Autoimmune encephalitis a term used in children presenting with neurological syndrome associated with serum and/ or cerebrospinal fluid antibodies directed against ion channels, receptors and associated proteins.
It comprises group of clinical syndrome that can occur at all ages but preferentially affect younger adult and children.
Auto antibodies against:
Neuronal cell surface protein
Synaptic receptors involved in transmission ,plasticity ,excitability.
Triggers: Tumors, Post viral infections, Post vaccination.
Autoimmune encephalitis includes ADEM
Anti NMDAR encephalitis
Encephalitis a/w GABA R antibodies
NMOSD
Opsoclonus-myoclonus & cerebellar - brainstem encephalitis
Bickerstaff encephalitis
Hashimoto encephalitis
Rasmussen encephalitis
Basal ganglia encephalitis
CLIPPERS
ROHHAD.
ADEM is MC cause of autoimmune encephalitis in children and adolescents.
Acute onset of polyfocal neurological deficit accompanied by encephalopathy and changes compatible with demyelination on MRI brain.
a presentation on autoimmune encephalitis, paraneoplastic syndrome. their types and various imaging and lab finding
their differential diagnosis
acute and long term management plans
brief description and summary of Acute disseminated Encephalomyelitis-most common Paediatric demyelinating disorder-clinical features, epidemiology. Approach between MS and ADEM
Anxiety, defined as dread or apprehension, is not considered pathologic, is seen across the life span, and can be adaptive (e.g. the anxiety one might feel during an automobile crash).
Anxiety becomes disabling.
Interfering with social interactions, development.
Achievement of goals or quality of life.
Can lead to slow self esteem, social withdrawal.
Academic underachievement.
The average age of onset of anxiety disorder is 11 years.
This is the most common psychiatric disorders of childhood.
Occurs in 5-18% of all children and adolescents.
Prevalence rate is comparable to physical disorders such as asthma and diabetes.
One of the most common childhood anxiety disorder.
Prevalence- 3.5-5.4%
Girls ˃ boys
Common in prepubertal children. Average age of onset 7.5 yrs.
It is developmentally normal when it begins about 10 month of age and tapers off by 18 month.
By 3 years of age, most children can accept the temporary absence of their mother or primary caregiver.
SAD is characterised by unrealistic and persistent worries about separation from home or a major attachment figure.
Nipah Virus (NiV) is a negative sense, single stranded, enveloped RNA virus.
Zoonotic virus
Family – Paramyxoviridae
Genus - Henipavirus
It is a BSL-4 pathogen.
The name "Nipah" refers to the place, Sungai Nipah (literally 'nipah river') in Malaysia, the source of the human case from which Nipah virus was first isolated.Nipah virus can remain viable for a few days in few fruit juices or mango fruits, and at least 7 days in palm milk.
Bats act as a breeding ground for many dangerous viruses, including Nipah, rabies, and Marburg viruses. Such viruses are not associated with any major pathological changes within the bat population.
Transmission of NiV occurs by eating contaminated food. Risks include contact, touch, breastfeeding, or exposure to an infected person, thereby making it easier to come in contact with a droplet of NiV infection.
More recently, experimental studies with aerosolized NiV in Syrian hamsters have found that NiV droplets (aerosol distribution) may cause NiV transmission during close contact. Drinking fresh palm milk is a very common method, and the use of Tari (ripe palm juice) is a powerful way to transmit the virus.
a presentation on autoimmune encephalitis, paraneoplastic syndrome. their types and various imaging and lab finding
their differential diagnosis
acute and long term management plans
brief description and summary of Acute disseminated Encephalomyelitis-most common Paediatric demyelinating disorder-clinical features, epidemiology. Approach between MS and ADEM
Anxiety, defined as dread or apprehension, is not considered pathologic, is seen across the life span, and can be adaptive (e.g. the anxiety one might feel during an automobile crash).
Anxiety becomes disabling.
Interfering with social interactions, development.
Achievement of goals or quality of life.
Can lead to slow self esteem, social withdrawal.
Academic underachievement.
The average age of onset of anxiety disorder is 11 years.
This is the most common psychiatric disorders of childhood.
Occurs in 5-18% of all children and adolescents.
Prevalence rate is comparable to physical disorders such as asthma and diabetes.
One of the most common childhood anxiety disorder.
