This document discusses alcohol related brain damage (ARBD), a spectrum of neurological and cognitive conditions caused by chronic alcoholism. It describes several specific conditions under the ARBD umbrella, including Wernicke's encephalopathy and Korsakoff's syndrome. Wernicke's encephalopathy is an acute neurological disorder caused by thiamine deficiency, with symptoms including eye movement abnormalities, gait issues, and altered consciousness. Korsakoff's syndrome is a chronic memory disorder that can develop after Wernicke's encephalopathy, characterized by anterograde amnesia and confabulation. The document outlines the molecular mechanisms, neuroimaging features, and neural substrates of these conditions.
Refractory seizures are seizures that do not respond to treatment with anti-epileptic drugs. They are most commonly seen in children and can be caused by unknown etiologies, genetic factors, or structural brain lesions. Evaluation of refractory seizures involves assessing for inborn errors of metabolism, infections, genetic syndromes, or other structural abnormalities through clinical exams, imaging, and metabolic testing of blood and urine. Intractable seizures are difficult to control and often associated with mesial temporal sclerosis, tuberous sclerosis, or other lesions.
Dr. john millichap kcnq2 Cure summit professional track learn more at kcnq2cu...scottyandjim
Dr. John Millichap speaking at 2014 Denver KCNQ2 Cure summit professionals track at Children's Hospital of Colorado. More information at www.kcnq2cure.org
This document discusses various epileptic encephalopathies in infants and children. It begins by defining epileptic encephalopathies as electro-clinical syndromes associated with a high probability of encephalopathic features that present or worsen after the onset of epilepsy. It then describes several specific neonatal and infantile epileptic syndromes in detail, including early myoclonic encephalopathy, Ohtahara syndrome, West syndrome, and malignant migrating partial epilepsy of infancy. It also discusses later childhood syndromes such as Landau-Kleffner syndrome and continuous spike-wave during slow-wave sleep syndrome. For each syndrome, it covers defining characteristics, etiology, investigations, treatment approaches, and prognosis
Portal-systemic encephalopathy is a brain disorder caused by liver dysfunction that allows toxins to reach the brain. It is characterized by alterations in mental status, neurological abnormalities, and distinctive EEG changes. The main underlying mechanism involves increased levels of ammonia in the bloodstream from the gut that are normally processed by the liver. Treatment focuses on reducing ammonia production in the colon through medications like lactulose and restricting protein intake. Prognosis depends on the underlying liver disease and can range from fully treatable acute episodes to chronic and potentially fatal cases.
This document discusses various types of autoimmune encephalitis. It begins by providing clues that can suggest an autoimmune cause over infectious, including a subacute onset and fluctuating course. It then covers several specific autoimmune encephalitis subtypes defined by the neuronal surface antigens involved, such as anti-NMDA receptor and anti-LGI1 encephalitis. For each subtype, it discusses clinical features, investigations, and treatment approaches. The document aims to help clinicians differentiate between autoimmune and infectious causes of encephalitis.
- Coma is a medical emergency in children that requires prompt evaluation and treatment to identify the underlying cause and prevent further neurological damage.
- The neurological examination provides important clues about potential causes and localization through assessment of factors like pupil response, brainstem reflexes, motor function, and presence of focal deficits or posturing.
- Initial diagnostic workup includes blood tests, imaging like CT scan or MRI, and lumbar puncture depending on clinical suspicion of conditions like infection, trauma, or metabolic derangement. Treatment focuses on stabilization, identifying and reversing any immediate life-threatening conditions, providing supportive care, and administering specific treatments targeted at suspected etiologies.
Derived from Greek word “enkephalos”- meaning brain.
“Pathos” meaning is disease.
The term “encephalopathy” is defined as altered mental status as a result of a diffuse disturbance of brain function.
This document provides an overview of encephalopathy, including:
- Encephalopathy is defined as an altered mental state caused by diffuse brain dysfunction. Common symptoms include confusion, memory loss, and personality changes.
- There are many potential causes of encephalopathy including metabolic disturbances, toxins, infections, liver failure, inflammation, drugs, demyelination, and lack of oxygen to the brain.
- EEG is often abnormal in encephalopathy, with features including triphasic waves and diffuse slowing correlating to severity of symptoms and impairment of consciousness.
Refractory seizures are seizures that do not respond to treatment with anti-epileptic drugs. They are most commonly seen in children and can be caused by unknown etiologies, genetic factors, or structural brain lesions. Evaluation of refractory seizures involves assessing for inborn errors of metabolism, infections, genetic syndromes, or other structural abnormalities through clinical exams, imaging, and metabolic testing of blood and urine. Intractable seizures are difficult to control and often associated with mesial temporal sclerosis, tuberous sclerosis, or other lesions.
Dr. john millichap kcnq2 Cure summit professional track learn more at kcnq2cu...scottyandjim
Dr. John Millichap speaking at 2014 Denver KCNQ2 Cure summit professionals track at Children's Hospital of Colorado. More information at www.kcnq2cure.org
This document discusses various epileptic encephalopathies in infants and children. It begins by defining epileptic encephalopathies as electro-clinical syndromes associated with a high probability of encephalopathic features that present or worsen after the onset of epilepsy. It then describes several specific neonatal and infantile epileptic syndromes in detail, including early myoclonic encephalopathy, Ohtahara syndrome, West syndrome, and malignant migrating partial epilepsy of infancy. It also discusses later childhood syndromes such as Landau-Kleffner syndrome and continuous spike-wave during slow-wave sleep syndrome. For each syndrome, it covers defining characteristics, etiology, investigations, treatment approaches, and prognosis
Portal-systemic encephalopathy is a brain disorder caused by liver dysfunction that allows toxins to reach the brain. It is characterized by alterations in mental status, neurological abnormalities, and distinctive EEG changes. The main underlying mechanism involves increased levels of ammonia in the bloodstream from the gut that are normally processed by the liver. Treatment focuses on reducing ammonia production in the colon through medications like lactulose and restricting protein intake. Prognosis depends on the underlying liver disease and can range from fully treatable acute episodes to chronic and potentially fatal cases.
