic and microscopic examination of the brain can reveal underlying causes of epilepsy, effects of previous seizures, and potentially the cause of death. SUDEP remains underreported and the mechanisms are still unknown, but studies point to alterations in central autonomic regions involved in cardio-respiratory regulation. A thorough neuropathological investigation is essential to identify disease mechanisms in epilepsy-related deaths.
This document summarizes several chromosomal abnormalities that are associated with epilepsy syndromes, including specific clinical and EEG patterns. It describes 1p36 monosomy, Wolf-Hirschhorn syndrome, Angelman syndrome, Miller-Dieker syndrome, 18q- syndrome, and ring chromosome 20 syndrome as having characteristic epileptic presentations. It notes that Down syndrome has a lower incidence of epilepsy than other intellectual disability syndromes, with onset typically in early childhood or third decade of life. The document concludes that further research is needed to understand the mechanisms underlying epilepsy in chromosomal abnormalities.
This document provides an overview of the approach to evaluating and diagnosing hypotonia in infants. It discusses the distinction between central and peripheral causes of hypotonia based on clinical features. The key steps in evaluation include obtaining a detailed history, performing a thorough physical exam to assess tone and weakness, and selecting initial investigations like CK levels and brain imaging. The most common etiologies of central hypotonia are hypoxic-ischemic injury and intraventricular hemorrhage in preterms. Common peripheral causes are spinal muscular atrophy and congenital myopathies. The goal of diagnosis is to identify life-threatening conditions, provide prognostic information, and guide further testing and management.
This document summarizes different types of generalized epilepsies based on their EEG patterns and clinical presentations. It discusses primary generalized epilepsies, which have no identifiable cause and normal brain imaging. It also discusses more serious secondary generalized epilepsies, which are caused by diffuse brain injury and often associated with developmental delays. Specific syndromes like Lennox-Gastaut syndrome and West syndrome are examined in detail based on their characteristic EEG patterns including slow spike wave discharges, hypsarrhythmia, and independent multifocal spike discharges.
There are two main types of seizures - partial and generalized. Partial seizures originate in one area of the brain while generalized seizures affect the whole brain. Some specific childhood epilepsies discussed include benign Rolandic epilepsy, Rasmussen's syndrome, childhood absence epilepsy, myoclonic epilepsies, Lennox-Gastaut syndrome, West syndrome (infantile spasms), and Landau-Kleffner syndrome. Many childhood epilepsies have genetic components and vary in their symptoms, treatment response, and long-term prognosis.
This document summarizes infantile spasms (West syndrome), a rare and severe type of epilepsy that typically occurs in infancy. It describes the clinical features including characteristic spasms and EEG pattern called hypsarrhythmia. Etiologies can include brain malformations, tuberous sclerosis, perinatal insults, and cryptogenic cases. Optimal treatment aims to quickly control seizures to improve developmental outcomes, with vigabatrin found to be most effective but also carrying risks of visual field defects.
Abstract: Bening Rolandic Epilepsy 3 1.Abstract Benign rolandic epilepsy or Bening epilepsy of childhood with centro-temporal spikes (BECT) is the most widely recognized epilepsy disorder in the pediatric age group, with a beginning between age 3 and 13 years. The average introduction is a fractional seizure with parasthesias and tonic or clonic action of the lower face related with drooling and dysarthria. Seizures regularly happen around evening time and may turn out to be generalized. They are typically rare and may not require antiepileptic medicates in any case, whenever treated, they will in general be effectively controlled. Youngsters with BECT are neurologically and psychologically normal. The EEG shows trademark high-voltage sharp waves in the centro-temporal districts, which are enacted with sleepiness and rest. Right now, BECT is effectively perceived. Be that as it may, atypical cases are normal and the meaning of BECT can get obscured. Albeit further examinations are not required in cases with common clinical and EEG discoveries and typical neurologic assessments, neuro-imaging studies might be required in atypical cases to preclude other pathology. The long-term. medical and psychosocial forecast of BECT is magnificent, with basically all children entering long- term remission by mid-adolescene.
Neuropathology of epilepsy epilepsy related deaths and sudepAlejandro Palacio
This document discusses neuropathology findings related to epilepsy-related deaths and sudden unexpected death in epilepsy (SUDEP). It notes that pathologists will commonly encounter deaths in patients with epilepsy, including sudden deaths, and a systematic post-mortem examination is required. Macroscopic and microscopic examination of the brain can reveal underlying causes of epilepsy, effects of previous seizures, and potentially the cause of death. SUDEP remains underreported and the mechanisms are still unknown, but studies point to alterations in central autonomic regions involved in cardio-respiratory regulation. A thorough neuropathological investigation is essential to identify disease mechanisms in epilepsy-related deaths.
This document summarizes several chromosomal abnormalities that are associated with epilepsy syndromes, including specific clinical and EEG patterns. It describes 1p36 monosomy, Wolf-Hirschhorn syndrome, Angelman syndrome, Miller-Dieker syndrome, 18q- syndrome, and ring chromosome 20 syndrome as having characteristic epileptic presentations. It notes that Down syndrome has a lower incidence of epilepsy than other intellectual disability syndromes, with onset typically in early childhood or third decade of life. The document concludes that further research is needed to understand the mechanisms underlying epilepsy in chromosomal abnormalities.
This document provides an overview of the approach to evaluating and diagnosing hypotonia in infants. It discusses the distinction between central and peripheral causes of hypotonia based on clinical features. The key steps in evaluation include obtaining a detailed history, performing a thorough physical exam to assess tone and weakness, and selecting initial investigations like CK levels and brain imaging. The most common etiologies of central hypotonia are hypoxic-ischemic injury and intraventricular hemorrhage in preterms. Common peripheral causes are spinal muscular atrophy and congenital myopathies. The goal of diagnosis is to identify life-threatening conditions, provide prognostic information, and guide further testing and management.
coma is a life threatening condition, if early diagnosis and prompt treatment is there we can prevent patient from comatoes stages and the complication. if patient is in coma there is a less chances to recover.
This document discusses neural tube defects, which result from failure of the neural tube to close during early development. It describes the classification, diagnosis, investigations, management, and prevention of various neural tube defects including anencephaly, encephalocele, spina bifida, meningocele, and myelomeningocele. Prenatal screening and folic acid supplementation are important for prevention, while management requires a multidisciplinary approach to address physical, developmental, and cognitive issues.
The document summarizes a seminar on spinal cord syndromes presented by Teka Degefa. It discusses the anatomy of the spinal cord and outlines common spinal cord syndromes including complete spinal cord transection, ventral cord syndrome, Brown-Séquard syndrome, central spinal cord lesions, posterior and lateral column diseases, anterior horn cell syndromes, combined anterior horn cell and pyramidal tract disease, conus medullaris syndrome, and cauda equina syndrome. Objectives of the seminar were to discuss spinal cord anatomy, differentiate common syndromes, and learn how to evaluate patients with spinal lesions.
