Neurology Flashcards
What are the three clinical features of autoimmune antibody associated demyelination?
- ADEM (encephalitis/myelitis)
- optic neuritis
- transverse myelitis
What are four types of demyelinating disorders?
- CNS autoimmune encephalitis
- autoimmune antibody associated demyelination
- MS
- neuromyelitis optica (Devic’s disease)
What are the clinical criteria for ADEM?
Acute disseminated encephalomyelitis (ADEM) is an immune mediated disease of the central nervous system (CNS) that produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter.
encephalopathy, a history of viral prodome, polyfocal neurological signs - weakness, ataxia, seziures.
anti NMDA encephalitis
Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis is one of the most common subtypes of autoimmune encephalitis. presenting with psychiatric and cognitive symptoms, movement disorders, seizures, sleep disorders, autonomic failure and changes in consciousness. 38% of patients had an underlying neoplasm, most of which were ovarian teratomas. Extreme delta brush, characterized by rhythmic delta activity at 1–3 Hz with superimposed bursts of rhythmic 20–30 Hz beta frequency activity “riding” on each delta wave. sleep wake cycle inversion and mouthing/dyskinesia (brain on fire movie)
pathophysiology of ADEM
The proposed mechanism of ADEM is that myelin autoantigens, such as myelin basic protein, proteolipid protein, and myelin oligodendrocyte protein, share antigenic determinants with those of an infecting pathogen
ddx for ADEM?
autoimmune encephalitis, NMDA, Leukodystrophy, mitochondrial disorder
What are the signs of optic neuritis?
acute loss of vision (blurred, colour vision lost), painful eye movement, RAPD. children under 10 years have bilateral involvement, over 10 years have unilateral involvement and 1/3 will develop a second attack ?MOG/MS/NMOSD
Transverse myelitis? features?
1/5 of children experiencing demyelinating attack; bimodal distribution, toddler and adolescent; male predominate in younger age group. Female in adolescent usually MS/NMOSD; subacute onset of weakness, numbness, myelopathic pain (acute back pain), urinary incontinence/retention
What is the prognosis of TM?
children have better prognosis than adults with 50% making complete recovery in 2 years. mortality is high with respiratory failure in high cervical cord lesion
sensory issue and bladder dysfunction are most common (15-50%) sequelae.
viral encephalitis
HSV1, 2, VZV, EBV, CMV, MMR, flu, enterovirus, echo virus
insect borne: japanese encephalitis. west nile virus,
rick borne : encephalitis
HSV encephalitis
Whichever way the virus gains access to the brain, in the acute illness, the damage that results from the viral infection and associated inflammation is often severe. Typically, the virus is initially present in a part of the brain called the limbic cortex. It may then spread to the adjacent frontal and temporal lobes of the brain. Headache.
Confusion.
Nausea
Fever.
Seizures
Drowsiness.
diagnosis: HSV PCR lp. treatment acyclovir
japanese encephalitis
spread by mosquito: Culex tritaeniorhynchus. lthough symptomatic Japanese encephalitis (JE) is rare, the case-fatality rate among those with encephalitis can be as high as 30%. Permanent neurologic or psychiatric sequelae can occur in 30%–50% of those with encephalitis.
Hypsarrythmia in infancy
Infantile spasms (West Syndrome)
EEG initially normal, and then multifocal polyspike waves in infancy
Dravet
SCN1A
Problem in voltage gated sodium channels, AVOID phenytoin
bisynchronous 3Hz spike waves
childhood absence seizures (2 spikes, one early childhood, one later)
centorotemporal spikes (activated by sleep)
Benign focal epilepsy of childhood
KCNQ2
slow spike wave
lennox gastault (drop attack)
paroxysmal 4 Hz theta waves and then fast spike and polyspike
Doose syndrome (monoclonic astatic epilepsy
EEG: 4-6 Hz poly-spike and slow wave discharges, often seen on sleep/wake changeover, occasionally with photostimulation
Juvenile Myoclonic Epilepsy
GABRA1 – GABA associated, basic bitches need keppra
GRIN2A
Landau-Kleffner
occipital spikes, Vomiting, pallor/flushing, loss of tone
PANAYIOTOPOULOS
EEG findings present when eyes are closed!
+ blindness
GASTAUT
EEG normal baseline, in seizures show centro-temporal spikes
Benign familial infantile epilepsy
PRRT
febrile seizures that progress to status
Genetic epilepsy with febrile seizures plus
sleep associated epilepsy syndromes
Landau-Kleffner
CSWS
As in the name, continuous spikes and waves during sleep
continuous spikes and waves during sleep, global developmental regression as seizing/sleeping
Normal background, when sleep deprived can see 3 Hz spikes and waves.
childhood absence epilepsy
Infantile spasms (West Syndrome) treatments
if assoc with Tuberus sclerosis: pred
if not: vigabatran
Dravet treatment
valproate, clobazam, not phenytoin
what do you not give in absence?
carbamazepine and phenytoin, just give ethoxamide
lennox gastault/myotonic astatic (doose) and juvenile myoclonic epilepst
sodium valproate
benign focal epilepsy of childhood
carbamazepine
Non convulsive status epilepticus
manifests primarily as altered mental status as opposed to the dramatic convulsions seen in generalized tonic-clonic status epilepticus. patients present with confusion or abnormal behavior, suggesting the diagnosis of absence status epilepticus, complex partial. also those in a coma
leion involving the anterior horn cells
SMA
gene: 22