This document discusses various types of autoimmune encephalitis. It begins by providing clues that can suggest an autoimmune cause over infectious, including a subacute onset and fluctuating course. It then covers several specific autoimmune encephalitis subtypes defined by the neuronal surface antigens involved, such as anti-NMDA receptor and anti-LGI1 encephalitis. For each subtype, it discusses clinical features, investigations, and treatment approaches. The document aims to help clinicians differentiate between autoimmune and infectious causes of encephalitis.
- Coma is a medical emergency in children that requires prompt evaluation and treatment to identify the underlying cause and prevent further neurological damage.
- The neurological examination provides important clues about potential causes and localization through assessment of factors like pupil response, brainstem reflexes, motor function, and presence of focal deficits or posturing.
- Initial diagnostic workup includes blood tests, imaging like CT scan or MRI, and lumbar puncture depending on clinical suspicion of conditions like infection, trauma, or metabolic derangement. Treatment focuses on stabilization, identifying and reversing any immediate life-threatening conditions, providing supportive care, and administering specific treatments targeted at suspected etiologies.
Derived from Greek word “enkephalos”- meaning brain.
“Pathos” meaning is disease.
The term “encephalopathy” is defined as altered mental status as a result of a diffuse disturbance of brain function.
This document provides an overview of encephalopathy, including:
- Encephalopathy is defined as an altered mental state caused by diffuse brain dysfunction. Common symptoms include confusion, memory loss, and personality changes.
- There are many potential causes of encephalopathy including metabolic disturbances, toxins, infections, liver failure, inflammation, drugs, demyelination, and lack of oxygen to the brain.
- EEG is often abnormal in encephalopathy, with features including triphasic waves and diffuse slowing correlating to severity of symptoms and impairment of consciousness.
LOCALISATION OF LESION CAUSING COMA.pptxNeurologyKota
1) The document discusses signs that can help localize lesions causing coma, including abnormalities in respiratory patterns, pupil size and response, eye movements, and corneal and limb reflexes.
2) Specific lesions like thalamic or brainstem hemorrhages can cause signs like wrong-way eyes or downward eye deviation.
3) Examining responses like the oculocephalic reflex or corneal reflex can help determine if the brainstem is intact and localize lesions.
1. The document discusses emerging drugs for the treatment of epilepsy, providing details on mechanisms of action, pharmacokinetics, clinical trials and adverse effects for several promising new anti-epileptic drugs.
2. These include brivaracetam, carisbamate, eslicarbazepine acetate, retigabine, perampanel, ganaxolone, and stiripentol. Drugs like brivaracetam and carisbamate are in Phase III trials as adjunctive therapies for partial onset seizures.
3. The newer drugs offer advantages over older anti-epileptics like fewer drug interactions and less toxicity profiles. They expand treatment options for
Approach to evaluation of a child with upper motor neuron disorderAleya Remtullah
This document provides an overview of upper motor neuron disorders (UMND) in children. It discusses various pathologies that can cause UMND including lesions in different areas of the brain and spinal cord. Specifically, it examines cortical lesions, basal ganglia disorders, brainstem lesions, spinal cord lesions, and cerebellar disorders. For each area, it provides details on specific lesions, signs and symptoms, investigations, and treatments. The document is intended to guide physicians in evaluating and diagnosing children presenting with possible UMND.
This document discusses neonatal seizures, beginning with an introduction stating they are not uncommon and often the first sign of neurological disorders. It then covers the pathophysiology, incidence, patterns, etiology, diagnosis, management, treatment including anticonvulsants, and prognosis of neonatal seizures over multiple pages with headings and subheadings. Key points include seizures occurring in 1 in 200 healthy newborns, various possible causes like hypoglycemia or infections, treatments involving anticonvulsants like phenobarbital or midazolam, and prognosis varying from normal outcome to neurological sequelae depending on factors like etiology and examination findings.
This document provides an overview of seizure disorders including basics, epidemiology, risk factors, pathophysiology, diagnosis, treatment, and prognosis. Some key points:
- Seizures are caused by excessive firing of neurons resulting in impaired brain function. Common causes include brain tumors, head injuries, infections, genetic factors.
- Around 200,000 new cases of epilepsy are diagnosed in the US each year, most commonly in children under 15 and older adults over 65.
- Diagnosis involves differentiating epileptic from non-epileptic seizures based on eyewitness accounts and EEG/MRI testing. Initial lab work checks for metabolic causes.
- Treatment primarily involves anti-epileptic medications chosen based
This document discusses seizures and epilepsy in children. It defines a seizure as abnormal excessive neuronal activity in the brain and epilepsy as two or more unprovoked seizures occurring more than 24 hours apart. Seizures can be focal, originating in one area of the brain, or generalized, involving both hemispheres simultaneously. Epilepsy syndromes are described. Imaging and EEG play roles in evaluating seizures, with MRI being preferred and EEG showing localized or generalized abnormalities.
This case summary describes a 4-year-old male child who presented with cough, cold, fever, and seizure followed by altered sensorium for 15 days prior to admission. He had been hospitalized for 15 days at a private hospital where he was on mechanical ventilation for 10 days and received a tracheostomy tube. Testing showed normal CBC, SE, CSF study, viral markers, CSF NMDA receptor study, and MRI. He received multiple antiepileptics and steroids with some improvement in condition but remained dependent on the tracheostomy tube. The summary discusses various types and causes of encephalitis including autoimmune encephalitis such as anti-NMDA receptor encephalitis, LGI1 encephalitis
Seizures and epilepsy are caused by abnormal neuronal activity in the brain due to an imbalance between excitation and inhibition. Seizures can be focal, originating in one area of the brain, or generalized, involving both sides of the brain. Focal seizures may or may not impair consciousness, while generalized seizures include tonic-clonic, absence, myoclonic, and atonic types. Investigations help determine the type and cause of seizures, while treatment involves lifestyle changes, anti-seizure medications, or surgery to reduce seizures. Status epilepticus is a medical emergency requiring rapid treatment to prevent complications.