Coma and brain death are discussed. Coma is defined as an unresponsive state where only brainstem reflexes remain. Causes of coma include metabolic disturbances, structural lesions, and brainstem lesions. Examination of comatose patients involves assessing motor responses, breathing patterns, pupil size/reactivity, and reflexive eye movements. Brain death is the irreversible loss of brain and brainstem function, inevitably leading to organ failure. Diagnosing brain death requires determining the cause is sufficiently severe, no improvement after treating reversible causes, and absence of brainstem reflexes over an observation period.
Learn about coma/lethergy/stupor/lockdown syndrome
Unconscious.
In psychiatry, it is always difficult to distinguish the different reduce level of conscious states from catatonia.
This presentation shows more light about coma and how we differentiate it from other forms
This document provides an overview of epilepsy and seizures, including:
1) Epilepsy is defined as a brain disorder characterized by recurrent, unprovoked seizures. Seizures are caused by abnormal neuronal activity in the brain.
2) There are many potential causes of seizures, including genetic factors, head trauma, tumors, drug or alcohol withdrawal, and other medical conditions.
3) Epilepsy affects people of all ages, with the lifetime risk of having a seizure being around 9% and the lifetime risk of an epilepsy diagnosis being around 3%.
Spina bifida is a birth defect where the spinal column does not fully close around the spinal cord. It occurs when the neural tube fails to close properly during early embryonic development. The main types are spina bifida occulta (mildest), meningocele (meninges protrude through opening), and myelomeningocele (most severe where spinal cord and membranes protrude). Symptoms range from minor skin marks to paralysis depending on location and severity of the defect. Treatment involves surgery to cover the exposed tissues and may include shunts to drain excess cerebrospinal fluid. Lifelong management focuses on rehabilitation, prevention of infections and complications, and addressing mobility, bladder, and bowel issues.
This document provides an overview of epilepsy, including its epidemiology, classification, causes, pathophysiology, investigations, management, and social considerations. Some key points:
- Epilepsy affects approximately 5% of children and less than 1% have epilepsy. The incidence is highest in pre-school years. Causes include genetic factors, injuries, infections, tumors, and other structural brain abnormalities.
- Seizures are classified based on their origin in the brain as generalized or localized/partial. Generalized seizures involve both sides of the brain while partial seizures originate in one area.
- Management involves drug therapy with anti-epileptic medications as first-line treatment, as well as lifestyle modifications and
Consciousness, ras and approach to comaNeurologyKota
This document provides information on examining patients presenting with coma. It defines consciousness and the reticular activating system. It describes the examination of a comatose patient, including assessing respiratory pattern, pupils, ocular motility, motor response, and differentiating structural from toxic-metabolic causes of coma. It also discusses signs of brain herniation seen in comatose patients.
How epilepsy effects the brain in parietal lobemisner
The document discusses parietal lobe epilepsy, a rare type of epilepsy affecting around 5% of patients. It can be caused by head trauma, birth difficulties, stroke, or tumors. Seizures originating in the parietal lobe can cause sensory and visual sensations like numbness, tingling, heat, or pain that may march from the face down the arm and leg. The parietal lobe is involved with touch, language, and mathematical skills.
This document provides information on assessing and managing patients with altered consciousness such as coma. It defines key terms like coma, delirium, and vegetative state. It describes how to perform a neurological examination to evaluate a patient's level of consciousness and determine if deficits are focal or diffuse. The examination should assess motor response, brainstem reflexes, respiratory pattern, and reflexes. Admission to the ICU is recommended for patients with a Glasgow Coma Scale of 8 or less or a deteriorating level of consciousness.
This document discusses various causes of coma and their localization based on clinical signs. It describes akinetic mutism, which can result from bilateral frontal or diencephalomesencephalic lesions, and hyperkinetic mutism caused by subcortical and cortical infarcts. It also discusses the vegetative state, locked-in syndrome, and how lesions of the ascending reticular activating system or specific brain regions can cause altered levels of consciousness. It provides details on localizing lesions based on respiratory patterns, temperature changes, pupillary signs, and ocular motility.
The document discusses coma, including its definition, causes, clinical assessment, investigations, differential diagnosis and management. Coma is characterized by a total lack of arousal and awareness lasting at least 1 hour. It can be caused by structural brain injuries or metabolic derangements and represents a severe impairment of cerebral function. A systematic clinical approach is needed to identify treatable causes of coma such as head injuries, infections, drugs or toxic exposures.
Third nerve palsy is a condition that leads to impairment of motor function as the third cranial nerve innervates most eye muscles. It can be congenital or acquired through conditions like diabetes, hypertension, tumors or trauma. Acquired third nerve palsy often involves patients over 45 and can cause ptosis and pupil involvement while congenital palsy is usually unilateral and incomplete without ptosis. The document discusses a case study of a 18-year old female referred for assessment of right eye exotropia secondary to trauma as a child. Her examination findings and diagnosis of right eye exotropia are presented along with a management plan of unilateral eye muscle surgery.
Alteration of consciousness can result from diminished alertness due to widespread brain abnormalities or reduced activity of the reticular activating system. Confusion is characterized by impaired attention/concentration and disorientation, while delirium involves additional symptoms like agitation, hallucinations, and convulsions. Levels of consciousness range from alert to comatose. Confusion can be caused by medical/surgical diseases, infections, drugs, or nervous system disorders and is evaluated through history, exam focusing on attentiveness/orientation, and controlling underlying illnesses. The Glasgow Coma Scale assesses eye, motor, and verbal responses to determine coma depth.
This document provides guidance on evaluating patients presenting with coma. It outlines the importance of first addressing acute respiratory and cardiovascular issues before performing a full neurological exam. A thorough history and physical exam can provide clues to the cause of coma, including checking vital signs, looking for signs of head injury, and assessing neurological responses. Key reflexes like pupillary light response and oculocephalic maneuvers help localize brain injuries. Laboratory tests and imaging studies like CT, MRI, and EEG can help diagnose structural lesions or metabolic derangements causing the coma.
The document describes the anatomy and causes of various cranial nerve palsies. It discusses the third, fourth, sixth, and trochlear nerves. For each nerve, it outlines the nuclear location, anatomical course, common causes of palsy for adults and children, associated signs and symptoms, and important diagnostic considerations. Evaluation may include medical history, examination of eye movements and pupil function, and neuroimaging in certain cases to identify potentially compressive lesions.
This document discusses the anatomy and function of the oculomotor nerve (cranial nerve 3). It describes the nucleus and course of the nerve, causes of lesions, clinical features of total oculomotor nerve palsy, treatment options which may include surgery or monitoring, and differential diagnoses. History, examination, and investigations are outlined to evaluate patients presenting with oculomotor nerve palsies.
