1) A 15-year-old female presented with fever, headache, vomiting and altered sensorium for a week and was diagnosed with acute disseminated encephalomyelitis (ADEM), an inflammatory disease of the central nervous system.
2) Investigations showed bilateral disc edema on fundus examination and MRI revealed extensive, multifocal white matter abnormalities.
3) Treatment involves high dose corticosteroids like methylprednisolone and intravenous immunoglobulin to reduce inflammation. The long-term risk of developing multiple sclerosis after ADEM is 25%.
brief description and summary of Acute disseminated Encephalomyelitis-most common Paediatric demyelinating disorder-clinical features, epidemiology. Approach between MS and ADEM
brief description and summary of Acute disseminated Encephalomyelitis-most common Paediatric demyelinating disorder-clinical features, epidemiology. Approach between MS and ADEM
Approach to different Demyelinating disorders in the Paediatric age-group. Namely- acute disseminated encephalomyelitis, paediatric multiple sclerosis, neuromyelitis optica. Approach, MRI features, differences, clinical features
Neuromyelitis optica spectrum disorders (NMOSD) is an autoimmune disorder of young adults' results from astrocytic aquaporin–4 (AQP–4) channelopathy. The AQP–4 IgG antibodies may be present in the context of some paraneoplastic disorders which should be suspected when NMOSD occurs in elderly patients.
Multiple sclerosis (MS) is a demyelinating disease in which the insulating covers of nerve cells in the brain and spinal cord are damaged.This damage disrupts the ability of parts of the nervous system to communicate, resulting in a range of signs and symptoms, including physical, mental, and sometimes psychiatric problems
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.
Approach to different Demyelinating disorders in the Paediatric age-group. Namely- acute disseminated encephalomyelitis, paediatric multiple sclerosis, neuromyelitis optica. Approach, MRI features, differences, clinical features
Neuromyelitis optica spectrum disorders (NMOSD) is an autoimmune disorder of young adults' results from astrocytic aquaporin–4 (AQP–4) channelopathy. The AQP–4 IgG antibodies may be present in the context of some paraneoplastic disorders which should be suspected when NMOSD occurs in elderly patients.
Multiple sclerosis (MS) is a demyelinating disease in which the insulating covers of nerve cells in the brain and spinal cord are damaged.This damage disrupts the ability of parts of the nervous system to communicate, resulting in a range of signs and symptoms, including physical, mental, and sometimes psychiatric problems
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.
Acute onset infection
Monophasic immune mediated polyneuropathy
Rapid progressive motor paralysis
Affects people of all ages and is not hereditary
Post infectious disease
It can follow by systemic infections
Auto immune in nature
OTHER TERMS
Acute inflammatory demyelinating poly radiculopathy (AIDP)
Acute idiopathic poly radiculo neuritis
Acute idiopathic neuritis
French polio
Landry Guillain Barre Syndrome
TYPES - Acute inflammatory demyelinating poly radiculo neuropathy (AIDP)
Acute Motor Axonal neuropathy(AMDN)
Acute Motor &Sensory Axonal neuropathy(AMSAN)
Miller Fisher Syndrome(MFS)
Polyneuritis Cranialis
CLINICAL MANIFESTATIONS
Paresthesia is frequent followed by paralysis in the extremities
Hypotonia
Areflexia Autonomic dysfunctions include orthostatic hypotension
Hypertension
Abnormal vagal responses
Bowel and bladder dysfunction
Facial flushing
Diaphoresis
Syndrome of inappropriate anti diuretic hormoneProgression of Guillain barre syndrome
include lower brain stem that involves the
Facial Nerve
Abducens Nerve
Oculo Motor Nerve
Hypoglossal Nerve
Trigeminal Nerve
Vagus Nerve
Pain Is a common symptom and It becomes worse at Night.
TREATMENT
On set to two weeks: Plasma pheresis (40-50 ml/kg four times a week
After two weeks: intravenous administration of high dose immunoglobulin (Sandoglobulin)
Beyond three weeks: plasma exchange and immunoglobulin therapies
Chest Physiotherapy
Artificial ventilation-Maintain Gas Exchange
COMPLICATIONS
Cardiac arrhythmias
Respiratory failures
Dys autonomia
Pneumonia
Adult Respiratory Distress Syndrome
Septicemia
Death
Tuberous sclerosis dr. amit vatkar, pediatric neurologistDr Amit Vatkar
Tuberous Sclerosis is a genetically inherited neurocutaneous syndrome can affect families in an autosomal dominant.
in this presentaion i will try and give u a review to the case and its management.
it will help u get a n outllook to diagnose a case of tuberous sclerosis
i have shown some images of the lesions present in the case to get a photographic memory.
1. IMAGE OF THE WEEK
THELENGANA A
PG 1STYR
FROM IMCU WARD
2. • 15 Yrs old female presented with h/o
Fever 2 days
Asymptomatic 10 days
Headache,vomiting
Altered sensorium for 1 week
No h/o seizures/visual disturbance
No h/o vaccination /exanthematous illness
3. O/E
vitals were stable
CNS examn :Pt was drowsy , arousable with
painful stimulus
PERL , DEM preserved
exaggerated DTR
B/L plantar extensor
fundus examination – B/L disc edema
Other systemic examination was unremarkable
13. ACUTE DISSEMINATED
ENCEPHALOMYELITIS
Inflammatory, nonvasculitic, demyeli
nating, immune
mediated, monophasic and
polysymptomatic disease of the
central nervous system
Post infectious encephalomyelitis,
Post vaccination encephalomyelitis
14. PATHOGENESIS
• Molecular mimickery: brain vaccines
– Th2 lymphocytes have increased reactivity to
myelin basic protein
• Inflammatory cascade concept:
– CNS infections triggering immune response,
damage to BBB, brain specific antigens spills into
systemic circulation and initiates immunologic
process
17. NEUROIMAGING
• MRI: extensive, multifocal, subcortical
white matter abnormalities
• MRI: subcortical white matter, may be grey
matter also,
• CT may be normal in 50% cases
• Convalescent MRI helpful in diffrentating with
MS, new lesions in MS
18. MRI Features
ADEM
• Patchy, poorly marginated areas of increased signal
intensity; large, asymmetric, multiple
• Four patterns:
– ADEM with less than 5 mm lesions
– Large, confluent lesions with edema and mass
effect
– ADEM with additional symmetric bithalamic
involvement
– Acute hemorrhagic encephalomyelitis (worst
prognosis)
20. TREATMENT
• Broad spectrum antibiotics and acyclovir until
an Infectious etiology is excluded.
• Methylprednisolone in a dose of 30 mg/kg per
day intravenously up to a maximum dose of
1000 mg per day X 5 days
• Plasmapharesis
• Intravenous immunoglobulin
• Cyclosporin , cyclophosphamide
• Methylpred + IVIG
21. PROGNOSIS
• Mortality: 10% in older studies, Now <2%
• Morbidity: visual, motor, autonomic, and intellectual
deficits and epilepsy.
– Problems persist after the first few weeks of
illness in only about 35% of cases, and in most of
these patients, the deficits resolve within 1 year of
onset.
22. FOLLOW UP
• The long-term (10-y follow-up) risk of patients
with ADEM for development of MS is 25%.
• Risk for MS is highest in children whose ADEM
onset was
– (1) afebrile,
– (2) without mental status change,
– (3) without prodromal viral illness or
immunization,
– (4) without generalized EEG slowing,
– (5) associated with an abnormal CSF immune
profile