This document discusses various treatable causes of dementia. Some key points:
- About 20% of dementia cases may have a reversible underlying condition such as depression, vitamin deficiencies, infections, or side effects from medications.
- Common potentially reversible forms include alcoholism, normal pressure hydrocephalus, metabolic disturbances like thyroid disorders or B12 deficiency.
- Identifying and treating the underlying reversible cause can help improve cognitive symptoms in some cases. Prompt treatment of conditions like Wernicke's encephalopathy, vitamin B12 deficiency, or Hashimoto's encephalopathy is especially important for minimizing neurological damage.
This document discusses seizures in neonates. It begins by defining seizures, convulsions, and epilepsy. It then notes that seizures are most common in neonates, occurring in 0.5-0.8% of term babies and 6-12% of babies under 1500g. Common causes of neonatal seizures include hypoxic ischemic encephalopathy, intracranial hemorrhage, infections, inborn errors of metabolism like hypoglycemia and hypocalcemia, and epilepsy syndromes. The immature neonatal brain is more excitable and prone to seizures due to reduced connectivity, an imbalance toward excitatory processes, and the excitatory effect of GABA in some regions. The document describes different seizure types and discusses
Wernicke encephalopathy is caused by thiamine (vitamin B1) deficiency. Thiamine plays a key role in brain energy metabolism. Lack of thiamine can damage brain tissue, especially in regions with high metabolic demand. Common causes include chronic alcoholism, prolonged starvation, pregnancy-related vomiting, and bariatric surgery. Symptoms include confusion, ataxic gait, and eye movement abnormalities. Diagnosis involves clinical assessment and testing for thiamine deficiency. Treatment involves high-dose parenteral thiamine supplementation, which can improve symptoms if administered early.
EEG FOR EPILEPSY AND ANTICONVULSIVANT DRUGS.pptxPANFRAGGER
Epilepsy is a neurological disorder characterized by recurrent seizures caused by abnormal electrical discharges in the brain. Seizures can be focal, arising from one area of the brain, or generalized, spreading across both hemispheres. Antiepileptic drugs work by enhancing inhibition or reducing excitation in the brain, such as by blocking sodium or calcium channels. Common antiepileptic drugs include phenytoin, carbamazepine, and valproic acid, but newer drugs such as levetiracetam have fewer side effects.
This document discusses epileptic encephalopathies, which are a group of heterogeneous brain disorders occurring during brain development where epileptiform activity contributes to cognitive and behavioral regression beyond the underlying pathology. It defines and classifies several early-onset epileptic encephalopathy syndromes according to age of onset such as Ohtahara syndrome, early myoclonic encephalopathy, West syndrome, Dravet syndrome, Lennox-Gastaut syndrome, and myoclonic-astatic epilepsy. It provides details on clinical presentation, EEG features, causes, and treatment approaches for each condition.
This document discusses renal and hepatic encephalopathy. It defines uremic encephalopathy as a brain disorder that occurs in patients with untreated or inadequately treated kidney disease. Symptoms range from mild to severe and fluctuate depending on kidney function. Uremic toxins that accumulate due to renal dysfunction are a primary cause. Treatment involves optimizing dialysis to adequately remove toxins. Other types of encephalopathy discussed include dialysis dementia, central pontine myelinolysis, seizures, and restless leg syndrome. Causes, clinical features, diagnosis and management are described for each condition.
This document discusses various inborn errors of metabolism that can cause epilepsy. It covers disorders of mitochondrial function, creatine metabolism, GLUT1 deficiency, hypoglycemia, storage disorders, disorders of the urea cycle and amino acid metabolism, organic acid disorders, purine/pyrimidine metabolism, disturbances to neurotransmitter systems, brain malformations, and some vitamin-responsive epilepsies like pyridoxine-dependent epilepsy and pyridox(am)ine phosphate oxygenase deficiency. Specific genetic disorders are discussed in each category along with their associated seizure types, diagnostic testing, treatment approaches, and prognosis.
This patient, a 65-year-old male, presented with 6 months of difficulty walking and 4 months of forgetfulness and hallucinations. Examination found he was semiconscious with rigidity and myoclonic jerks. Imaging and tests were consistent with a prion disease like Creutzfeldt-Jakob disease (CJD). A lumbar puncture found elevated proteins. He was diagnosed with a rapidly progressive neurodegenerative condition likely CJD.
Wernicke encephalopathy is caused by thiamine (vitamin B1) deficiency. Thiamine plays a key role in brain energy metabolism. Lack of thiamine can damage brain tissue, especially in regions with high metabolic demand. Common causes include chronic alcoholism, prolonged starvation, pregnancy-related vomiting, and bariatric surgery. Symptoms include confusion, ataxic gait, and eye movement abnormalities. Treatment involves high dose parenteral thiamine supplementation.
Polyglutamine (polyQ) diseases are a group of neurodegenerative disorders caused by expanded CAG repeats encoding long polyQ tracts in proteins. There are 9 known polyQ diseases including Huntington's disease, spinocerebellar ataxias, and spinal and bulbar muscular atrophy. They are characterized by protein misfolding, inclusion body formation, and neurodegeneration. While there is no cure, treatment focuses on managing symptoms and slowing progression. Gene silencing and stem cell therapies are under investigation but long-term drug treatment has been disappointing so far.
This document discusses progressive myoclonic epilepsy (PME), a group of rare genetic neurological disorders characterized by myoclonus and epileptic seizures with progressive neurological decline. It describes several specific forms of PME, including neuronal ceroid lipofuscinoses (NCLs), Lafora body disease, Unverricht-Lundborg disease, and myoclonic epilepsy with ragged-red fibers. For each, it covers clinical features, genetics, investigations such as EEG and MRI findings, pathology, treatment approaches, and prognosis. The document provides a detailed review and comparison of these progressive myoclonic epilepsy syndromes.
Neonatal-Seizures diagnosis and managementFelixBoamah3
This document discusses neonatal seizures. It begins by defining seizures and describing the different types seen in neonates. The most common cause is hypoxic ischemic encephalopathy. Other common causes include intraventricular hemorrhage and acute metabolic disorders. Phenobarbital is the first-line treatment, with phenytoin and benzodiazepines as subsequent options. Prognosis depends on the underlying etiology, with focal clonic seizures and those from subarachnoid hemorrhage or late hypocalcemia having better outcomes. Anti-seizure medications should be tapered slowly after seizure control is achieved.
