neuro8

Question 1

vermal lesions

Answer 1

prominent truncal and gait ataxia

Question 2

cerebellar hemispheric lesions

Answer 2

ipsilateral limb ataxia

Question 3

cerebellar hemorrhage or infarction presentation

Answer 3

abrupt onset of vertigo, vomiting, and inability to walk

Question 4

alcoholic cerebellar degeneration

Answer 4

consequence of longstanding alcohol abuse; vermis bears the brunt of damage; progressive gait and truncal ataxia that evolves over a period of weeks or months

Question 5

postinfectious cerebellitis

Answer 5

kids between ages 2 and 7; usually follows varicella or other viral infx; acute onset of limb and gait ataxia and dysarthria; lasts a few weeks and full recovery

Question 6

paraneoplastic cerebellar degeneration

Answer 6

pancerebellar; usually evolves over a period of weeks and thenstabilizes, leaving the patient with profound disability; typically assoc with gyn or SCLC and may manifest prior to dx of tumor

Question 7

MRI in paraneoplastic cerebellar degeneration

Answer 7

normal

Question 8

CSF in paraneoplastic cerebellar degeneration

Answer 8

may have an elevated protein or a lymphocytic pleocytosis, but is frequently normal; sometimes there are autoantibodies anti-Yo and anti-Hu

Question 9

freidrich ataxia

Answer 9

aut recessive; progressive ataxia that usually affects the arms more than the legs; severe dysarthria; onset usuallyin childhood; classicly loss of reflexes, spasticity, and extensor plantar responses

Question 10

inherited episodic ataxia

Answer 10

brief episodes of ataxia, vertigo, and n/v

Question 11

EA1

Answer 11

cuased by mutations in a voltage gated potassium channel; episodes are brief and an interattack skeletal muscle myokymia is associated

Question 12

EA2

Answer 12

mutations in the pore-forming alpha 1 subunit of the P/Qtype voltage gated calcium channel; attacks are longer, lasting a few mins; interictal nystagmus; progressive irrev ataxia may develop late in the disease

Question 13

aut dom spinocerebellar degenerations

Answer 13

cerebellar ataxia with ot without additions neuro signs and a fam hx consisten with aut dom inheritance; mild cognitive decline is often a late feature

Question 14

many spinocerebelar ataxias for which the genetic defect has been identified are caused by what

Answer 14

CAG trinucleotide repeats

Question 15

Miller Fisher syndrome

Answer 15

triad of ataxia (due to proprioceptive loss), areflexia, and ophthalmoplegia; variant of guillan barre; IgG anti-GQ1b antibodies in the serum; full recovery

Question 16

hemiparetic gait

Answer 16

affected leg is stuff and does not flex at the hip, knee, or ankle; leg is circumducted; arm is flexed and adducted and does not swing freely

Question 17

spastic (paraparetic) gait

Answer 17

essentially a bilateral hemiparesis; adductor tone is increased and the legs tend to cross during walking (scissoring gait)

Question 18

akinetic-rigid gait

Answer 18

posture is stooped, with flex of the shoulders, neck and trunk; gait is narrow based, slow, and shuffling with small steps and reduced arm movement

Question 19

gait initiation difficulty

Answer 19

seen in akinetic rigid gait

Question 20

festination

Answer 20

rapid small steps

Question 21

frontal gait

Answer 21

posture is flexed, and the feet may be slighly apart; gait initiation is impaired; advances with small, shuffling, and hesistant steps; abortive stepping movements without being able to move forward

Question 22

waddling gait

Answer 22

hip-gurdle weakness; pelvis and trunk tilt toward the opposite side during walking

Question 23

psychogenic gait

Answer 23

range of presentations;

Question 24

hemiparetic gait

Answer 24

suggests hemispheric dysfunction, most often stroke

Question 25

paraparetic gait

Answer 25

suggests spinal cord disease

Question 26

frontal gait suggests what kind of cause?

Answer 26

hydrocephalus, a neurodegenerative process, or bifrontal or diffuse subcortical disease

Question 27

slapping gait

Answer 27

indicates large fiber sensory or dorsal column dysfunction

Question 28

other symptoms assoc with freidrich ataxia

Answer 28

hearing loss, glucose intolerance/DM, hypertrophic cardiomyopathy, cardiac arrythmias