Coma and related disorders of consciousnessraj kumar
The document discusses various states of reduced consciousness including coma, stupor, drowsiness, vegetative state, akinetic mutism, and locked-in syndrome. It describes the signs, symptoms, typical causes and anatomical basis for each state. Coma is characterized by unarousability while stupor allows arousal with strong stimulation. Vegetative state involves awake but unresponsive patients. The locked-in syndrome allows eye movement but no other voluntary movement. The document outlines how lesions in different areas of the brainstem and thalamus underlie each state.
Topic of the month.... The role of gamma knife in the management of benign br...Professor Yasser Metwally
This document discusses the use of radiation therapy to treat benign intracranial tumors. It provides an overview of the different radiation therapy strategies including stereotactic radiosurgery (SRS), stereotactic radiotherapy (SRT), and fractionated radiation therapy. It describes the key factors in determining which strategy to use such as tumor location, needed dose, and tolerance of surrounding tissues. The document also reviews different radiation sources and delivery methods including 3D conformal radiation therapy and intensity-modulated radiation therapy (IMRT).
This document summarizes a case report published by Professor Yasser Metwally in January 2010. It describes a 50-year-old male patient who presented with limited horizontal eye movement for 4 years. Imaging showed a posterior pontine glioma. The patient declined treatment and later presented with worsening symptoms. MRI revealed a diffuse brain stem glioma involving the pons, midbrain and crus cerebri. The patient died 2 days after hospitalization. The case report is accompanied by 5 figures illustrating the imaging findings.
coma is a life threatening condition, if early diagnosis and prompt treatment is there we can prevent patient from comatoes stages and the complication. if patient is in coma there is a less chances to recover.
This document discusses neural tube defects, which result from failure of the neural tube to close during early development. It describes the classification, diagnosis, investigations, management, and prevention of various neural tube defects including anencephaly, encephalocele, spina bifida, meningocele, and myelomeningocele. Prenatal screening and folic acid supplementation are important for prevention, while management requires a multidisciplinary approach to address physical, developmental, and cognitive issues.
The document summarizes a seminar on spinal cord syndromes presented by Teka Degefa. It discusses the anatomy of the spinal cord and outlines common spinal cord syndromes including complete spinal cord transection, ventral cord syndrome, Brown-Séquard syndrome, central spinal cord lesions, posterior and lateral column diseases, anterior horn cell syndromes, combined anterior horn cell and pyramidal tract disease, conus medullaris syndrome, and cauda equina syndrome. Objectives of the seminar were to discuss spinal cord anatomy, differentiate common syndromes, and learn how to evaluate patients with spinal lesions.
Coma and brain death are discussed. Coma is defined as an unresponsive state where only brainstem reflexes remain. Causes of coma include metabolic disturbances, structural lesions, and brainstem lesions. Examination of comatose patients involves assessing motor responses, breathing patterns, pupil size/reactivity, and reflexive eye movements. Brain death is the irreversible loss of brain and brainstem function, inevitably leading to organ failure. Diagnosing brain death requires determining the cause is sufficiently severe, no improvement after treating reversible causes, and absence of brainstem reflexes over an observation period.
Learn about coma/lethergy/stupor/lockdown syndrome
Unconscious.
In psychiatry, it is always difficult to distinguish the different reduce level of conscious states from catatonia.
This presentation shows more light about coma and how we differentiate it from other forms
This document provides an overview of epilepsy and seizures, including:
1) Epilepsy is defined as a brain disorder characterized by recurrent, unprovoked seizures. Seizures are caused by abnormal neuronal activity in the brain.
2) There are many potential causes of seizures, including genetic factors, head trauma, tumors, drug or alcohol withdrawal, and other medical conditions.
3) Epilepsy affects people of all ages, with the lifetime risk of having a seizure being around 9% and the lifetime risk of an epilepsy diagnosis being around 3%.
Spina bifida is a birth defect where the spinal column does not fully close around the spinal cord. It occurs when the neural tube fails to close properly during early embryonic development. The main types are spina bifida occulta (mildest), meningocele (meninges protrude through opening), and myelomeningocele (most severe where spinal cord and membranes protrude). Symptoms range from minor skin marks to paralysis depending on location and severity of the defect. Treatment involves surgery to cover the exposed tissues and may include shunts to drain excess cerebrospinal fluid. Lifelong management focuses on rehabilitation, prevention of infections and complications, and addressing mobility, bladder, and bowel issues.
This document provides an overview of epilepsy, including its epidemiology, classification, causes, pathophysiology, investigations, management, and social considerations. Some key points:
- Epilepsy affects approximately 5% of children and less than 1% have epilepsy. The incidence is highest in pre-school years. Causes include genetic factors, injuries, infections, tumors, and other structural brain abnormalities.
- Seizures are classified based on their origin in the brain as generalized or localized/partial. Generalized seizures involve both sides of the brain while partial seizures originate in one area.
- Management involves drug therapy with anti-epileptic medications as first-line treatment, as well as lifestyle modifications and
Consciousness, ras and approach to comaNeurologyKota
This document provides information on examining patients presenting with coma. It defines consciousness and the reticular activating system. It describes the examination of a comatose patient, including assessing respiratory pattern, pupils, ocular motility, motor response, and differentiating structural from toxic-metabolic causes of coma. It also discusses signs of brain herniation seen in comatose patients.
How epilepsy effects the brain in parietal lobemisner
The document discusses parietal lobe epilepsy, a rare type of epilepsy affecting around 5% of patients. It can be caused by head trauma, birth difficulties, stroke, or tumors. Seizures originating in the parietal lobe can cause sensory and visual sensations like numbness, tingling, heat, or pain that may march from the face down the arm and leg. The parietal lobe is involved with touch, language, and mathematical skills.
This document provides information on assessing and managing patients with altered consciousness such as coma. It defines key terms like coma, delirium, and vegetative state. It describes how to perform a neurological examination to evaluate a patient's level of consciousness and determine if deficits are focal or diffuse. The examination should assess motor response, brainstem reflexes, respiratory pattern, and reflexes. Admission to the ICU is recommended for patients with a Glasgow Coma Scale of 8 or less or a deteriorating level of consciousness.
This document discusses various causes of coma and their localization based on clinical signs. It describes akinetic mutism, which can result from bilateral frontal or diencephalomesencephalic lesions, and hyperkinetic mutism caused by subcortical and cortical infarcts. It also discusses the vegetative state, locked-in syndrome, and how lesions of the ascending reticular activating system or specific brain regions can cause altered levels of consciousness. It provides details on localizing lesions based on respiratory patterns, temperature changes, pupillary signs, and ocular motility.
The document discusses coma, including its definition, causes, clinical assessment, investigations, differential diagnosis and management. Coma is characterized by a total lack of arousal and awareness lasting at least 1 hour. It can be caused by structural brain injuries or metabolic derangements and represents a severe impairment of cerebral function. A systematic clinical approach is needed to identify treatable causes of coma such as head injuries, infections, drugs or toxic exposures.
