Neurology_TN_UW_0823 Flashcards
Greatest risk factor for prematurity
Cerebral Palsy
EEG of complex partial seizures
Normal or brief discharges
What is pseudomotor cerebri
idiopathic intracranial hypertension
Homocystinuria?
A/R disorder due to cystathionine synthase deficiency results in error in methionine metabolism
What are some of the pathognomoic feature/events in homocystinuria?
Intellectual disability and cerebrovascular accidents/thromobosis
Tx for homocytinuria
vit supplementation, antiplatelet or anticoagulation to prevent thromboembolic events
Loss of consciousness is seen with what kidn of partial seizures
Complex partial and partial seizure with secondary generalization
Infant botulism is caused by? Where can it come from?
Clostridium botulinum spores. Honey and also spores from environmental dust/soil.
What is the presentation of infant botulisnism?
Descending flaccid paralysis, autonomic symptoms like constipation, drooling, bulbar palsies (ptosis, sluggish pupillary responses, poor suck/gag reflex)
What is the treatment for infant botulism
Human derived botulism immune globin
Epidural hematoma clinical ppt
Lucid interval and then rapid deterioriation
CT appearance of epidural hematoma
Biconvex mass on CT scan
tx for epidural hematoma
If patients show deteriorating neurological status or increasing ICP = emergent craniotomy
Ascending polyneuropathy after recent GI infection?
Guillan barre
Underlying pathology of guillan barree involves what ?
Peripheral motor nerves
Final stage of guillan barre?
Flaccid paralysis, absent deep tendon reflexes and nerve conduction abnormalities
What does CSF in guillan barre show?
Elevated protein levels and normal cell count
Illnesses that involve anterior horn cells?
spinal muscular atrophy (flaccid paralysis in infancy), poliomyelitis, and ALS
What is the best means of monitoring respiratory function in patients with Guillan barre
Serial measurements of forced vital capacity
What are important predisposing risk factors for brain abscesses
otitis media, mastoiditis, frontal or ethmoid sinusitis, dental infection, bacteremia from other sites of infection, congenital heart disease
Classic triad of brain abscesses
Fever, headaches (nocturnal or morning), focal neurological changes. Seizures present in 25% of cases.
What is polycythemia
Increased concentration of hemoglobin
Infantile spasms can develop into
West syndrome (infantile spasms, psychomotor developmental arrest, hyperarrythmia) or lennox gastuaut
Typical EEG for infantile spasms
Hypsarrythmia (high voltage slow waves, spikes and polyspikes, background disorganization)
Infantile spasm onset
4-8 months
Tx for infantile spasms
Vigabatrin, ACTH, benzodiazepines
Lennox-Gastaut onset?
3-5 years
Lennox-Gastaut triad?
1) multipe seizure types 2) diffuse cognitive dysfunction 3) slow generalized spike and slow wave EEG
Tx for lennox-gaustaut
Valproic acid, benzoes, ketogenic diet; however, response often poor
Juvenile myoclonic epilepsy aka
Janz
Janz onset?
12-16 years of age. A/D with variable penetrance
PPT of Janz
Myoclonus particularly in morning, frequently presents as generalized tonic-clonic seizures
Typical EEG for Janz
3.5-6 Hz irregular spike and wave, increased with photic stimulation.
Tx for Janz
lifelong valporic acid, prognosis is excellent
Childhood absence epilepsy. Peak onset?
6-7 years. F>M. Strong genetic predisposition
Childhood absence epilepsy seizure description
Less than 30 seconds, no post-ictal state, may have multiple per day
Absence seizure EEG
3hz spike and wave
Tx for absence seizure
Valproic acid or ethosuximide
What is the benign focal epilepsy of childhood with centrotemporal spikes onset?
5-10 years of age, 16% of all non-febrile seizures
What are the seizures like in benign focal epilepsy of childhood with centrotemporal spikes?
focal motor seizures invovling tongue, mouth, face, UE usually occuring in sleep-wake transition states
typical EEG for benign focal epilepsy of childhood with centrotemporal spikes?
repeititve spikes in centro temporal area with normal background.
Febrile seizures Epi
3-5% of all children, M>F, age 6 months - 6 years
Types of febrile seizures
Simple and complex
Simple vs complex febrile seizures
Simple:
if febrile seizure lasts >15 minutes, suspect?
