spinal cord lesions

Question 1

describe lesion in polio and spinal muscular atrophy/werdnig Hoffmann disease

Answer 1

anterior horn

LMN lesion - flaccid paralysis

Question 2

describe lesions in multiple sclerosis

Answer 2

mostly white matter of the cervical region

due to demyelination (oligodendrocytes)

Question 3

presentation of multiple sclerosis

Answer 3

scanning speech
intention tremor
nystagmus

Question 4

scanning speech
intention tremor
nystagmus

Answer 4

multiple sclerosis

Question 5

describe lesion in amyotrophic lateral sclerosis

Answer 5

combined UMN and LMN
no sensory
no oculomtor defects

Question 6

what genetic associated with amyotrophic lateral sclerosis

Answer 6

superoxide dismutase 1

Question 7

presentation of amyotrophic lateral sclerosis pelase

Answer 7

combined UMN and LMN signs
commonply presents as fasciculations with eventual atrophy and weakness of hands
fatal

Question 8

what is used to help with amyotrophic lateral sclerosis pelase

Answer 8

RILUZOLE modestly increases survival by decreasing presynaptic glutamate release

Question 9

what is RILUZOLE

Answer 9

for amyotrophic lateral sclerosis - decreases presynaptic glutamate release - results in modest increase in survival
riLUzole for LOU gehrigs disease

Question 10

describe lesion I ncomplete occlusion of anterior spiral arter

Answer 10

affects all but the dorsla colomns and lissauers tract

Question 11

what region of the spc is typically spared from anterior spinal artery occlusion

Answer 11

below T8 is supplied by the artery of Adamkiewicz so above T8 is more common to see bc of watershed vulnerability between the anterior and posterior spinal arteries

Question 12

describe tabes dorasalis

Answer 12

caused by tertiary syphilis - degeneration/demyelination of dorsal colomns and roots

Question 13

presentation to tabes dorsalis pelase

Answer 13

degeneration/demyelination fo dorsal colomns and roots
impaired sensation and proprioception – progressive sensory ataxia due to inability to sense and feel legs – poor coordiation

Question 14

what is tabes dorsalis associated with

Answer 14

charcot joints
shooting pain
Argyll Robertson pupils - accommodating but not reactive

Question 15

physical exam with tabes dorsalis please

Answer 15

no DTR

positive rhomberg test

Question 16

lesion in syringomyelia

Answer 16

syrinx expands from central canal and damages anterior white commissure - bilateral loss fo pain and temperature

Question 17

where to syringomyelias usually occur

Answer 17

C8-T1

Question 18

what posterior fossa malformation are syringomyelias associated with

Answer 18

chiari I - usually asmtpmoatic

can have headaches and cerebellar symptoms + synromgomyelia effects

Question 19

what happens in vitam B12 deficiency

Answer 19

subacute combined degeneration
demyelination of 
a) dorsal colomns
b) lateral corticospinal tracts
c) spinocerebellar tracts

Question 20

presentation fo vitamin B 12deficiency

Answer 20

dorsal colomn demyelination: paresthesia, impaired position and brintaion sense
demyelination of lateral corticospinal tracts: fa doesn’t say, maybe paresthesias
spinocerebellar demyelinagion: ataxic gait

Question 21

what causes polio

Answer 21

fecal oral spread of the polio virus

Question 22

pathogenesis of polio please

Answer 22

replicates int eh orophyarnx and small intestine before spreading through the bloodstream to the CNS – infection auses desetruction of the anterior horn fo spinal cord LMN death

Question 23

symptoms of polio please

Answer 23

LMN lesion signs: weakness, hypotonia, flaccid paralysis, fasciculations, hyporeflexia, muscle atrophy
signs of infection: malaise, headache, fever, nausea

Question 24

clinical findinfs in polio please

Answer 24

CSF with increased wbc, slight increase protein, no change in glucose
cirus can be recovered from stool o rthroat

Question 25

what is spinal muscular atrophy/werdnig Hoffmann disease

Answer 25

congenital degeneration of anterior horns of spinal cord – LMN lesion
FLOPPY BABY with marked hypotonia and tongue fasciculations
median age of death is 7 months
AUTOSOMAL RECESSIVE

Question 26

floppy baby with marked hypotonia and tongue fasciculations

Answer 26

spinal muscular atrophy
Werdnig Hoffman disease
AR recessive degeneration fo anterior horn cells

Question 27

describe genetics of freidrich ataxia please

Answer 27

AR trinucleotide repeat disorder of GAA on chromosome 9 for FRATAXIN, an iron binding protein – impairment of mitochondrial function

Question 28

pathophysiology of friedrich ataxia please

Answer 28

frataxin wanky, an iron binding protein - impaired mitochondrial function - defentration of multiple spinal cord tracts - muscle weakness, loss of DTRs, vibratory sense, proprioception

Question 29

clinical presentation fo friedrick ataxia please

Answer 29

staggering gait
frequent falling
nystagmus
dysarthria
pes cavus (foot high arch)
hammer toes
diabetes mellitus 
hypertrophyic cardiomyopathy
@ childhood with kyphoscoliosis

Question 30

complications of friedrich ataxia

Answer 30

diabetes mellitus, hypertrophic cardiomyopathy

kyphosis in childhood

Question 31

what is major cause of death in friedrich ataxia

Answer 31

presents in childhood with kyphoscoliosis

Question 32

what is brown sequard syndrome

Answer 32

hemisectino fo the spinal cord

Question 33

describe clinical presentation fo brown sequard plsease

Answer 33

ipsilateral UMN signs below level of lesion - corticospinal tract damage
ipsilatera loss fo tactile vibration and proprioception two below - lissaurs tract lesion
contralateral pain and temp loss below level of lesion - spinothalamic tract damage
ipsilateral loss of all senation at the level of the lesion
ispilatear LMN at the level of the lesion

Question 34

what addition symptom to expect if brown sequard occurs above T1

Answer 34

horner syndrome due to damage of oculosympahtetic pathway