Third nerve palsy is a condition that leads to impairment of motor function as the third cranial nerve innervates most eye muscles. It can be congenital or acquired through conditions like diabetes, hypertension, tumors or trauma. Acquired third nerve palsy often involves patients over 45 and can cause ptosis and pupil involvement while congenital palsy is usually unilateral and incomplete without ptosis. The document discusses a case study of a 18-year old female referred for assessment of right eye exotropia secondary to trauma as a child. Her examination findings and diagnosis of right eye exotropia are presented along with a management plan of unilateral eye muscle surgery.
Alteration of consciousness can result from diminished alertness due to widespread brain abnormalities or reduced activity of the reticular activating system. Confusion is characterized by impaired attention/concentration and disorientation, while delirium involves additional symptoms like agitation, hallucinations, and convulsions. Levels of consciousness range from alert to comatose. Confusion can be caused by medical/surgical diseases, infections, drugs, or nervous system disorders and is evaluated through history, exam focusing on attentiveness/orientation, and controlling underlying illnesses. The Glasgow Coma Scale assesses eye, motor, and verbal responses to determine coma depth.
This document provides guidance on evaluating patients presenting with coma. It outlines the importance of first addressing acute respiratory and cardiovascular issues before performing a full neurological exam. A thorough history and physical exam can provide clues to the cause of coma, including checking vital signs, looking for signs of head injury, and assessing neurological responses. Key reflexes like pupillary light response and oculocephalic maneuvers help localize brain injuries. Laboratory tests and imaging studies like CT, MRI, and EEG can help diagnose structural lesions or metabolic derangements causing the coma.
The document describes the anatomy and causes of various cranial nerve palsies. It discusses the third, fourth, sixth, and trochlear nerves. For each nerve, it outlines the nuclear location, anatomical course, common causes of palsy for adults and children, associated signs and symptoms, and important diagnostic considerations. Evaluation may include medical history, examination of eye movements and pupil function, and neuroimaging in certain cases to identify potentially compressive lesions.
This document discusses the anatomy and function of the oculomotor nerve (cranial nerve 3). It describes the nucleus and course of the nerve, causes of lesions, clinical features of total oculomotor nerve palsy, treatment options which may include surgery or monitoring, and differential diagnoses. History, examination, and investigations are outlined to evaluate patients presenting with oculomotor nerve palsies.
Coma and related disorders of consciousnessraj kumar
The document discusses various states of reduced consciousness including coma, stupor, drowsiness, vegetative state, akinetic mutism, and locked-in syndrome. It describes the signs, symptoms, typical causes and anatomical basis for each state. Coma is characterized by unarousability while stupor allows arousal with strong stimulation. Vegetative state involves awake but unresponsive patients. The locked-in syndrome allows eye movement but no other voluntary movement. The document outlines how lesions in different areas of the brainstem and thalamus underlie each state.
Topic of the month.... The role of gamma knife in the management of benign br...Professor Yasser Metwally
This document discusses the use of radiation therapy to treat benign intracranial tumors. It provides an overview of the different radiation therapy strategies including stereotactic radiosurgery (SRS), stereotactic radiotherapy (SRT), and fractionated radiation therapy. It describes the key factors in determining which strategy to use such as tumor location, needed dose, and tolerance of surrounding tissues. The document also reviews different radiation sources and delivery methods including 3D conformal radiation therapy and intensity-modulated radiation therapy (IMRT).
This document summarizes a case report published by Professor Yasser Metwally in January 2010. It describes a 50-year-old male patient who presented with limited horizontal eye movement for 4 years. Imaging showed a posterior pontine glioma. The patient declined treatment and later presented with worsening symptoms. MRI revealed a diffuse brain stem glioma involving the pons, midbrain and crus cerebri. The patient died 2 days after hospitalization. The case report is accompanied by 5 figures illustrating the imaging findings.
This document summarizes a case study of a 60-year-old male patient who presented with rapidly progressive left-sided hemiplegia and increased intracranial pressure. CT scans showed diffuse abnormalities in the left thalamus, internal capsule, and basal ganglia, as well as an enhanced lesion in the left thalamus. The diagnosis was determined to be a thalamic glioma. The author's website provides regularly updated case studies and references for further information.
A 22-year-old male presented with increased intracranial pressure, meningeal irritation signs, and bilateral papilledema. MRI images showed enhancement of the leptomeninges and pachymeninges, ventricular dilation, and periventricular lesions. He was diagnosed with lymphomatous meningitis affecting both the leptomeninges and pachymeninges.
This document discusses sodium channel dysfunction and various related disorders. It begins by describing the physiology and pharmacology of voltage-gated sodium channels. It then discusses several sodium channelopathies involving muscle channels, cardiac channels, and brain channels. Specific channelopathies covered in more detail include generalized epilepsy with febrile seizures plus, severe myoclonic epilepsy of infancy (Dravet syndrome), and familial hemiplegic migraine. The document examines the genetic basis and functional properties of disease-causing mutations in the SCN1A gene associated with these conditions. Key findings include that GEFS+ mutations tend to cause gain of function through increased persistent sodium current, while SMEI mutations generally cause loss of function through
Cavernous carotid aneurysms are the most common type of extradural intracranial aneurysm. They are usually congenital and saccular in shape, arising from branches of the internal carotid artery within the cavernous sinus. About a quarter of cases are bilateral. Rupture can cause a carotid-cavernous fistula. Large unruptured aneurysms can compress nearby structures through mass effect as they expand upward and laterally within confined spaces of the skull. Cranial nerves are frequently compressed, which can impair eye movements or vision. On angiography, a thrombosed aneurysm may be difficult to detect due to surrounding tissue opacification.
The document discusses the benefits of exercise for mental health. Regular physical activity can help reduce anxiety and depression and improve mood and cognitive function. Exercise causes chemical changes in the brain that may help protect against mental illness and improve symptoms for those who already suffer from conditions like anxiety and depression.
The document discusses the benefits of exercise for mental health. Regular physical activity can help reduce anxiety and depression and improve mood and cognitive functioning. Exercise causes chemical changes in the brain that may help protect against developing mental illness and improve symptoms for those who already have a condition.
Research section. Cervical spondylitic myelopathy, Clinico-radiological appro...Professor Yasser Metwally
Research section. Cervical spondylitic myelopathy, Clinico-radiological approach: Correlation with the Hemorheological parameters and vascular risk factors
http://yassermetwally.com
http://yassermetwally.net
This patient presented with transverse myelitis involving the cervical spinal cord, with a history of previous transverse myelitis in the dorsal region and left optic neuritis. MRI showed longitudinally extensive lesions involving the cervical and dorsal spinal cord and left optic nerve enlargement. This clinical and radiological picture is consistent with neuromyelitis optica (NMO), also known as Devic's disease, an inflammatory disorder that predominantly involves the optic nerves and spinal cord. NMO can be difficult to distinguish from multiple sclerosis but tends to cause more severe episodes of optic neuritis and transverse myelitis.
