Neuromuscular

Question 0

Pin point pupils- causes

Answer 0

Coma, narcotics, pilocarpine treatment, Horner syndrome, pontine hemorrhage, pesticide, nerve gas, tertiary syphilis.

Question 1

Basilar skull fracture signs/mgmt

Answer 1

Otorrhea, rhinorehea, battle sign. Observe for 72 hours, at which point leak and infection risk should be over.

Question 2

Dilated pupils - causes

Answer 2

Epinephrine, sympathomimetics, antihistamines, atropine

Question 3

Paediatric stroke- some signs and risks

Answer 3

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

Question 4

Myasthenia Gravis

Answer 4

Anti-AChR Ig. Presents with progressive weakness, ptosis early on.
Normal conduction velocity. Tiring EMG. Reversed with anti-AChE.

Question 5

Tay Sachs disease

Answer 5

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.

Question 6

Phenylketonuria

Answer 6

Auto rec. no conversion of phenylalanine to tyrosine, with toxic metabolite build up. Seizures, delay. Strict dietary control.

Question 7

Homocysteinuria/homocysteinemia

Answer 7

Methionine met. Disorders. Auto recessive. Homocysteinemia associated with stroke. Homocystinuria: retardation, ectopic lentis, marfanoid, stroke.

Question 8

Congenital muscular dystrophies

Answer 8

Auto recessive. Ie. duchenne and Fukuyama. Variable progression: resp insufficiency, limb weakness, bulbar weakness, contractures, seizures, retardation. Poor sucking. Decreased movements in uterine

Question 9

Duchenne muscular dystrophy

Answer 9

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

Question 10

Some ddx for an abnormal sacral spine area. (Dimple, hair, tumor, etc)

Answer 10

Tethered cord, diastematomyelia (split cord), lipomeningomyelocoele, dermal sinus tracts, dermoids,, cystocoele

Question 11

Myotonic muscular dystrophy

Answer 11

Most common dystrophy of adults . Auto dominant. Multisystem. Cataracts, clumsy, waddling gait, myotonia , progressive weakness, sudden cardiac death.

Question 12

Migraines in kids vs adults

Answer 12

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.

Question 13

Headache red flags

Answer 13

Fever, thunderclap, eye pain, jaw pain, focal neuro sx, neck stiffness, worse in AM, wakes person, changed LOC

Question 14

Tuberculous meningitis

Answer 14

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.

Question 15

2 subtentorial tumors

Answer 15

Medullablastoma, cerebellar astrocytoma.

Question 16

Febrile seizure causes

Answer 16

Roseola, URTI, gastroenteritis, shigellosis. 2-5 min generalized seizure, normal CSF.

Question 17

Idiopathic intracranial hypertension

Answer 17

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.

Question 18

Ataxia ddx

Answer 18

Infectious, post infectious, metabolic/toxins, hydrocephalus, tumor, cerebellar lesion, MS, inner ear, neuroblastoma (opsoclonus myoclonus syndrome).

Question 20

Medulloblastoma

Answer 20

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.

Question 21

Tuberous Sclerosis

Answer 21

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.

Question 22

McCune Albright Syndrome

Answer 22

Affects skin, bones and endocrine. Large irregular and unilateral cafe au lait spots, fibrous bony dysplasia, precocious puberty.

Question 23

Two ticks that cause paralysis

Answer 23

Rocky mountain wood tick, eastern dog tick. Starts as ataxia, then loss of reflexes then ascending paralysis. No fever. Remove the tic!

Question 24

Neurofibromatosis Type I

Answer 24

The most frequent of hamartoses. Gene deletion of NF1 affecting cutaneous, bony and neurologic. Associated with neurofibromas and cafe au lait spots.

Question 25

Diagnostic criteria for NF1

Answer 25

2/7 of:
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.

Question 26

Achromic skin patches and seizures….

Answer 26

tuberous sclerosis

Question 27

infection causing cerebral (not periventricular seizures)

Answer 27

congenital Toxoplasmosis.

Question 28

Guillan Barre Syndrome

Answer 28

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.

Question 29

Agents implicated in GBS

Answer 29

Campylobacter jejuni and URTI’s

Question 30

DDx for GBS

Answer 30

HIV neuropathy, Tick paralysis, shellfish poisoning, vitamin B12, folate or thiamine deficiency, toxic neuropathies, porpyria, polio,

Question 31

GBS treatment and natural history

Answer 31

Monitor in hospital. IVIG, steroids, plasmaphoresis. Peak of symptoms is around day 12. Recovery starts 2-4 weeks after that. Mean recovery 200 days.

Question 32

tic definition

Answer 32

non-rhythmic, spasmodic, involuntary, stereotyped. Suppressible.

Question 33

transient tic disorder

Answer 33

boys usually. OFten a family history. Lasts around 1 yr.

Question 34

Gilles de la Tourette

Answer 34

ADHD, Tics, OCD

Question 35

Spinal muscular atrophy

Answer 35

Clue: hypotonia and difficulty with feeds at birth.

Question 36

Lyme disease

Answer 36

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.

Question 36

Argyl Robertson pupil

Answer 36

Prostitutes pupil. Bilateral small, accommodates (constricts) but not reactive to light. Highly specific for neurosyphilis.

Question 37

Marcus gunn pupil

Answer 37

Relative afferent pupillary defect

Question 38

Red flags for child with limp

Answer 38

Wt loss, fever, chills, night sweats,
Refusal to wt bear, lethargic, ill.

Night pain. Morning stiffness.

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