Histololgy of Female Reproductive System.pptxAyeshaZaid1
Dive into an in-depth exploration of the histological structure of female reproductive system with this comprehensive lecture. Presented by Dr. Ayesha Irfan, Assistant Professor of Anatomy, this presentation covers the Gross anatomy and functional histology of the female reproductive organs. Ideal for students, educators, and anyone interested in medical science, this lecture provides clear explanations, detailed diagrams, and valuable insights into female reproductive system. Enhance your knowledge and understanding of this essential aspect of human biology.
LOCALISATION OF LESION CAUSING COMA.pptxNeurologyKota
1) The document discusses signs that can help localize lesions causing coma, including abnormalities in respiratory patterns, pupil size and response, eye movements, and corneal and limb reflexes.
2) Specific lesions like thalamic or brainstem hemorrhages can cause signs like wrong-way eyes or downward eye deviation.
3) Examining responses like the oculocephalic reflex or corneal reflex can help determine if the brainstem is intact and localize lesions.
1. The document discusses emerging drugs for the treatment of epilepsy, providing details on mechanisms of action, pharmacokinetics, clinical trials and adverse effects for several promising new anti-epileptic drugs.
2. These include brivaracetam, carisbamate, eslicarbazepine acetate, retigabine, perampanel, ganaxolone, and stiripentol. Drugs like brivaracetam and carisbamate are in Phase III trials as adjunctive therapies for partial onset seizures.
3. The newer drugs offer advantages over older anti-epileptics like fewer drug interactions and less toxicity profiles. They expand treatment options for
Approach to evaluation of a child with upper motor neuron disorderAleya Remtullah
This document provides an overview of upper motor neuron disorders (UMND) in children. It discusses various pathologies that can cause UMND including lesions in different areas of the brain and spinal cord. Specifically, it examines cortical lesions, basal ganglia disorders, brainstem lesions, spinal cord lesions, and cerebellar disorders. For each area, it provides details on specific lesions, signs and symptoms, investigations, and treatments. The document is intended to guide physicians in evaluating and diagnosing children presenting with possible UMND.
This document discusses neonatal seizures, beginning with an introduction stating they are not uncommon and often the first sign of neurological disorders. It then covers the pathophysiology, incidence, patterns, etiology, diagnosis, management, treatment including anticonvulsants, and prognosis of neonatal seizures over multiple pages with headings and subheadings. Key points include seizures occurring in 1 in 200 healthy newborns, various possible causes like hypoglycemia or infections, treatments involving anticonvulsants like phenobarbital or midazolam, and prognosis varying from normal outcome to neurological sequelae depending on factors like etiology and examination findings.
This document provides an overview of seizure disorders including basics, epidemiology, risk factors, pathophysiology, diagnosis, treatment, and prognosis. Some key points:
- Seizures are caused by excessive firing of neurons resulting in impaired brain function. Common causes include brain tumors, head injuries, infections, genetic factors.
- Around 200,000 new cases of epilepsy are diagnosed in the US each year, most commonly in children under 15 and older adults over 65.
- Diagnosis involves differentiating epileptic from non-epileptic seizures based on eyewitness accounts and EEG/MRI testing. Initial lab work checks for metabolic causes.
- Treatment primarily involves anti-epileptic medications chosen based
This document discusses seizures and epilepsy in children. It defines a seizure as abnormal excessive neuronal activity in the brain and epilepsy as two or more unprovoked seizures occurring more than 24 hours apart. Seizures can be focal, originating in one area of the brain, or generalized, involving both hemispheres simultaneously. Epilepsy syndromes are described. Imaging and EEG play roles in evaluating seizures, with MRI being preferred and EEG showing localized or generalized abnormalities.
This case summary describes a 4-year-old male child who presented with cough, cold, fever, and seizure followed by altered sensorium for 15 days prior to admission. He had been hospitalized for 15 days at a private hospital where he was on mechanical ventilation for 10 days and received a tracheostomy tube. Testing showed normal CBC, SE, CSF study, viral markers, CSF NMDA receptor study, and MRI. He received multiple antiepileptics and steroids with some improvement in condition but remained dependent on the tracheostomy tube. The summary discusses various types and causes of encephalitis including autoimmune encephalitis such as anti-NMDA receptor encephalitis, LGI1 encephalitis
Seizures and epilepsy are caused by abnormal neuronal activity in the brain due to an imbalance between excitation and inhibition. Seizures can be focal, originating in one area of the brain, or generalized, involving both sides of the brain. Focal seizures may or may not impair consciousness, while generalized seizures include tonic-clonic, absence, myoclonic, and atonic types. Investigations help determine the type and cause of seizures, while treatment involves lifestyle changes, anti-seizure medications, or surgery to reduce seizures. Status epilepticus is a medical emergency requiring rapid treatment to prevent complications.
This document discusses various treatable causes of dementia. Some key points:
- About 20% of dementia cases may have a reversible underlying condition such as depression, vitamin deficiencies, infections, or side effects from medications.
- Common potentially reversible forms include alcoholism, normal pressure hydrocephalus, metabolic disturbances like thyroid disorders or B12 deficiency.
- Identifying and treating the underlying reversible cause can help improve cognitive symptoms in some cases. Prompt treatment of conditions like Wernicke's encephalopathy, vitamin B12 deficiency, or Hashimoto's encephalopathy is especially important for minimizing neurological damage.