Prevalence- 3.5-5.4%
Girls ˃ boys
Common in prepubertal children. Average age of onset 7.5 yrs.
It is developmentally normal when it begins about 10 month of age and tapers off by 18 month.
By 3 years of age, most children can accept the temporary absence of their mother or primary caregiver.
SAD is characterised by unrealistic and persistent worries about separation from home or a major attachment figure.
Nipah Virus (NiV) is a negative sense, single stranded, enveloped RNA virus.
Zoonotic virus
Family – Paramyxoviridae
Genus - Henipavirus
It is a BSL-4 pathogen.
The name "Nipah" refers to the place, Sungai Nipah (literally 'nipah river') in Malaysia, the source of the human case from which Nipah virus was first isolated.Nipah virus can remain viable for a few days in few fruit juices or mango fruits, and at least 7 days in palm milk.
Bats act as a breeding ground for many dangerous viruses, including Nipah, rabies, and Marburg viruses. Such viruses are not associated with any major pathological changes within the bat population.
Transmission of NiV occurs by eating contaminated food. Risks include contact, touch, breastfeeding, or exposure to an infected person, thereby making it easier to come in contact with a droplet of NiV infection.
More recently, experimental studies with aerosolized NiV in Syrian hamsters have found that NiV droplets (aerosol distribution) may cause NiV transmission during close contact. Drinking fresh palm milk is a very common method, and the use of Tari (ripe palm juice) is a powerful way to transmit the virus.
In research, the term sample is used to denote individuals who are observed for exposure to certain risk factors, outcomes and related variables.
Ultimately what we conclude from the sample, is often generalized to whole population from which the sample is selected.
Universe (whole population)- Entire group of the study population is known as universe or whole population. Population is often too large to cover in its entirety.
Sampling Unit- Each member of the whole population is known as sampling unit.
Sampling Frame- A list where all individuals from the whole population are drawn up is known as sampling frame.
Sample- Sample is a small representative part of the whole population.
Advance life support refer to a constellation of interventions needed to support the vital physiological process during a critical illness, while we await response with definitive therapy. These life support measures are instituted to prevent cardiac arrest.
To recognise physiological derangements that arise out of multiple etiologies and stabilize them first.
EVALUATE – IDENTIFY – INTERVENE
The steps of evaluation are
1.Initial impression
2. Primary assessment
3. Secondary assessment
4. Diagnostic test
Gives insight to overall physiological status and functioning of the brain.
TICLS
Tone: Look for general posture of the child has adopted
Interactive: Is the child responsive and interacting appropriately, unresponsive or lethargic.
Consolable: Irritable, consolable or inconsolable
Look\Gaze: How is the child looking at mother, any vacant gaze
Speech: Is the child able to speak or vocalise as is appropriate for age or is there a paucity\weak\hoarseness of voice.
IDENTIFY = Abnormality in any of these parameters point towards a brain dysfunction
Impaired consciousness is a significant alteration in the awareness of self and environment with varying degree of wakefulness.
Unconsciousness persisting for at lest 1 hr – Coma.
Younger children more likely to have coma or altered sensorium secondary to non-traumatic etiology, where as traumatic brain injury is more common in older children.
Always rule out reversible causes of coma, like hypoglycemia, hyperglycaemia and electrolyte imbalance.
Any severe systemic illness can cause altered consciousness as a result of hypoxic ischemic insult, which if on-going can aggravate raised ICT.
Patient was apparently all-right one and half years back, when he developed sudden onset severe constricting type retrosternal pain at rest with radiation to left arm associated with shortness of breath, nausea and vomiting. He was admitted in the local hospital , ECG was done and told to have heart attack. He was discharged after 7 days and advised to attend a higher cardiac centre for coronary angiography. But he didn’t followed up after that and was on irregular oral medication.
A 62 years old diabetic, smoker, non-hypertensive male presented with acute coronary syndrome like symptoms one and half year back followed by angina of CCS class II which progressed to CCS class III over last 3 months. Patient also had shortness of breath which progressed from NYHA Class II to NYHA Class IV over last 3 months with history suggestive of orthopnea.
D/D
Ischemic heart disease
Hypertrophic cardiomyopathy
Valvular heart disease (Aortic valvular disease)
Dilated cardiomyopathy
RSOV
Long case examination done during MBBS and MD examination. Neurology case is mostly the long case. History, general examination , systemic examination, provisional diagnosis, investigation and final diagnosis are the sequential steps. Neurology examinations includes higher mental function, cranial nerve examination, motor and sensory system examination, cerebellar signs, gait, peripheral nerves, spine and skull and peripheral nerve examination.