Meningitis or toxin
Migraine Epidemiology
4-5% of school children; prevalence F:M = 2:1 after puberty; heterogenous autosomal dominant inheritance
Types of migraines
Common (without aura) - most common in children. a/w N/V, classic (with aura), complicated (basilar, ophthalmoplegic, confusional, hemiplegic)
Migraines clinical features
in infants - irritable, sleepy, pallor, vomiting; young child - periodic headaches with n/v, relieved by rest. Usually unilateral throbbing headaches in kids with photofobia and phonofobia
What is the pharm prophylaxis for migraines?
beta blockers (propanolol), TCAs (amitryptiline), antihistamines, Ca chanel blockkers, anticonvulsants (depakote). Children >12 years can use nasal spray sumitriptan other triptans.
What is the ppt for tension headaches
bilateral pressing tightness anywhere on cranium, or suboccipital regional, usually frontal, hurting or aching quality not throbbing. Usually lasts more than 30minutes can wax and wane and last for days. No N/V not aggravated by physical activity. Most hcildren have insight into origin (poor self image, fear of school failure)
Tx for tension headaches
Reassurance explanation. Supportive counseling. Rule out refractory errors in eyesight as cause of tension
Organic etiology in headaches usually suggested by?
Occipital headache and red flags
Characteristics of increased ICP headaches
Diffuse early morning headaches, early morning vomiting, headache wornsed by increased ICP (cough, sneeze, valsava). As ICP increases, headache is constant. Child irritable and lethargic
What is cerebral palsy
nonprogressive central motor impairment syndrome due to insult or anomaly of the immature CNS. Extent of intellectual impairment varies, presentation of the impairment changes with age
Etiology of CP
association with low birth weight babies, often obscure - no definite cause in 1/3rd cases, only 10% related to intrapartum asphyxia, 10% due to postnatal insults
Types of CP
1) Spastic (70-80% of cases) 2) athetoid/dyskinetic (10-15%) 3) ataxic
What is the clinical ppt of spastic CP
truncal hypotonia in 1st year; increased tone, increased reflexes, clonus. Affects one limb (monoplegia), affects one side of body (hemiplegia), affects both legs (diplegia), affects all limbs (quadriplegia).
What are the brain areas invovled in spastic CP?
UMN of pyramidal tract; diplegia a/w periventricular leukomalaca (PVL) in premature babies; quadriplegia associated with asphyxia
What is the clinical ppt of athetoid/dyskinetic CP? What areas of the brain are involved?
athetosis (involuntary wrihing movements) +/- chorea (involuntary jerking movements). Can involve face and tongue (dysarthria). Basal ganglia (may be associated with kernicterus)
NF-1 incidence
1:3000. A/D
Mutation of NF-1
17q11.2
Dx of NF-1 requires
2 or more of 1) >=6 café au lait spots (>5mm if prepubertal, >1.5cm if post pubertal) 2)>= 2 neurofibromas of any type or one plexiform neurofibroma 3) >= 2 Llsch nodules 4) optic glioma 5) freckling in axillary or groin area 6) distinctive bony lesion like sphenoid dysplasia or cortical thinning of long bones 7) first degree relative with NF-1
NF-2 incidence?
1:33000. A/D
Dx of NF-2
Either bilateral vestibular scwannomas or first degree relative with NF-2 and either a neurofibroma, meningioma, glioma or schwannoma
NF-2 a/w
posterior subcapsular cataracts
Sturge weber syndrome
Port-wine nevus syndrome in V1 distribution with associated angiomatous malformations of brain causing contralateral hemiparesis and hemiatrophy.
Sturge weber a/w
Glaucoma, seizures and mental retardation
Tuberous Sclerosis?
A/D
Findings in TS
Adenoma sebaceum (angiokeratomas on face, often in malar distribution) , shagreen patch (isolated plaque over lower back, buttocks), ash leaf hypopigmentation seen with Woods lamp; cardiac rhabdomyomas, kidney angiomyolipoma, mental retardation and seizures
What brain findings in TS?
Subependymal nodules may evolve into giant cell astrocytomas (may cause obstructive hydrocephalus), cerebral cortex tubers (areas of cerebral dysplasia)
What is acute disseminated encephalomyelitis?
Immune mediated inflammatory disorder of the CNS, widespread demyelination mostly affecting white matter of brain and spinal cord
ADEM usually preceded by
Viral infection or vaccination although no clear precedent event in 26% of cases
What is the median age/onset of ADEM, m/f ratio, and incidence in NA?
5-8 years old, 2:1 male to female ratio and 0.4 per 100,000 children
MRI findings of ADEM?
large multifocal poorly marginated regions of demyelination affecting bilateral subcortical white amtter and deep grey matter (thalamus, basal ganglia).