This document discusses seizures in neonates. It begins by defining seizures, convulsions, and epilepsy. It then notes that seizures are most common in neonates, occurring in 0.5-0.8% of term babies and 6-12% of babies under 1500g. Common causes of neonatal seizures include hypoxic ischemic encephalopathy, intracranial hemorrhage, infections, inborn errors of metabolism like hypoglycemia and hypocalcemia, and epilepsy syndromes. The immature neonatal brain is more excitable and prone to seizures due to reduced connectivity, an imbalance toward excitatory processes, and the excitatory effect of GABA in some regions. The document describes different seizure types and discusses
Wernicke encephalopathy is caused by thiamine (vitamin B1) deficiency. Thiamine plays a key role in brain energy metabolism. Lack of thiamine can damage brain tissue, especially in regions with high metabolic demand. Common causes include chronic alcoholism, prolonged starvation, pregnancy-related vomiting, and bariatric surgery. Symptoms include confusion, ataxic gait, and eye movement abnormalities. Diagnosis involves clinical assessment and testing for thiamine deficiency. Treatment involves high-dose parenteral thiamine supplementation, which can improve symptoms if administered early.
EEG FOR EPILEPSY AND ANTICONVULSIVANT DRUGS.pptxPANFRAGGER
Epilepsy is a neurological disorder characterized by recurrent seizures caused by abnormal electrical discharges in the brain. Seizures can be focal, arising from one area of the brain, or generalized, spreading across both hemispheres. Antiepileptic drugs work by enhancing inhibition or reducing excitation in the brain, such as by blocking sodium or calcium channels. Common antiepileptic drugs include phenytoin, carbamazepine, and valproic acid, but newer drugs such as levetiracetam have fewer side effects.
This document discusses epileptic encephalopathies, which are a group of heterogeneous brain disorders occurring during brain development where epileptiform activity contributes to cognitive and behavioral regression beyond the underlying pathology. It defines and classifies several early-onset epileptic encephalopathy syndromes according to age of onset such as Ohtahara syndrome, early myoclonic encephalopathy, West syndrome, Dravet syndrome, Lennox-Gastaut syndrome, and myoclonic-astatic epilepsy. It provides details on clinical presentation, EEG features, causes, and treatment approaches for each condition.
This document discusses renal and hepatic encephalopathy. It defines uremic encephalopathy as a brain disorder that occurs in patients with untreated or inadequately treated kidney disease. Symptoms range from mild to severe and fluctuate depending on kidney function. Uremic toxins that accumulate due to renal dysfunction are a primary cause. Treatment involves optimizing dialysis to adequately remove toxins. Other types of encephalopathy discussed include dialysis dementia, central pontine myelinolysis, seizures, and restless leg syndrome. Causes, clinical features, diagnosis and management are described for each condition.
This document discusses various inborn errors of metabolism that can cause epilepsy. It covers disorders of mitochondrial function, creatine metabolism, GLUT1 deficiency, hypoglycemia, storage disorders, disorders of the urea cycle and amino acid metabolism, organic acid disorders, purine/pyrimidine metabolism, disturbances to neurotransmitter systems, brain malformations, and some vitamin-responsive epilepsies like pyridoxine-dependent epilepsy and pyridox(am)ine phosphate oxygenase deficiency. Specific genetic disorders are discussed in each category along with their associated seizure types, diagnostic testing, treatment approaches, and prognosis.
This patient, a 65-year-old male, presented with 6 months of difficulty walking and 4 months of forgetfulness and hallucinations. Examination found he was semiconscious with rigidity and myoclonic jerks. Imaging and tests were consistent with a prion disease like Creutzfeldt-Jakob disease (CJD). A lumbar puncture found elevated proteins. He was diagnosed with a rapidly progressive neurodegenerative condition likely CJD.
Wernicke encephalopathy is caused by thiamine (vitamin B1) deficiency. Thiamine plays a key role in brain energy metabolism. Lack of thiamine can damage brain tissue, especially in regions with high metabolic demand. Common causes include chronic alcoholism, prolonged starvation, pregnancy-related vomiting, and bariatric surgery. Symptoms include confusion, ataxic gait, and eye movement abnormalities. Treatment involves high dose parenteral thiamine supplementation.
Polyglutamine (polyQ) diseases are a group of neurodegenerative disorders caused by expanded CAG repeats encoding long polyQ tracts in proteins. There are 9 known polyQ diseases including Huntington's disease, spinocerebellar ataxias, and spinal and bulbar muscular atrophy. They are characterized by protein misfolding, inclusion body formation, and neurodegeneration. While there is no cure, treatment focuses on managing symptoms and slowing progression. Gene silencing and stem cell therapies are under investigation but long-term drug treatment has been disappointing so far.
This document discusses progressive myoclonic epilepsy (PME), a group of rare genetic neurological disorders characterized by myoclonus and epileptic seizures with progressive neurological decline. It describes several specific forms of PME, including neuronal ceroid lipofuscinoses (NCLs), Lafora body disease, Unverricht-Lundborg disease, and myoclonic epilepsy with ragged-red fibers. For each, it covers clinical features, genetics, investigations such as EEG and MRI findings, pathology, treatment approaches, and prognosis. The document provides a detailed review and comparison of these progressive myoclonic epilepsy syndromes.
Neonatal-Seizures diagnosis and managementFelixBoamah3
This document discusses neonatal seizures. It begins by defining seizures and describing the different types seen in neonates. The most common cause is hypoxic ischemic encephalopathy. Other common causes include intraventricular hemorrhage and acute metabolic disorders. Phenobarbital is the first-line treatment, with phenytoin and benzodiazepines as subsequent options. Prognosis depends on the underlying etiology, with focal clonic seizures and those from subarachnoid hemorrhage or late hypocalcemia having better outcomes. Anti-seizure medications should be tapered slowly after seizure control is achieved.
Histololgy of Female Reproductive System.pptxAyeshaZaid1
Dive into an in-depth exploration of the histological structure of female reproductive system with this comprehensive lecture. Presented by Dr. Ayesha Irfan, Assistant Professor of Anatomy, this presentation covers the Gross anatomy and functional histology of the female reproductive organs. Ideal for students, educators, and anyone interested in medical science, this lecture provides clear explanations, detailed diagrams, and valuable insights into female reproductive system. Enhance your knowledge and understanding of this essential aspect of human biology.