Mediquiz organised for MBBS students. Image rounds, spotters, connecting dots, guess the gaits are varieties of questions. Diseaes that celebrities are suffereing from also asked.
Long case examination done during MBBS and MD examination. Neurology case is mostly the long case. History, general examination , systemic examination, provisional diagnosis, investigation and final diagnosis are the sequential steps. Neurology examinations includes higher mental function, cranial nerve examination, motor and sensory system examination, cerebellar signs, gait, peripheral nerves, spine and skull and peripheral nerve examination.
The human body is uniquely designed to live in Earth’s gravity. In space, the body begins to adapt to the microgravity environment.
When an astronaut goes into space, his or her body will immediately begin to experience a multitude of changes that cause the astronaut to feel different and even look slightly different!
Much more research needs to be done to develop countermeasures to the body’s changes in microgravity.
Space Medicine establishes the countermeasures to overcome the physiological effects of space.
The results will help to improve the health of astronauts and pave the way for long-term space exploration, such as a trip to Mars. Space medicine and space physiology are often viewed as two aspects of space life sciences, with the former being more operational, and the latter being more investigational. Space medicine tries to solve medical problems encountered during space missions. These problems include some adaptive changes to the space environment, including weightlessness, radiation, the absence of the 24-hour day/night cycle; as well as some non-pathologic changes that become maladaptive on return to Earth, such as muscle atrophy and bone demineralization. Space physiology tries to characterize body responses to space, especially weightlessness, reduced activity, and stress. It provides the necessary knowledge required for an efficient space medicine.
Atrial fibrillation is the most common arrhythmia. AF is the common cause of stroke/CVA. Conventionally anti coagulation with warfarin is preferred. Now left atrial appendage closure devices are also considered as a treatment strategy. Two RCT PROTECT AF and PREVAIL shows Non-inferiority compared to warfarin.
Three primary end points stroke (hemorrahagic or ischemic), systemic embolism and cardiovascular or unexplained death.
The choice to include CV/unexplained death, as an end point, an event unlikely to be affected by either treatment arm, biases towards non-inferiority. Due to so many loopholes in RCTs and higher rates complications with watchman, Anticoagulation is still considered as the first choice.
We need more number of large RCT to show that LAA Device closure is a feasible alternative.
Fever, common cold and cough in pediatric age groups are common. Acute bronchiolitis is a diagnostic term used to describe the clinical picture produced by several different lower respiratory tract infections in infants and very young children (younger than 1yr ,some clinicians extend it to the age of 2 yr). Pneumonia defined as inflammation of lung parenchyma.
It is the leading infectious cause of death globally among children younger than 5 yr.
The introduction of antibiotics and vaccine against measles , pertussis ,haemophilus influenzae type b and PCV vaccine reduces the pneumonia related mortality over past 15 yr.
INTERPRETATION OF COMMON BIOCHEMICAL TESTS INCLUDING LFT & RFT.pptxDr Debasish Mohapatra
Biochemical tests are commonly used in day-to-day practices for diagnosis of diseases. Liver function test and renal function tests are common tests done.
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
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
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...Oleg Kshivets
Overall life span (LS) was 1671.7±1721.6 days and cumulative 5YS reached 62.4%, 10 years – 50.4%, 20 years – 44.6%. 94 LCP lived more than 5 years without cancer (LS=2958.6±1723.6 days), 22 – more than 10 years (LS=5571±1841.8 days). 67 LCP died because of LC (LS=471.9±344 days). AT significantly improved 5YS (68% vs. 53.7%) (P=0.028 by log-rank test). Cox modeling displayed that 5YS of LCP significantly depended on: N0-N12, T3-4, blood cell circuit, cell ratio factors (ratio between cancer cells-CC and blood cells subpopulations), LC cell dynamics, recalcification time, heparin tolerance, prothrombin index, protein, AT, procedure type (P=0.000-0.031). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and N0-12 (rank=1), thrombocytes/CC (rank=2), segmented neutrophils/CC (3), eosinophils/CC (4), erythrocytes/CC (5), healthy cells/CC (6), lymphocytes/CC (7), stick neutrophils/CC (8), leucocytes/CC (9), monocytes/CC (10). Correct prediction of 5YS was 100% by neural networks computing (error=0.000; area under ROC curve=1.0).
