Neuro 3 pt2 Flashcards Preview

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Flashcards in Neuro 3 pt2 Deck (19)
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1
Q

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

A

Adult Tay-Sachs Dz

2
Q

What clearly differs Adult Tay-Sachs Dz from ALS?

A

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

3
Q

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

A

Spinal Muscular Atrophy

4
Q

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

A

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

5
Q

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

A

Multifocal Motor Neuropathy with Conduction Block

6
Q

What distinguishes Multifocal Motor Neuropathy with Conduction Block from ALS?

A

it may respond to IVIG or chemo

7
Q

Fasiculations, amyotrophy, sensory changes absent

Rapid course to death (3yrs) as in ALS

A

Primary Lateral Sclerosis (PLS)

8
Q

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

A

Familial Spastic Paraplegia

9
Q

How does Familial Spastic Paraplegia spread in the body?

A

bottom -> up

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

10
Q

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

A

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)

11
Q

What is the most specific motor activity affected by MS?

A

Bladder is affected >90%

12
Q

MS patient’s voice will sound:

A

Scanning speech aka Cerebellar speech (robotic voice)

13
Q
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)
A

Optic Neuritis

14
Q

MS Testing

A

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

15
Q

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

A

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

16
Q

What does CIS MS stand for?

What is it?

A

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

17
Q

CIS sx (5)

A

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

18
Q

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

A

Optic neuritis

19
Q

Tx of MS

A
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