Cell Therapy Expansion and Challenges in Autoimmune DiseaseHealth Advances
There is increasing confidence that cell therapies will soon play a role in the treatment of autoimmune disorders, but the extent of this impact remains to be seen. Early readouts on autologous CAR-Ts in lupus are encouraging, but manufacturing and cost limitations are likely to restrict access to highly refractory patients. Allogeneic CAR-Ts have the potential to broaden access to earlier lines of treatment due to their inherent cost benefits, however they will need to demonstrate comparable or improved efficacy to established modalities.
In addition to infrastructure and capacity constraints, CAR-Ts face a very different risk-benefit dynamic in autoimmune compared to oncology, highlighting the need for tolerable therapies with low adverse event risk. CAR-NK and Treg-based therapies are also being developed in certain autoimmune disorders and may demonstrate favorable safety profiles. Several novel non-cell therapies such as bispecific antibodies, nanobodies, and RNAi drugs, may also offer future alternative competitive solutions with variable value propositions.
Widespread adoption of cell therapies will not only require strong efficacy and safety data, but also adapted pricing and access strategies. At oncology-based price points, CAR-Ts are unlikely to achieve broad market access in autoimmune disorders, with eligible patient populations that are potentially orders of magnitude greater than the number of currently addressable cancer patients. Developers have made strides towards reducing cell therapy COGS while improving manufacturing efficiency, but payors will inevitably restrict access until more sustainable pricing is achieved.
Despite these headwinds, industry leaders and investors remain confident that cell therapies are poised to address significant unmet need in patients suffering from autoimmune disorders. However, the extent of this impact on the treatment landscape remains to be seen, as the industry rapidly approaches an inflection point.
Does Over-Masturbation Contribute to Chronic Prostatitis.pptxwalterHu5
In some case, your chronic prostatitis may be related to over-masturbation. Generally, natural medicine Diuretic and Anti-inflammatory Pill can help mee get a cure.
- Video recording of this lecture in English language: https://youtu.be/kqbnxVAZs-0
- Video recording of this lecture in Arabic language: https://youtu.be/SINlygW1Mpc
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
These lecture slides, by Dr Sidra Arshad, offer a quick overview of the physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar lead (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
6. Describe the flow of current around the heart during the cardiac cycle
7. Discuss the placement and polarity of the leads of electrocardiograph
8. Describe the normal electrocardiograms recorded from the limb leads and explain the physiological basis of the different records that are obtained
9. Define mean electrical vector (axis) of the heart and give the normal range
10. Define the mean QRS vector
11. Describe the axes of leads (hexagonal reference system)
12. Comprehend the vectorial analysis of the normal ECG
13. Determine the mean electrical axis of the ventricular QRS and appreciate the mean axis deviation
14. Explain the concepts of current of injury, J point, and their significance
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. Chapter 3, Cardiology Explained, https://www.ncbi.nlm.nih.gov/books/NBK2214/
7. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
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2. ARBD
• Alcohol related brain damage (ARBD) is a term used to cover a
spectrum of conditions and disorders induced by chronic
alcoholism which leads to a constellation of neurological,
cognitive, behavioural and neuropsychiatric symptoms.
• These disease entities merge into each other, clinically overlap
and combine synergistically to contribute to the phenomena of
neurological and cognitive dysfunction resulting in alcohol
related brain damage.
• The clinical features and neuroradiologic (functional
neuroimaging) distinctive features help in differentiating these
entities.
6. BRAIN REWARD CIRCUITRY
• Brain Reward Circuitry : network of four circuits involved in drug
abuse and addiction
• Reward circuit _Nucleus Accumbens DA-regulated reward circuit
• Motivation circuit _Orbitofrontal cortex, Subcallosal cortex
• Memory circuit _Amygdala (Conditioned-learned associations )
and Hippocampus (memory of expected reward)
• Cognitive Control circuit _Prefrontal cortex and Cingulate gyrus
• OFC-Amygdala-NA reward circuit
Without the inhibitory frontal cognitive control, a positive-
feedback loop is set forth that result in compulsive drug intake.
The prefrontal cortex is involved in self-monitoring, decision-
making processes, and in inhibitory control. The disruption of the
prefrontal cortex leads to loss of self-directed/willed behaviour in
favour of automatic sensory-driven behaviour (high reward
dependence).
10. LAB MARKERS-THIAMINE STATUS
• Red Blood Cell Thiamine Pyrophosphate (RBCTPP)
• Red Blood Cell Transketolase (RBCTTK)
• Blood Pyruvate
• GGTP (gamma-glutamyl transferase)
• ALT/AST ratio
• Alcoholic toxic neuropathy- Small fiber- Predominant sensory-
Axonal
• Thiamine deficient Neuropathy- Large fiber- Predominantly motor-
Demyelinating
• Thiamine deficiency Screening Test : Thiamine Deficiency
Questionnaire (TDQ)
Sgouros X. Alcohol & Alcoholism 2004; 39:227-232
11. WKS-SUSCEPTIBILITY
• Variation in Transketolase biochemical activity
• Variation in Transketolase-like 1 gene (TKTL1 gene)
• GABA-A subunit receptor gene-chr 5q33; susceptibility to alcohol
dependence and Korsakoff’s syndrome
• Genetic variants of Thiamine Transporter Protein gene; Thiamine
transport systems
13. PARENTERAL THIAMINE
Active transport is saturable at low Thiamine levels
High plasma:CNS concentration gradient is needed for passive diffusion
for rapid correction of CNS thiamine levels
14. PABRINEX
• Vitamin B1 (Thiamine) = 250mg
• Vitamin B2 (Riboflavin) = 4mg
• Vitamin B6 (Pyridoxine) = 50mg
• Nicotinamide = 160mg
• Vitamin C = 500mg
• Prophylaxis: 250mg IV OD 3-5 days
• Established WE: 500mg IV TID 3-5 days; then 250mg IV/IM OD
continued until clinical response
Supplement with 10-30mEq Mg, 60-180mEq K, 10-
40mmol/L PO4
Alcohol & Alcoholism 2005; 40:155-156 / 2002; 37:513-521
16. Multiple, small, new haemorrhages in the brainstem and the thalamus, with some spongiosis
(characteristic loosening of the neuropil) between the haemorrhages without interstitial infiltration
of macrophages; capillary proliferation, reactive astrocytes
17. A 57-year-old alcoholic man was admitted for nausea,
vomiting, and diarrhoea for 1 month, and confusion, imbalance,
and auditory and visual hallucinations for 1 week. His daughter
noticed that he had “funny eye movements” over the previous
week. A diagnosis of Wernicke’s encephalopathy was made.