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
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Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
New Drug Discovery and Development .....NEHA GUPTA
The "New Drug Discovery and Development" process involves the identification, design, testing, and manufacturing of novel pharmaceutical compounds with the aim of introducing new and improved treatments for various medical conditions. This comprehensive endeavor encompasses various stages, including target identification, preclinical studies, clinical trials, regulatory approval, and post-market surveillance. It involves multidisciplinary collaboration among scientists, researchers, clinicians, regulatory experts, and pharmaceutical companies to bring innovative therapies to market and address unmet medical needs.
2. INTRODUCTION:
◦ Autoimmune encephalitis a term used in children presenting with neurological syndrome associated
with serum and/ or cerebrospinal fluid antibodies directed against ion channels, receptors and
associated proteins.
◦ It comprises group of clinical syndrome that can occur at all ages but preferentially affect younger
adult and children.
◦ Auto antibodies against:
1. Neuronal cell surface protein
2. Synaptic receptors involved in transmission ,plasticity ,excitability.
◦ Triggers:
1. Tumors
2. Post viral infections
3. Post vaccination
4. ADEM:
◦ MC cause of autoimmune encephalitis in children and adolescents.
◦ Acute onset of polyfocal neurological deficit accompanied by encephalopathy and
changes compatible with demyelination on MRI brain.
5. EPIDEMIOLOGY:
◦ It can occur at any age most cases reported 5-8 yrs, slight male predominance.
◦ Usually it is monophasic, if recurrence occur 3 month or longer after the first episode termed as
MDEM (Multiphasic Disseminated Encephalo-Myelitis).
◦ 50% ADEM MOG (Myelin Oligodendrocyte Glycoprotein) Ab +, almost all case of MDEM MOG Ab +.
◦ ADEM can be followed by demyelination in a new location & if MOG-Ab negative - MS
◦ ADEM f/b relapse in specific location like
Optic nerve -ADEM-ON
◦ Optic nerve and spinal cord- NMOSD . Both frequently a/w MOG-Ab positive.
7. CLINICAL MANIFESTATION:
◦ Initial symptoms include- lethargy ,fever ,headache ,vomiting ,meningeal sign and seizure.
◦ Encephalopathy:
1. Is hallmark
2. Ranging from change in behaviour , persistent irritability to coma.
◦ Focal neurological deficit:
1. Difficult to ascertain in obtunded or very young child ,
2. Common neurological signs- visual loss, ataxia, motor and sensory deficit, bowel bladder dysfunction
if spinal cord demyelination occurs.
◦ It is usually rapidly progressive over days may need ICU admission particularly when
1. Brain stem dysfunction
2. Raised ICT.
8. DIAGNOSIS:
Clinical
CSF • Often normal, pleocytosis with lymphocytic or
monocytic predominance.
• Proteins can be elevated but true OCB rare.
• MOG AB +ve
Serum • MOG Ab +ve
EEG • Generalised slowing, but focal slowing or epileptiform
discharge may present.
MRI (Imaging study of choice) • B/L large multifocal sometimes confluent edematous
mass like lesions with variable enhancement within
white & gray matter.
• Typically appears to be of similar age.
• Repeat MRI after 3- 12 months – improvement or
complete resolution.
CT Scan • Normal/Hypodense lesions
10. Treatment:
Symptomatic treatment
• Empirical antibiotics and antivirals should be considered when infective evaluations are pending
High dose steroids
• IV methylprednisolone- 20-30 mg/kg for 5 days (max. 1000mg/day)
• Followed by oral prednisolone 1-2 mg/kg/day over 4-6 weeks (max. 40-60 mg/day)
For refractory or severe cases
Others options
• IVIG – 2 gm/kg over 2-5 days
• Plasmapheresis- 5-7 exchange every other days
11. Prognosis:
◦ Most children experiences full motor recovery.
◦ But residual defect may be seen- cognitive deficit & behavioural changes.
Differential diagnosis:
◦ Multiple sclerosis
◦ Leukodystrophy
◦ Vasculitis
◦ Tumor
◦ Ab associated disorder
12.
13. Anti NMDAR Encephalitis:
(N-Methyl D-Asparatate Receptor)
◦ Second MCC of AE.