This document summarizes recent studies on schizophrenia spectrum disorders (SSDs) in people with intellectual disabilities. It finds that:
1) The prevalence of SSDs in intellectual disabilities is estimated to be at least 3%, though accurate rates are difficult to determine.
2) Presentation of SSDs differs in those with intellectual disabilities, who often experience more severe symptoms and functional impairment.
3) Assessment of SSDs in intellectual disabilities remains challenging and current tools have limited sensitivity.
4) Further research is still needed, particularly randomized controlled trials of treatments and interventions for SSDs in intellectual disabilities.
The document discusses different types of headaches including primary and secondary headaches. Primary headaches include tension headaches, migraines, and cluster headaches where the headache is the main issue. Secondary headaches have an underlying cause like infection, trauma, or vascular issues. Treatment depends on the type with acute treatments like NSAIDs, triptans, or ergot derivatives for attacks and preventative medications for frequent headaches.
Issues in brainmapping...The role of EEG in epileptic syndromes associated wi...Professor Yasser Metwally
1. EEG is essential for evaluating epilepsy and can provide information about background activity, epileptiform discharges, and help diagnose specific epilepsy syndromes.
2. The document discusses various epilepsy syndromes like symptomatic, cryptogenic, and idiopathic epilepsy and how EEG characteristics help differentiate these.
3. Specific syndromes discussed include infantile spasms and West syndrome, characterized by hypsarrhythmia on EEG, and Lennox-Gastaut syndrome, characterized by slow spike and wave activity on EEG.
Epilepsy is a chronic neurological disorder characterized by recurrent seizures. Around 50 million people worldwide have epilepsy, with nearly 90% living in developing regions. Epilepsy can often be controlled with anti-epileptic drug treatment, though many in developing areas lack access to treatment. Seizures have various clinical manifestations depending on their location and spread in the brain. Diagnosis is based on the characteristics of seizures, though tests like EEG and MRI can provide additional information. Treatment involves lifestyle management of seizures and long-term use of anti-epileptic drugs to control seizures, with slow titration and withdrawal of drugs to avoid adverse effects.
This document summarizes key aspects of EEG interpretation and quantification. It discusses the clinical significance of sharp activity in EEGs and how it can indicate epilepsy. It also notes the limitations of visual EEG interpretation and efforts to standardize interpretation through structured methods. Finally, it introduces digital signal processing and pattern recognition techniques that aim to objectively quantify EEG signals.
Dravet syndrome is a rare and severe form of epilepsy that begins in infancy. It is characterized by frequent febrile seizures in the first year of life followed by other types of seizures and developmental delays. Genetic testing reveals mutations in the SCN1A gene in many patients. Treatment involves medications like valproate and benzodiazepines as well as a ketogenic diet, but seizures often remain difficult to control. The prognosis includes permanent neurological and cognitive impairments.
The document provides guidelines for the management of childhood epilepsy. It discusses the classification of seizures and epilepsy syndromes. Some key seizure types include partial seizures, generalized seizures like absence seizures, myoclonic seizures, atonic seizures, and tonic-clonic seizures. It also discusses approaches to diagnosing and treating common idiopathic or genetic epilepsy syndromes in children like benign childhood epilepsy with centrotemporal spikes and childhood absence epilepsy. Treatment involves antiepileptic drugs and considering neuroimaging in some cases.
EEG in idiopathic generalised epilepsiesNeurologyKota
This document provides information on idiopathic generalized epilepsies (IGE), which are a group of epilepsy disorders characterized by generalized bilateral spike-wave complexes on EEG. It describes the main seizure types seen in IGE including absence seizures, myoclonic seizures, and generalized tonic-clonic seizures. It also discusses several specific IGE syndromes like childhood absence epilepsy, juvenile absence epilepsy, and juvenile myoclonic epilepsy. For each syndrome it provides details on clinical presentation, EEG features, treatment approaches, and prognosis.
It contains description and salient points to diagnose various epileptic encephalopathies seen during infancy such as early myoclonic encephalopathies, Otahara syndrome, Dravet syndrome, West syndrome.
Presentation with extensive details of neonatal seizure. Covering its etiology, diagnosis and treatment . Neonatal seizure is one of the commonest clinical situation faced by any one working in a neonatal unit. Furthermore it is a favourite topic of many examiners in MD/DCH/DNB Pediatrics exams.
This document discusses neonatal seizures, including their incidence, causes, clinical presentation, investigations, and management. Some key points include:
- Seizures affect 0.1% of term infants and up to 10% of very low birth weight infants. Common causes include hypoxic ischemic encephalopathy, infection, hemorrhage, and congenital malformations.
- Presentation depends on etiology but may include oculo-facial, central/autonomic, clonic, tonic, or myoclonic seizures. Investigations include bloodwork, imaging, EEG, and metabolic screening.
- Management involves treating any underlying cause, starting anticonvulsants if seizures are prolonged or frequent, and
Epilepsy in children is common clinical problem encountered in practice both by general practitioners and paediatricians. so knowledge about different types of epilepsies is mandatory in diagnosis the disease early and instituting appropriate treatment
Dr. Shamanthakamani Narendran provides an overview of epilepsy, including its definition, classification, causes, diagnosis, treatment, and management. Epilepsy is a chronic neurological condition characterized by recurrent seizures and affects approximately 50 million people worldwide. It is usually controlled through medication, though not cured. The causes can be genetic, due to injury or illness, or idiopathic. Treatment involves medication to prevent or reduce seizures, and in some cases surgery may be an option.
This document provides an overview of epilepsy including:
- Epilepsy is a chronic neurological condition characterized by recurrent seizures and affects around 50 million people worldwide.
- Seizures have various classifications based on factors like location in the brain, observable manifestations, underlying medical conditions, and triggers.
- Epilepsy is typically diagnosed and managed through medication but may also involve lifestyle changes, surgery, dietary therapies, or vagus nerve stimulation in some cases.
- The causes can be genetic, due to brain injury or infection, and in some cases the cause is unknown. Proper response in an emergency involves preventing injury and calling for help for prolonged seizures.
The document discusses various epileptic syndromes categorized by age of onset - neonatal, infancy, childhood, adolescence-adult. For each syndrome, it provides information on defining features, age of onset, seizure types, EEG patterns, treatment and prognosis. The syndromes discussed include benign familial neonatal epilepsy, Ohtahara syndrome, West syndrome, Panayiotopoulos syndrome, Lennox-Gastaut syndrome, juvenile myoclonic epilepsy and others.
This document discusses epilepsy, including:
- Defining epilepsy as involuntary muscle contractions and relaxations due to brain disturbances.
- Childhood epilepsy is common, with causes including birth defects, infections, injuries and genetic factors.