Despite treatment with IV thiamine and nutritional
supplementation, the patient progressed 2 days later to coma
with total ophthalmoplegia. Two weeks after admission, the
patient was oriented to person, had recovered some horizontal
eye movements, remembered the names of his children, and
could follow simple commands.
WE CASE I
18.
19.
20. WE:NEUROIMAGING
FLAIR MR images demonstrated increased signal around the third
and fourth ventricles, Sylvian aqueduct, mamillary bodies (indicated
by arrow), and tectum.
21. A 61-year-old man was admitted with slight confusion. He had
been a heavy drinker for several years and had general fatigue.
A doctor who examined him first considered the possibility of
electrolyte disturbance and gave him an infusion including
electrolytes and glucose. After starting IV injection, the patient
fell into a deep coma. On neurologic examinations, he had
diminished doll’s head eye phenomenon and weakened deep
tendon reflexes. Laboratory findings showed severe vitamin B1
deficiency, an elevation of -glutamyl transpeptidase, and
macrocytic anemia. MRI was typical for Wernicke
encephalopathy. Although thiamine was started 5 days after
admission, the patient died of pneumonia without recovery
after 3 months. We should not forget that the activation of
glycolysis consumes vitamin B1 and that an infusion including
glucose to patients with vitamin B1 deficiency is fatal.
WE CASE II
22. WE:NEUROIMAGING
Fluid-attenuated inversion recovery brain MR images show
hyperintensity lesions around the fourth ventricle (A), in the
mamillary bodies (B), and around the third ventricle and
hypothalamus (C).
23. A 26-year-old woman presented with a 4-day history of
progressive lateral ophthalmoplegia, inability to stand, gait
ataxia, apathy, and confusion. The diagnosis of acute
Wernicke encephalopathy was consistent with characteristic
changes seen on her admission MRI.Three hours after
initiating IV thiamine, her lateral gaze returned, Romberg
test was negative, and cognition improved dramatically. Her
clinical improvement was correlated with normalization of
hyperintense T2 MRI signals 5 days later. This dramatic
turnaround underscores the necessity of IV thiamine in
acute Wernicke encephalopathy.
WE CASE III
30. KORSAKOFF SYNDROME
• Possible role of the cholinergic system in the memory deficit of
Wernicke-Korsakoff syndrome
• Morphometric measurements of the volume of magnocellular
neurones had been documented to be decreased in the NBM
(Cortex 1988; 24:367-388)
• AChEI in Korsakoff Syndrome
Increased attention and working memory abilities as evidenced
by improved scores in TMT B and logical memory on the WMS-R;
improvement in MMSE, cognitive, memory, behavioural
outcomes and QOL
Galantamine (Psychosomatics 2004; 45:4)
Donepezil (Alcohol & Alcoholism 2001; 36:553-555; Alcohol
& Alcoholism 2004; 40:151-154)
31. MARCHIAFAVA-BIGNAMI DISEASE
• Progressive demyelination and central necrosis of corpus
callosum
• Extra-callosal white matter involvement: frontal, parietal,
anterior-posterior commissures, middle cerebellar peduncles,
corona radiata
• Extracallosal cortico-cortical association fibers; cortico-
subcortical projection fibers involved; disruption of white matter
connectivity and distributed neural circuits/networks within and
between hemispheres (Hemispheric disconnection syndromes)
• Widespread cerebral functional involvement (PET, SPECT
studies)
• Acute onset MBD
• Chronic MBD
32. MARCHIAFAVA-BIGNAMI DISEASE
• Acute MBD: delirium, stupor, coma, seizures, long tract signs,
aphasia, ataxia
• Chronic MBD: frontal type dementia, paratonic rigidity, frontal
release reflexes, signs of callosal disconnection
33. MBD
The corpus callosum degenerates and splits into three layers
(“layered necrosis”). Central layer: necrosis;dorsal & ventral layers
spared; ‘Sandwich sign’
Callosal involvement Extra-callosal involvement:
deep frontal WM, corona
radiata
34. PONTINE/EXTRAPONTINE MYELINOLYSIS
• Characteristic symmetrical demyelination of central portion of
the base of the pons
• Clinical features CPM: spastic paraparesis/tetraparesis,
pseudobulbar palsy (dysphagia, dysarthria, mutism, emotional
lability, locked in syndrome, bilateral/unilateral abducens palsy,
conjugate gaze palsies; altered mental status; Horner’s
syndrome
• Sites of EPM: cerebellum, cerebral peduncles, lateral geniculate
body, thalamus, lentiform nuclei, caudate & internal capsule and
cerebral white matter
• EPM: extrapyramidal signs, cerebellar ataxia, dystonia;
behavioural abnormalities
38. ALCOHOL RELATED COGNITIVE DETERIORATION /
IMPAIRMENT
ACD/ARCI
• Ethanol/metabolites-neurotoxic per se (ARD)
• Frontal Dysexecutive Syndrome (DES)
• Alcohol related encephalopathies
WKS
MBD
Pellegra encephalopathy
Acquired hepatocerebral degeneration
• Alcohol related brain injury (ARBI)
• Co morbid substance abuse
39. ALCOHOL RELATED DEMENTIA
Oslin D, Atkinson RM, Smith DM, et al: Alcohol-related dementia: proposed clinical
criteria. Int J Geriatr Psychiatry 1998; 13:203–212
Oslin DW,Cary MS. Alcohol related dementia: Validation of diagnostic criteria. Am J
Geriatr Psychiatry 2003; 11:441-447
40. ARD
• Ventricular enlargement, selective loss of subcortical white
matter, gray matter loss, cerebral atrophy, alterations on
neuronal size, number, architecture, and synpatic & dendritic
complexity (graded brain dysmorphology)
• Lack of distinctive, well defined neuropathology
• Personality changes, apathy, poor judgement, poor memory,
cortical signs (aphasia, apraxia, agnosia)