◦ Female > Male ( < 12 years Male > Female)
◦ Underlying teratoma (40% of female >12 yrs ovarian teratoma)
◦ Pathogens:
1. Mycoplasma pneumoniae
2. HSV-1
3. HHV-6
4. Enterovirus
5. Influenzae
HSV Encephalitis
(Ab against GluN
1 subunit of
NMDAR)
2-12 weeks after
treatment
completion
Relapse of
symptoms-
Choreoathetosis
post HSV
Encephalitis
15. Clinical features:
Usually evolve in stages
Prodormal symptoms
• Viral illness like
fever, headache.
Psychiatric
manifestation
• Rapidly progressive
anxiety
• Mood disturbances
• Catatonia
• Bizzare behaviour
• Memory deficit
• Language
disintegration
• Insomnia
After few days to weeks
• Decreased level of
consciousness
• Seizure
• Limb or oral
dyskinesia
• Choreoathetoid
movements
• Autonomic
instability (tachy-
brady, fluctuation of
BP, hypoventilation,
hyperthermia,
sialorrhea)
In Teenagers & young adults:
16. In toddlers & infants:
Psychiatric manifestation- may
be missed
• Irritability
• New onset temper tantrum
• Agitation
• Aggression
• Reduced speech
• Mutism
• Autistic like regression
After few days to weeks
• Decreased level of
consciousness
• Seizure
• Limb or oral dyskinesia
• Choreoathetoid movements
• Autonomic instability- less
sever
• Some children may develop
cerebellar ataxia &
hemiparesis
17. Diagnosis:
Clinical
CSF Moderate lymphocytic pleocytosis
Increased protein synthesis & OCB ( less frequent)
NMDAR Ab – 100% sensitivity
Serum NMDAR Ab-85% sensitivity
EEG Focal or diffuse slow activity in delta & theta range
Characteristic- Extreme delta brush
MRI Brain Abnormal in 35%
T2 FLAIR – cortical and subcortical signal abnormality
MRI/USG Abdomen & Pelvis R/O teratoma
19. Treatment:
◦ Symptomatic
management
◦ Tumor Removal
◦ Immunotherapy
First line
• Steroid, IVIg or Plasma exchange
50% treatment
failure
• Rituximab
• May used in combination or after 1st line therapy
If no response
• Cyclophosphamide
20. Prognosis:
Mortality Rate- 7%
Recovery (substantially/fully)- 80%
• May take as long as 2 years after symptoms onset
• Last symptoms to improve social interaction, language, executive function
Relapse- 15%
Milder than initial episodes, response well to immunotherapy
Efficacy of chronic immunosuppressant (azathioprine,MMF) in preventing relapse is unknown
21. D/D of NMDAR AE
DIFFERENTIAL DIAGNOSIS Differentiating features
Viral Encephalitis CSF findings, psychosis, dyskinesia -less
New onset psychosis No neurological manifestations
Neuroleptic malignant syndrome Dyskinesia, catatonia - absent
Relapsing post HSV encephalitis Viral PCR, response to acyclovir, progression of
necrotic changes on MRI
Drug/Toxins Ketamine, phencyclidine (dopamine agonist)
Systemic rheumatic disease Involvement of other organs (skin, joint, kidney,
blood vessels, blood forming cells)
CNS vasculitis FLAIR T2 MRI- Ischemia & microhemorrhages
Acquired demyelinating disease ADEM & NMOSD
22. Encephalitis with Ab against GABA-A
Receptor:
◦ Rare AE, that can affect children.
◦ In adults may occur with thymoma.
◦ Present with status epilepticus, refractory seizure.
◦ MRI (T2/FLAIR) brain shows- multifocal hyperintense abnormality.
◦ Treatment- Immunotherapy & removal of tumor.
23. Ophelia Syndrome
◦ Occur in a/w Hodgkin lymphoma.
◦ Predominantly affect young adults, teenager & children.
◦ Some develops Ab against mGluR5 receptor involved in learning & memory.
◦ Management:
1. Removal of tumor
2. Immunotherapy
24. Hashimoto Encephalopathy:
◦ Steroid responsive encephalopathy with autoimmuno-thyroiditis.
◦ Detection of TPO Ab in patients with acute/subacute encephalitis.
◦ Detection of TPO Ab is a marker of autoimmunity rather than a disease specific marker.