- Seizures are classified based on location in the brain and symptoms. Diagnostic tests include EEG and MRI.
- Treatment involves anticonvulsant drugs to control seizures. Surgery may help for uncontrolled epilepsy localized to one brain region. Ketogenic diets and vagus nerve stimulators are also used. Nursing focuses on safety during seizures and proper medication administration.
Epilepsy is a chronic neurological condition characterized by recurrent, unprovoked seizures. Seizures occur due to excessive firing of neurons in the brain. Epilepsy has many potential causes including genetic factors, brain injury, infections, tumors, or developmental problems. Evaluation involves an EEG and neuroimaging to classify seizure type and identify underlying causes. Treatment focuses on medication or other options like the ketogenic diet, vagus nerve stimulation, or epilepsy surgery to control seizures. Children with uncontrolled epilepsy may experience social or academic challenges at school.
This document discusses EEG patterns in neonates. It describes the four typical EEG patterns seen in full-term neonates related to wakefulness and sleep cycles. It also summarizes EEG maturation in preterm neonates and discusses abnormal EEG patterns including severe abnormalities that indicate poor prognosis and mild abnormalities. The document outlines different ictal EEG patterns associated with neonatal seizures and technical aspects important for neonatal EEG recording.
- Many motor phenomena in neonates are non-epileptic, including tremors, jitteriness, myoclonus, and hyperekplexia. These are often misdiagnosed as seizures.
- Tremors and jitteriness are very common in neonates and are usually benign, caused by immature spinal inhibitory interneurons or elevated catecholamines. Fine tremors especially are typically benign.
- Myoclonus can also occur, such as benign neonatal sleep myoclonus. Hyperekplexia involves an exaggerated startle response and muscle rigidity.
- It is important to differentiate epileptic from non-epileptic events to
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Radiological pathology of cortical laminar necrosis
Issues in brainmapping...Age dependant epileptic syndromes
1. Version 21 A Monthly Publication presented by Professor Yasser Metwally May 2010
AGE DEPENDENT EPILEPTIC SYNDROMES [PART 3]
The age of the patients has great influence on the ictal, clinical and EEG characteristics of the epileptic seizures disorders and it also determines
the course and prognosis of epilepsy in general. This is particularly true in the first two decades of life, especially for infancy and early childhood.
There are certain age- determined epileptological entities or epileptic conditions which appear to be monolithic, in spite of a wide variety of
etiologies and probably also despite variations in the localization of cerebral involvement. The role of age in epileptic seizure disorder has been
substantiated by experimental work and clinical electroencephalographic investigations. We find in age-determined epileptic conditions a) certain
condition-related types of seizures, b) certain condition-related EEG patterns, and c) certain condition-related characteristics of course and
prognosis.
In the past, this aspect of epileptic seizure disorder had been neglected; too much emphasis was universally placed on locus and cause of the
seizure. From the historical view- point, the description of infantile spasm and the discovery of a specific EEG pattern called hypsarrhythmia
mark the first individualization of an age-determined polyetiological epileptic condition with certain clinical-electroencephalographic criteria.
There is a long historical evolution of the very common condition known as febrile convulsions. the clinical picture of benign Rolandic epilepsy
became clear due to the efforts of numerous authors. Neonatal convulsions, which are a particularly heterogeneous group, must also be listed in
this context and the thought-provoking notion of primary generalized epilepsy also belongs in this category.
PRIMARY GENERALIZED EPILEPSY
Introduction
The entire concept of a primary generalized epilepsy has its foundation in the EEG. Who would have thought that a simple petit mal absence
with its rather subtle clinical symptomatology could be the result of massive generalized synchronous epileptic discharges? The first observation
of the ictal EEG pattern of petit mal has been the starting point for numerous attempts to explain the phenomenon of primary generalized
seizure discharges.
2. Terminology
The term "primary generalized epilepsy" has been introduced by the International League against Epilepsy. This term deserves general
acceptance. It implies that the clinical and electroencephalographic phenomena of the seizures occurring in this epileptic condition are
generalized from the start. This term has superseded older terms such as "centrencephalic epilepsy", "cortico-reticular epilepsy", and "common
generalized epilepsy". Its weakness lies in the fact that it seems to burn all bridges for a retreat if a truly focal cortical onset with extremely
rapid generalization should ever be convincingly demonstrated in the future.
Age and Prevalence
The age depends on the type of seizure. Classical petit mal absences mostly start at age 4 to 6 yr; a special group starts at age 9 to 15 yr.
Myoclonus and grand mal attacks usually start at age 11 to 14 yr. Improvement or full seizure control after age 20-25 yr is very common. A
special manifestation is the petit mal absence status, which may occur in older children, adolescents, adults, and even the elderly.
Primary generalized epilepsy is sometimes preceded by a period of febrile convulsions in infancy and early childhood. It is never preceded by
severe conditions such as infantile spasms or the Lennox-Gastaut syndrome.
Ictal Manifestations
The petit mal absence was discussed in detail in the section dealing with types of seizures. The two different forms are 1) simple petit mal
absences, starting at age 4 or shortly thereafter, with a large number of absences/day (sometimes exceeding 100/day); and 2) "juvenile" petit
mal absences with an onset at age 9 up to 15 yr, more prolonged or mixed with more motor activity.
Petit mal absences show a wide variety of mild to moderate motor accompaniment; rhythmical eye blinking in synchrony with the spike waves
is the most common motor component. Retropulsion of the head is quite common ("retropulsive petit mal); adversive movements and some
rhythmically repetitive oral motions may also occur.
Children with petit mal absences often start having grand mal seizures in early adolescence. Figures range from 31 % to 54%.
In most of these cases, the grand mal seizures do not pose a major problem and are readily brought under control.
Grand mal attacks in patients with primary generalized epilepsy are very often preceded by sudden bilateral myoclonus. These myoclonic jerks
may also occur as isolated events, especially in the morning hours after a night of insufficient sleep. Many patients with this combination of
grand mal and myoclonus may never have experienced any petit mal absences earlier in childhood. This petit mal-free form is a special variant
of primary generalized epilepsy which also shows slightly different inter-ictal bursts in the EEG. These bursts are relatively short and
dominated by 4/sec or 4-5/sec spike wave
complexes which are, contrary to the
classical 3/sec or 3-4/sec spike waves, not
readily activated by hyperventilation. Many
of these patients are flicker-sensitive
(photoconvulsive response) and positive
family histories are more often obtained in
this group. Myoclonus may also be
associated with brief petit mal absences.
Very prolonged absence-like stages, attacks
of petit mal- like stupor, or petit mal
automatisms have been termed "Petit mal
status", whereas the modern terminology
recommends the term "absence status."