• Alcohol + ApoE4 MCI AD
• Risk of dementia (AD, VaD) increased with increasing alcohol
consumption only in those individuals carrying the
apolipoprotein e4 allele
• There is a J or U shaped relation between alcohol drinking and
cognitive impairment or dementia
• In patients with mild cognitive impairment, up to 1 drink/day (15
g) of alcohol or wine may decrease the rate of progression to
dementia (AD, VaD) Neurology 2007; 68:1790-1799
42. DYSEXECUTIVE SYNDROME
• Frontal lobe vulnerability : increased susceptibility of frontal
brain systems to alcohol related brain damage; selective
neuronal loss in superior frontal association cortex, loss of
frontal lobe volume (white matter loss); grey matter loss
• DES characterised by three categories of cognitive dysfunction
(Berrios GE. JNNP 2000; 68:731-737)
Isolated impaired executive functions with preserved
intelligence and memory
modified dysexecutive pattern in which memory as well as
executive functions were impaired with intelligence preserved
general cognitive deterioration
43. FrDES
Maladaptive behaviour; loss of control, inability to abstain from
alcohol, repetitive habitual behaviour, and lack of forward
planning, regulating behaviour, goal directed behaviours, poor
judgement, poor problem solving abilities; poor short term
memory planning, problem solving, impulsivity, apathy, social &
personal neglect,lack of insight
Abnormal frontal neuropsychological tests (TMT B, WCST,
CWST,FAB, Verbal Fluency Test, BADS)
Vulnerability of right hemisphere: Impaired emotional
processing; decreased empathy; impairment in recognising facial
expression of emotions; emotionally flat, loss of insight etc
similar to Frontotemporal dementia syndrome
44. PELLEGRA ENCEPHALOPATHY
o Niacin deficiency encephalopathy
Encephalopathic pellegra- acute form
Insidious onset pellegra- chronic form
o Acute form:
Confusion, delirium,generalised cogwheel rigidity,Gegenhalten
phenomenon, dysarthria, myoclonus,
peripheral neuropathy,seizures
o Chronic form:
Dementia (apathy, memory failure, depression),
Dermatitis (Casal’s necklace rash, stomatitis, glossitis)
Diarrhoea
o Co-exist with WKS, MBD
o Thiamine and Vit B6 treatment can trigger development of
encephalopathic pellegra: imperative to supplement niacin
along with vitamin B complex; 300-1000mg/day acute form, 50
100mg/day after recovery
45. ALCOHOLIC CEREBELLAR DEGENERATION
• Related to Thiamine deficiency
• Loss of cerebellar cortical neurones: anterior and superior
vermis
• Gait ataxia (broad based, unstable) , Signs of limb
incoordination LL>UL
46. VITAMIN B12 RELATED NEUROLOGIC DISORDER
• Dementia
• Neuropsychiatric symptoms: personality changes, depression,
affective syndromes, psychosis (“megaloblastic madness”),
delusions, hallucinations, mania, ataxia
• Optic neuropathy, peripheral neuropathy, SCD
• Symptoms occur commonly in the absence of anemia, or
elevated MCV, a borderline low serum cobalamin: not rare and
should not be considered as evidence against the diagnosis of
cobalamin deficiency
• Useful confirmatory evidence of cobalamin deficiency: elevated
serum levels of methylmalonic acid (MMA) and total
homocysteine (Hcy) [100% specificity] ; with treatment MMA &
Hcy levels normalises (Lindenbaum J. NEJM 1988; 318:1720-
1728)
47. VITAMIN B12 RELATED LEUCOENCEPHALOPATHY
• Selective white matter involvement: diffuse
leucoencephalopathy; Disruption of WM tracts-Dysfunction of
distributed neural networks- “ White matter dementia”;
neurobehavioural syndrome
• Detrimental effect of cobalamin deficiency on the integrity of
myelin
• Leukoencephalopathy, evidenced by confluent white matter
abnormalities on MRI, is increasingly recognized as a possible
presenting sign of cobalamin deficiency, even in the absence of
anemia or myelopathy (Organic psychosis without anemia/
SCD)
• Reversible with treatment
• Diffuse supratentorial leucoencephalopathy; supratentorial WM
loss; severe periventricular/paraventricular WM bulk loss;
hydrocephalus (mimics central demyelination syndromes)
• Remarkable confluent white matter abnormalities on MRI
50. ALCOHOL RELATED FOLATE DEFICIENCY
• Folate related Dementia
• Neuropsychiatric symptomatology
• Spasticity; folate induced SCD
• Vit B12 and folate deficiency can co exist
• The treatment of cobalamin deficiency with folic acid will
result in deterioration in the neuropsychiatric status, while
improving the haematological indices (CH3 TH4 trap
hypothesis)
51. ACQUIRED NON-WILSONIAN HEPATOCEREBRAL
DEGENERATION (AHD)
• Chronic brain disorder caused by liver dysfunction and long-
standing portal-systemic shunting; repeated episodes of
hepatic insufficiency leading to CNS dysfunction; irreversible
neurological condition
• Dementia, frontotemporal cognitive dysfunction, cerebellar
signs (ataxia, intention tremors), extrapyramidal signs:
symmetric akinetic-rigid syndrome (Parkinsonian features),
movement disorders (chorea, athetosis, orobuccal dyskinesia,
myoclonus, tremors), corticospinal signs (progressive spastic
paraparesis), axonal / autonomic peripheral neuropathy
(myeloneuropathy)
• Cirrhosis-related Parkinsonism, post-shunt myelopathy and
cerebellar degeneration
• Normal serum/ urine copper & ceruloplasmin; absent KF ring
• Elevated serum Manganese levels
52. ACQUIRED NON-WILSONIAN HEPATOCEREBRAL
DEGENERATION
• MRI: Symmetrical hyperintense SI in T1WI in Globus pallidus,
midbrain (crus cerebri, substantia nigra) with sparing of red
nuclei, tectum of pons with normal T2WI
• AHD + CPM/EPM