Neurology Flashcards
What type of genetic disorder is Huntington disease?
Autosomal dominant
What is the patho of Huntington disease?
trinucleotide repeats (CAG/glutamine) of the Huntington gene (chromo 4)
What age does Huntington disease usually appear?
30-50yr
What are the 3 classic signs of Huntington disease?
progressive chorea, rigidity, and dementia, frequently associated with seizures.
How is Huntington dx diagnosed?
sx + family hx+ genetic confirmation
What is seen on imaging for Huntington dx?
cerebral & striatal (caudate nucleus & putamen) atrophy
Txt for Huntington dx?
None, fatal in 15-20yr, genetic counseling
1st FDA approved txt for chorea associated with Huntington dx?
Tetrabenazine
MC cause of primary headaches?
tension headaches
Bilateral, pressing, tightening “band like” non-throbbing headache?
tension headache
What is seen on PE for tension headaches?
pericranial muscle tenderness to the head, neck, or shoulders
Tx for tension headache?
NSAIDS
Dx of tension headaches?
clinical
severe, unilateral periorbital or temporal pain headache?
cluster headache
What can trigger a cluster headache?
alcohol, stress, specific foods
When are cluster headaches worse?
at night
Horner’s syndrome?
ptosis, miosis, anhidrosis
What is seen on PE for cluster headaches?
horner’s syndrome, nasal congestion, rhinorrhea, conjunctivitis, lacrimation
1st line txt for cluster headache?
100% oxygen followed by anti-migraine meds
1st line prophylaxis for cluster headaches?
Verapamil
What is the MC type of migraine?
migraine without aura
What type of focal neurologic symptoms is the most common type during a migraine?
visual
When should a CT be used to dx migraine?
- onset of migraine > 50y
- first or worse severe headache
- new daily, persistent headache
1st line txt for migraine?
NSAIDS, Acetaminophen, Aspirin
What can be added to improve meds during migraines?
caffeine
2nd line txt for migraines?
Triptans or Ergotamines
Prophylactic txt for migraines?
anti-hypertensives = BB and CCB
C/I to triptans?
ischemic stroke, heart dx, uncontrolled hypertension, pregnancy
Adv event of triptans?
chest tightness from vasoconstriction
Adv event of ergotamines?
rebound headache
Used for abortive migraine therapy?
IV Metoclopramide
What nerve does bell palsy affect?
VII
What is bell palsy closely related too?
Herpes simplex virus reactivation
What sx are seen in bell palsy?
Ear pain, unilateral facial weakness, taste disturbance, inability to completely close eyelid
Txt for Bell palsy?
Supportive- eye drops
Prednisone
Acyclovir
What txt for bell palsy should be given within the first 72hrs to decrease recovery time?
Prednisone
When used in combination can in severe cases can improve symptoms and timing of recovery of bell palsy?
Acyclovir and glucocorticoids
In bell palsy weakness and paralysis only affects?
The face
What is the MC cause of Gillian barre?
Campylobacter jejuni
What is gullain barre syndrome?
Demyelination polyradiculopathy of the peripheral nervous system
Sx of guillain barre?
Symmetric ascending weakness and sensory changes
What is a life threatening symptom of guillain barre?
Weakness of respiratory muscles
Sx of guillan barre?
Nerve conduction studies
CSF analysis
What is seen on CSF for Guillain barre?
High protein
Normal WBC
Normal glucose
Txt for Guillain Barre?
Plasmapheresis or IVIG
What txt should not be used for Guillan Barre?
Prednisone
What is the prognosis for Guillan Barre?
60% full recovery in 1yr
What is MS?
autoimmune, inflammatory demyelinating disease of the CNS
What axon degeneration does MS affect?
white matter (brain & spinal cord)
What type of MS is the MC?
relapsing-remitting
What is the MC presenting symptoms of MS?
weakness and visual disturbances
What visual disturbances are seen in MS?
diplopia, optic neuritic
What is Uhthoff’s phenomenon?
worsening of symptoms w/ heat
What is Lhermitte’s sign?
neck flexion causes lightning shock type pain radiating from the spine down the leg
The best initial and accurate test for MS?
MRI w/ gadolinium (contrast)
What proof is needed to dx MS?
2 areas of white matter involvement
If MRI is inconclusive what other test can be used to dx MS?
Lumbar puncture
What is seen on lumbar puncture for MS?
increased IgG & oligoclonal bands
1st line txt for MS?
IV glucocorticoids
Prevention of MS?
Beta-interferon or Glatiramer
What vitamin deficiency puts you at risk for MS?
Vitamin D
Patho of Myasthenia gravis?
autoantibodies against acetylcholine postsynaptic receptor at the neuromuscular junction
2 main symptoms of Myasthenia gravis?
ocular weakness
generalized weakness