These states will be discussed in the section
on status epilepticus. While all of the ictal
manifestations of primary generalized
epilepsy tend to occur in children and
adolescents, the absence status not only
occurs in elderlies but may even have its
onset in old age. Figure 1. The 3 c/s spike/wave discharge with frontal dominance
EEG
Most of the relevant EEG findings have been described in detail in the chapter on abnormal paroxysmal EEG patterns. May it suffice to
reiterate that the 3/sec or 3-4 c/s spike wave pattern is the EEG correlate of the classical petit mal absence and also occurs, often abundantly, in
the interval, sometimes in drowsiness and sleep. These generalized bursts, with or without clinical absence, are readily triggered by
hyperventilation and may materialize after a few deep breaths in untreated patients. Intermittent photic stimulation may occasionally trigger
petit mal absences with 3/sec spike waves; more often, it is associated with generalized polyspikes of frontal accentuation, with or without
clinical myoclonic jerking, most often at frequencies of 14-18 flashes/sec. As was pointed out above, photosensitivity is more often noted in
patients with 4/sec or 4-5/sec spike wave bursts and a history of grand mal and/or myoclonus.
3. The phenomenon of myoclonus is almost invariably linked with polyspike discharges as far as patients with primary generalized epilepsy are
concerned. Polyspikes also contaminate the spike wave sequences in children with massive myoclonus as a variant of petit mal. In the majority of
patients with primary generalized epilepsy, the EEG frequency spectrum appears to be normal aside from the generalized paroxysmal bursts, the
so called background activity. A remarkable exception is the occurrence of prolonged stretches of rhythmical high voltage 3/sec waves in occipital
leads with moderate spread into the vicinity; these bursts occur in a significant number of children with petit mal absences. this rhythm is found in
55% of the patients with petit mal absences and persists in 60% of the cases following seizure control. Hyperventilation almost invariably
activates this rhythm. In some cases, a very small spike component is discernible between the large rhythmical delta waves. The rhythmical
posterior activity may be enhanced under treatment with ethosuximide (Zarontin), while the 3/sec spike wave complex disappears. Children with
petit mal absences and rhythmical occipital delta trains fall into a special epileptological category. The occipital rhythmical slow activity is a very
favorable prognostic sign.
The sleep records of patients with primary generalized epilepsy show frequent bursts of spikes, polyspikes, and spike waves; with common
association between the K complex and the frontal midline maximum of the spike discharges. This maximum over the frontal midline is almost
invariably present, not only in sleep but also in the waking state. This indicates that arousal plays an important role in the generation of these
discharges. REM sleep is associated with an attenuation or total suppression of bilateral synchronous paroxysmal bursts. In exceptional cases, the
maximum of the 3/sec spike wave bursts lies in the vertex region rather than in frontal midline; these children also show Rolandic spikes.
It goes without saying that genetic factors are particularly important in the field of primary generalized epilepsy. Generalized synchronous seizure
discharges follow an autosomal-dominant pattern of genetic transmission, with variable penetrance regardless of presence or absence of seizures
with an unusual characteristic of a very low penetrance at birth which rises to nearly complete penetrance (close to 50%) for age 4.5 to 16.5 years"
with a gradual decline to almost no penetrance at age 40 yr. These figures are in excellent agreement with the incidence of generalized
synchronous seizure discharges of the 3-4/sec spike-wave type as well as the clinical seizure manifestations of primary generalized epilepsy.
The Presence of Focal Spikes in Patients with Primary Generalized Epilepsy
A certain type of focal spikes in childhood ("benign Rolandic spikes") may be occasionally present in children who, suffer from petit mal
absences, especially after suppression of the absences and the spike waves with medication. This view would shed more light on the demonstration
of genetic factors in children with midtemporal spikes.
Concluding Remarks
The challenging problem of primary generalized epilepsy remains unsolved. Genetic predisposition and age (chiefly 4-20 yr) are significant
factors. It is a specifically human disorder; for this reason, animal models can provide only partial insight into pathogenetic mechanisms. In the
human, the generalized synchronous seizure discharge originates from the interhemispheric frontal portion bilaterally. Arousing stimuli play a
crucial role in the detonation of these discharges. In some patients, however, the mechanism of photosensitivity is paramount .
BENIGN ROLANDIC EPILEPSY
Introduction
In children with spikes over the central region and/or adjacent midtemporal and parietal areas, a benign and readily controllable type of epileptic
seizure disorder with focal motor seizures and/or grand mal is the rule. An increasing number of reports over the past 40 yr gives testimony to
growing awareness of this special form of childhood epilepsy.
This form of childhood epilepsy is occasionally listed among the primary generalized epilepsies despite its prominent focal features in the ictal
and electroencephalographic semiology. Such a classification certainly appears to be provocative. There are indeed certain relationships between
benign Rolandic epilepsy and primary generalized epilepsies and conversion from one form to the other may occur. There is certainly good
reason to separate benign Rolandic epilepsies from the bulk of focal (partial) epileptic seizure disorders which will be presented somewhat later.
Age and Prevalence
Benign Rolandic epilepsy occurs at age 3 to 12 yr. The majority of these children are in the range from 6 to 10 yr. Disappearance of the seizures
during adolescence (or even prior to puberty) is the rule. The seizures may occasionally recur much later in life, probably due to seizure-
facilitating factors such as severe illness or toxic-metabolic factors.
The sex distribution shows that boys are more often affected. The prevalence is not quite clear and might be somewhere between 5 and 10% in a
population of epileptics below age 15 yr.
Ictal Manifestations
The seizures, regardless of focal or grand mal character, tend to occur during nocturnal sleep, mostly during the last hour of sleep or in the first 2
hr. About 80% of the attacks occur in sleep and, of the remaining 20%, about 10% take place shortly after awakening. (Note the similarities with
primary generalized epilepsy.) Nocturnal seizures may awaken the child afterward. Preservation of consciousness and hence the ability to
4. describe the experienced seizure was found in 58%; this indicates the predominance of focal seizures. Grand mal (tonic-clonic) seizures were
noted in 26%.
The seizures are hardly ever seen by the physician, even by the epileptologist who sees sizeable numbers of these children. One therefore depends
heavily on descriptions by the patient Or his family; nocturnal video- tape or biotelemetry recordings are quite helpful.
Focal seizures often involve the face. The midtemporal spike localization has been thought to be related to paroxysmal activity in the very closely
located lower portion of the motor strip (facio-laryngo-pharyngeal muscles). Hemifacial twitching is definitely more common than clonic motions
in the contralateral arm; least common is clonic activity in the leg. In some cases, the entire half of the body participates, but a typical Jacksonian
march does not seem to occur. Speech arrest is quite common (39% of the seizures). This is apparently an ictal anarthria with preserved internal
speech.
Oropharyngeal involvement is very often reported, with sounds described as "guttural," "gargling," "throaty," "wheezing," or "as if going to
vomit." Feelings of suffocation are reported as coming from the mouth but not from the chest. These patients also have focal seizures which are
not Rolandic, with blindness, vertigo, and torsion of the body as ictal signs. This underscores the complexity of the underlying
neurophysiological mechanisms.