◦ So testing of more relevant Ab like NMDAR Ab should be done.
25. Bickerstaff Encephalitis:
◦ Rapid progression (<4wks) of
1. B/L external ophthalmoplegia
2. Ataxia
3. Decreased level of consciousness
◦ Mostly affect adults, but children of 3 yrs old have been identified.
◦ Serum GQ1b IgG Ab found in 66% cases.
◦ May develop hyporeflexia and overlap with MFS.
◦ MRI T2 signal abnormality (30%)- Brainstem, thalamus & cerebellum.
◦ Good response to immunotherapy.
26. Rasmussen encephalitis:
◦ Inflamatory encephalopathy c/b
1. Refracory focal seizures
2. Cognitive deterioration
3. Focal neurological deficit.
◦ Frequently affect children 6-8 yr old but
adolescents &adults can be affected.
Etiology:
◦ Unknown.
◦ Theories proposed are neuronal antibody &
T- cell mechanisms - triggered by viral
infection.
MRI Brain- Cerebral hemiatrophy
27. Management of Rasmussen encephalitis:
1. High dose steroid, IVIG or plasma exchange.
2. Rituximab & intraventricular Gamma interferon effective in isolated cases.
3. Tacrolimus- better outcome of neurological function & slower progression of cerebral atrophy
but no effect on seizure control.
4. Adalimumab( monoclonal antibody against TNF-alpha) shows seizure control &preservation of
cognitive function in 50% cases.
5. Most effective treatment for controlling seizure is functional hemispherectomy i.e. surgical
disconnection of affected hemisphere.
28. Autoimmune limbic encephalitis:
◦ Inflamatory process of the limbic system including medial temporal lobe, amygdala, cingulate gyrus.
◦ Most commonly found in adults.
◦ Some patients there is underlying tumor i.e. leukemia, ganglioneuroblastoma, neuroblastoma, small cell
carcinoma of ovary.
◦ Autoantibodies are-
1. VGKCs /LGI1(Leucine rich glioma inactivated 1)
2. Caspr2 antibodies
30. Opsoclonus-myoclonus and other type of brainstem-
cerebellar encephalitis:
◦ In children- 50% have underlying NEUROBLASTOMA.
◦ In teenagers and young adults underlying TERATOMA usually in ovaries.
◦ Initially present with- irritability, ataxia, falling, myoclonus, tremor and drooling.
◦ Later- hypotonia, Opsoclonus c/b rapid, chaotic, multidirectional eye movement without saccadic interval.
◦ Immunotherapy improves abnormal eye movement but residual behavioral, language, cognitive problems
persists.
◦ Delay in treatment appears to be a/w poor outcome , therefore in case of neuroblastoma removal of the
tumor should not delay the start of immunotherapy.
31. CLIPPERS:
◦ Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids
◦ Pontine predominant encephalomyelitis
◦ Usually present with episodic diplopia and facial parasthesia.
◦ MRI Brain – symmetric curvilinear gadolinium enhancement around the pons & extend variably into
medulla, cerebellum, midbrain and spinal cord.
◦ Responds to high dose of steroids , may worsen during steroid tapering, requiring chronic steroid.
Brainstem & spinal cord
dysfunction
32. ROHHAD:
◦ Rapid-onset Obesity with Hypothalamic dysfunction, Hypoventilation & Autonomic Dysregulation.
◦ An autoimmune or
paraneoplastic etiology.
◦ Response to immunotherapy
in few patients.
Children having
normal
development till
2-4 yrs
Rapid onset of
hyperphagia,
weight gain,
abnormal
behaviour
• Social disinhibition,
impulsivity, lethargy,
outburst of euphoria &
laughing
Autonomic
dysfunction &
central
hypoventilation
• Abnormal pupillary
responses, thermal
dysregulation, GI
dysmotility
33. NMOSD
◦ Typical involvement of optic nerve & spinal cord.
◦ Encephalopathy in the form of diencephalic or area postrema syndromes.
◦ Can have AQP4 or MOG Ab. Some are seronegative.
◦ Involvement of brain areas rich in AQP4 (periaqueductal gray matter, hypothalamus, optic nerve
and central involvement of the spinal cord).
◦ High risk of relapses and long term disability.
◦ Requires chronic immunotherapy.
◦ Patients with MOG Ab have better outcome than those with AQP4 Ab or seronegative cases.