Neuro 3 pt2

Question 1

characterized by clumsiness in childhood, progressive motor weakness in adolescence, and spinocerebellar, LMN symptoms, and adulthood dysarthria
Slow decline in intelligence c psychosis common
atrophy of cerebellum seen on MRI

Answer 1

Adult Tay-Sachs Dz

Question 2

What clearly differs Adult Tay-Sachs Dz from ALS?

Answer 2

atrophy of cerebellum seen on MRI —you won’t see this with ALS.

Question 3

Most are early onset “floppy babies”
LMN Dz
loss of large motor neurons; muscle bx shows denervation atrophy

Answer 3

Spinal Muscular Atrophy

Question 4

What helps distinguish Spinal Muscular Atrophy from ALS AND Tay-Sachs?

Answer 4

the young age of early onset here w/ Spinal Muscular Atrophy

Question 5

LMN Dz
Focal blocks of conduction caused by auto-antibodies causing focal demyelination
Not typically assoc c corticospinal signs.

Answer 5

Multifocal Motor Neuropathy with Conduction Block

Question 6

What distinguishes Multifocal Motor Neuropathy with Conduction Block from ALS?

Answer 6

it may respond to IVIG or chemo

Question 7

Fasiculations, amyotrophy, sensory changes absent

Rapid course to death (3yrs) as in ALS

Answer 7

Primary Lateral Sclerosis (PLS)

Question 8

Symptoms of progressive spastic weakness starting in distal lower extremities
Long survival because respiration is spared

Answer 8

Familial Spastic Paraplegia

Question 9

How does Familial Spastic Paraplegia spread in the body?

Answer 9

bottom -> up

Rises up from the legs, but never gets to the brain. Definitely makes it up into the spinal cord though.

Question 10

Define these presentations to dx Multiple Sclerosis:
L’hermitte’s sign
Internuclear opthalmoplegia
Uhtoff’s phemena

Answer 10

L’hermitte’s sign – cervical cord problem – when they flex their neck they feel electric shocks down the apine
Internuclear ophthalmoplegia – when you test extraocular movements, the abducting eye will have horizontal nystagmus.
Uhtoff’s phenomena(excercise or incr body temp worsens symptoms)

Question 11

What is the most specific motor activity affected by MS?

Answer 11

Bladder is affected >90%

Question 12

MS patient’s voice will sound:

Answer 12

Scanning speech aka Cerebellar speech (robotic voice)

Question 13

decreasing visual acuity
decr. color perception centrally
usu. monocular
periorbital pain from EOM 
Afferent pupillary defect usu. present (Marcus Gunn pupil – put a direct ligiht source on pupil and pupils DILATE)

Answer 13

Optic Neuritis

Question 14

MS Testing

Answer 14

MRI shows Dawson’s Fingers are oval lesions perpendicular to ventricle

Question 15

McDonald Criteria to dx MS (hint: it has to be MULTIPLE)

Answer 15

Dissemination in Space
Dissemination in Time
2 separate areas at least for one month to be ‘multiple’

Question 16

What does CIS MS stand for?

What is it?

Answer 16

Clinically Isolated Syndrome of MS
Single attack compatible c MS, such as optic neuritis
80% CIS pts. & MRI lesion develop MS, but 20% are self-limiting

Question 17

CIS sx (5)

Answer 17

Motor: Weakness of LE > UE, spasticity, hyperreflexia, +babinski
Speech can be scanning

Question 18

A) Fat-sat T2-weighted coronal shows hyperintensity in R optic nerve (arrow)
B) Post gado fat-sat T1-weighted cornoal showing abnl enhancement R optic n. (arrow)
4 yrs later, sag FLAIR shows two plaques radiating outward from corpus callosum (arrowheads) suggestive of MS

Answer 18

Optic neuritis

Question 19

Tx of MS

Answer 19

corticosteriods used for acute flare-ups with plasmaphoresis reserved for nonresponders
antineoplastics- SC qd, suppress T&B cells, 
monoclonal antibody (immunomodulator) designed to hamper potentially damaging immune cells from crossing BBB