EEG
Spiking over central-midtemporal area in children is of limited
epileptogenicity. It is reasonable to presume that 50-70% of these
children have seizures and the remaining 50-30% are seizure-free. These
latter patients are referred to the EEG laboratory because of a variety of
symptoms such as behavior disorder, headaches, and other complaints or
derivations.
The spatial distribution of the spike activity requires an appropriate
number of electrodes; the International Electrode System is particularly
suitable. Otherwise, the Rolandic cortex may lie between a frontal and a
parietal electrode and strictly local spiking may escape detection,
especially when the midtemporal region is not explored ideally. In my
personal experience, a central maximum of spike discharge is slightly
more often noted than a midtemporal maximum.
The spikes themselves are large and may be either spikes in the strict
Figure 2. Trains of spikes/sharp waves recorded in a
sense or sharp waves (see chapter on abnormal paroxysmal patterns).
quasirhythmical pattern in a child with benign Rolandic epilepsy.
Spiking is usually enhanced in light nonREM sleep, during which the
discharges may become extremely abundant. Their random character may
give way to quasirhythmical or periodical spiking at intervals of less than
1 see; previously unilateral spikes become bilateral synchronous or asynchronous. In many children, Rolandic spikes are found in the sleep
portion only. REM sleep restores the unilateral character of the spiking. Bilateral parieto-occipital 4/sec spike wave-like discharges of moderate
voltage is occasionally seen in the waking patient.
The rest of the tracing is usually normal in these patients and the frequency spectrum corresponds to age. Central mu rhythm is sometimes
present and there is reason to presume that central spikes of childhood may be gradually replaced by mu rhythm, at least in some of the patients.
There is indubitable evidence that, in a limited number of patients, the central spike activity can be blocked by contralateral fist clenching or,
even better, by alternate clenching and opening of the fist. This provides further evidence for the functional character of the spikes.
In a small number of cases, the spike activity shows a consistent maximum over the vertex or over the centroparietal midline region, which may
be the sole region of spiking, so that omission of midline leads would result in missing the abnormality. Some of these patients show focal motor
or sensory ictal activity of leg predominance but, more often, the ictal symptoms do not correspond with the spike localization.
Clinical Signs of Nonictal Character
Neurological deficits are not compatible with benign Rolandic epilepsy. This condition is based on dysfunction rather than structural pathology.
It is worthwhile, however, to search carefully for a true intracranial lesion. Arteriovenous malformation occasionally may be the cause of the
discharges and associated seizure.
Central, midtemporal, or parietal spikes or spikes over the midline may also occur in children with evidence of cerebral palsy, in mostly diplegic,
quadriplegic, or choreoathetoid forms. In these children, Rolandic spikes have a different connotation and do not herald a good prognosis for the
seizure disorder. The reader will find more extensive discussion in the section on cerebral palsy.
Behavior disorders are very common in children with true Rolandic spikes; they may range from hyperkinetic behavior and signs of minimal
cerebral dysfunction to severe anxiety neurosis. Various types of headaches may occur. The intelligence is normal in true benign Rolandic
epilepsy.
5. Course
The seizures are easily controlled with routine anticonvulsive treatment. Treatment may even be withheld unless seizures repeat themselves.
Freedom from seizures in adolescence is the rule. The return of a single major convulsion may occasionally occur under the influence of
infections, stress, or toxic substances. These cases show no resurgence of the central spike focus, which renders the EEG diagnosis very
difficult. The presence of central mu rhythm may serve as a hint that the patient has had central spikes in the past, but such conclusions can be
made only with reservations.
Etiological Considerations
This form of epilepsy is due to dysfunction rather than pathology. A genetic basis is the most logical thought.
Problem of Differential Diagnosis
Benign Rolandic epilepsy must be differentiated from:
1. Children with Rolandic spikes and no seizures whatsoever (these children are certainly not epileptics; about 30 to 50% of the children with
Rolandic spikes have no overt clinical seizures).
2. Children with Rolandic spikes and a history of antecedent brain damage or cerebral palsy.
3. Children who have typical psychomotor seizures and evidence of temporal lobe epilepsy which may gradually progress in severity; these
children may have atypical spike localization (central, midtemporal), while the classical anterior temporal sharp wave focus does not
materialize before adolescence.
4. Children with midtemporal spikes, marked tendency to generalization and spike wave formation, clinically with aphasia, probably a
temporary inflammatory condition which gradually subsides.
5. Children with frequent focal motor seizures which become progressively worse: "malignant" Rolandic epilepsy of childhood.
6. Children with centroparietal spikes elicited by tactile stimulation of corresponding cutaneous areas of the body (see under benign parietal
epilepsy). The differentiation of these conditions rests on a careful combined clinical-electroencephalographic assessment of each case.
The EEG shows considerable slowing. There is evidence of constant muscle activity, but no authentic cerebral spikes are demonstrable.
Spike Foci Outside the Rolandic Region in Children.
Occipital spike foci are usually found between the ages of 2 to 5 yr. These children show no neurological or ophthalmological deficit;
about 40% of them have clinical seizures, mostly grand mal, with good prognosis.
Frontal spike foci in children are associated with epileptogenicity, with about 80% having overt seizures, and a guarded prognosis.
Multiple spike foci (two or more areas of independent spiking) are also highly epileptogenic; the prognosis is guarded and probably
fairly good if Rolandic spikes predominate.
Considerations of Basic Mechanisms
True benign Rolandic epilepsy is likely to be based on temporary paroxysmal hyperirritability of the motor cortex, which naturally has a lower
threshold of epileptic excitability. This is merely a working hypothesis in need of further substantiating evidence.
"Malignant" Rolandic Epilepsy
Cases of progressively worsening focal motor seizures and prolonged episodes of epilepsia partialis continua are quite rare but probably
constitute a special epileptological entity. Motor deficits and mental decline are associated with the seizure disorder. Their etiology is poorly
defined; chronic localized encephalitis may be one of the causes (Rasmussen syndrome). Hemispherectomy seems to be the only effective
treatment; limited cortical excisions or lobectomics are ineffective.
The EEG shows endless sequences of ictal spike discharges during focal motor attacks but becomes uninformative in states of epilepsia
partialis continua, which probably originate from deep structures or possibly from lamina V of the motor cortex without participation of the
superficial layers.
Benign Parietal Epilepsy
The vast majority of these patients were children between the ages of 4 and 8 yr.The occurrence of spikes over the parietal region (parasagittal
zone) following contralateral tactile stimuli characteristic of this condition. The paroxysmal response to tactile stimuli was found to be
enhanced in nonREM sleep. Only 20% of children had clinical seizures; the remaining children were referred because of behavior problems.
Children with Midtemporal Spikes, Progressive Aphasia and Seizures
This syndrome of childhood epilepsy with progressive aphasia has stimulated much interest over the past 40 yr and may be regarded as an