Flashcards in Neuromuscular Deck (39):
Basilar skull fracture signs/mgmt
Otorrhea, rhinorehea, battle sign. Observe for 72 hours, at which point leak and infection risk should be over.
Pin point pupils- causes
Coma, narcotics, pilocarpine treatment, Horner syndrome, pontine hemorrhage, pesticide, nerve gas, tertiary syphilis.
Dilated pupils - causes
Epinephrine, sympathomimetics, antihistamines, atropine
Paediatric stroke- some signs and risks
Vomiting, lethargy, seizures, headache, focal weakness. Often precipitated by infection or dehydration.
Risks: blood disorders (factor V Leiden), chd, malignancy, nephrotic syndrome, anti-phospholipid Ig ...etc
Anti-AChR Ig. Presents with progressive weakness, ptosis early on.
Normal conduction velocity. Tiring EMG. Reversed with anti-AChE.
Tay Sachs disease
Auto rec. Progressive neuro degeneration and developmental delay. Macular cherry red spot. Phonophobia. Lysosomal storage disorder with substrate accumulating in and killing neurons. Various forms, classic infantile fatal by 4 yrs. Ashkenazi risk.
Auto rec. no conversion of phenylalanine to tyrosine, with toxic metabolite build up. Seizures, delay. Strict dietary control.
Methionine met. Disorders. Auto recessive. Homocysteinemia associated with stroke. Homocystinuria: retardation, ectopic lentis, marfanoid, stroke.
Congenital muscular dystrophies
Auto recessive. Ie. duchenne and Fukuyama. Variable progression: resp insufficiency, limb weakness, bulbar weakness, contractures, seizures, retardation. Poor sucking. Decreased movements in uterine
Duchenne muscular dystrophy
Xlinked. First seen as a waddling gait 2-6 years (hip weakness). Dx: serum cpk levels, spine X-ray, EMG, muscle biopsy.
1) weakness onset in leg 2) hyperlordosis/wide gait 3) hypertrophy 4) progressive 5) reduced EMG 6) no fever, bowel or bladder sx
Some ddx for an abnormal sacral spine area. (Dimple, hair, tumor, etc)
Tethered cord, diastematomyelia (split cord), lipomeningomyelocoele, dermal sinus tracts, dermoids,, cystocoele
Myotonic muscular dystrophy
Most common dystrophy of adults . Auto dominant. Multisystem. Cataracts, clumsy, waddling gait, myotonia , progressive weakness, sudden cardiac death.
Migraines in kids vs adults
Adults: throbbing, unilateral, moderate to severe, avoidance/reduced activity. With N/V or photo/ phonophonia. 4 hrs to 3 days.
Kid: bifrontal, few hours. No aura, not diffuse throbbing.
Both: fm hx terminate:sleep vomit.
Headache red flags
Fever, thunderclap, eye pain, jaw pain, focal neuro sx, neck stiffness, worse in AM, wakes person, changed LOC
Disseminated infection to lymph nodes and beyond gets to brain /meninges. 1) form rich foci (subpial/ependymal caseous lesions) 2) rupture:SAH. Poor prognosis - stroke, hypercoaguable).
Ct, MRI, spinal tap. Serum CBC glucose. Lymphocytic pleocytosis.
Headache/n/v/photophobia/papilledema/ fever = prodrome.
2 subtentorial tumors
Medullablastoma, cerebellar astrocytoma.
Febrile seizure causes
Roseola, URTI, gastroenteritis, shigellosis. 2-5 min generalized seizure, normal CSF.
Idiopathic intracranial hypertension
Aka pseudo tumor cerebri. Papilledema and chronic high ICP. Headache, Diplopia, pulsatilla tinnitus, can have radicular pain. Usually affects obese women of child bearing age. Complications to optic nerve: blindness.
Infectious, post infectious, metabolic/toxins, hydrocephalus, tumor, cerebellar lesion, MS, inner ear, neuroblastoma (opsoclonus myoclonus syndrome).
30% of ped tumours. PNET. Likes to metastasize within CNS, can also met systemically. Complications: jydrocephalus(major),
Can be associated with Turcott and Ataxia telangectasia syndromes.
Due to mutation causing vascular malformations all over the body. Ash Leaf lesions, Shagreen lesions, sebaceous adenomas, infantile spasms, seizures, cardiac tumors, mental retardation, calcification on CT. Autosomal dominant.
McCune Albright Syndrome
Affects skin, bones and endocrine. Large irregular and unilateral cafe au lait spots, fibrous bony dysplasia, precocious puberty.
Two ticks that cause paralysis
Rocky mountain wood tick, eastern dog tick. Starts as ataxia, then loss of reflexes then ascending paralysis. No fever. Remove the tic!
Neurofibromatosis Type I
The most frequent of hamartoses. Gene deletion of NF1 affecting cutaneous, bony and neurologic. Associated with neurofibromas and cafe au lait spots.
Diagnostic criteria for NF1
5+ cafe au lait spots, axillary freckling, 2+ lisch nodules (iris hamartomas), 2+ neurofibromas or 1 plexiniform neuroma, bony lesions, optic glioma, affected 1st degree relative.
Achromic skin patches and seizures....
infection causing cerebral (not periventricular seizures)
Guillan Barre Syndrome
Most important cause of acute flaccid paralysis outside of polio. Demyelinating neuropathy with ascending weakness. Post infectious and immune mediated against peripheral nerves.
Ascending, symmetric. Affects legs then arms, trunk, resp, cranial nerves. Sensory changes often precede weakness.
Dx: mostly clinical. high CSF protein. Screen to exclude other ddx. nerve conductino helpful.
Agents implicated in GBS
Campylobacter jejuni and URTI's
DDx for GBS
HIV neuropathy, Tick paralysis, shellfish poisoning, vitamin B12, folate or thiamine deficiency, toxic neuropathies, porpyria, polio,
GBS treatment and natural history
Monitor in hospital. IVIG, steroids, plasmaphoresis. Peak of symptoms is around day 12. Recovery starts 2-4 weeks after that. Mean recovery 200 days.
non-rhythmic, spasmodic, involuntary, stereotyped. Suppressible.
transient tic disorder
boys usually. OFten a family history. Lasts around 1 yr.
Gilles de la Tourette
ADHD, Tics, OCD
Spinal muscular atrophy
Clue: hypotonia and difficulty with feeds at birth.
Borrellia Burgdorferi ( Deer tick). Within 1 wk, flu like sx and erythema migrans (90%). 50% of patients will get dissiminated disease with neurologic, muscular and cardiac symptoms (heart block). Can also present with a migratory polyarthritis.
Argyl Robertson pupil
Prostitutes pupil. Bilateral small, accommodates (constricts) but not reactive to light. Highly specific for neurosyphilis.
Marcus gunn pupil
Relative afferent pupillary defect