Non Compressive Spinal Cord Diseases

Question 1

Non compressive myelopathies?

Answer 1

7
Inflammatory-TM
Demyelination-MS
Vascular disorders- anterior spinal A thrombosis
Vasculitis-SLE
nutritional-vit B12 def
Infections-HIV,syphilis
Degenerative-MND,syringomyelia

Question 2

Causes of TM?

Answer 2

1.idiopathic
2.infections-EBV,CMV,VZVrabies,mycoplasma
3.post infectious(immune mediated)- influenza,measles,varizella

Question 3

Features of TM?

Answer 3

5
Acute inflammation of the cord
Usually restricted to 1-2 seg of the cord
Symptoms over few hours
Max deficit usually within 24 hrs
Loss of motor,sensory and sphincter function below the lesion

Question 4

Ix and Mx of TM?

Answer 4

Ix- MRI,LP(^ wbc)
Tx- steroids ,plasmaparesis ( if autoimmune)

Question 5

Pathophysiology of MS?

Answer 5

Osmosis
Histology- inflammatory T cells,B cells,macrophages,demyelination amd axonal degeneration = demyelinating plaques

Question 6

Clinical features of MS?

Answer 6

-Multifocal dessiminated demyelination in CNS, disseminated in time and space
-symptoms depends on location of the lesion
Optic N-optic neutitis
Brain stem-CN palsy
Cerebellum-ataxia and incoordination
Spinal cord-TM
-other features
Charcot’s neurological triad (dysathria,nystagmus,intentional tremors)
Numbness and paresthesia
Bowel and bladder symptoms
Poor concentration,critical thinking,depression,anxiety

Question 7

Types of MS?

Answer 7

4
RRMS-most common
Primary progressive MS
SPMS
progressive relapsing MS

Question 8

Diagnosis of MS?

Answer 8

4
1.demonstrate dissemination in time and space either clinically or on MRI
2.MRI brain -plaques in >90%
3.CSF-oligo-clonal band (high kevels of ab)
4.VEP visual evoke potential (measure response to visual stimuli in optic neuritis)

Question 9

Tx of MS?

Answer 9

2
Acute demyelination-high dose IV steroids
Disease modifying therapy (long term)-interferon beta 1a or 1b (decrease release of cytokines and ^ regulatory T cells),immunosuppressive drugs

Question 10

Prognosis and dermographics?

Answer 10

Cumulative disability within 20 yrs of onset
50% require ambulatory therapy
F:M 2:1
Peak incidence -30 and 40
Incidence ^ with ^ latitude
Related to vit D def

Question 11

Blood supply of SC and clinical features of anterior spinal A thrombosis?

Answer 11

-A 2/3 by A spinal A
P 1/3 by P spinal A
-Acute onset (hallmark of vascular events)
-dif from TM by spareing posterior column

Question 12

Pathophysiology and other features of subacute combined degeneration of thr cord (vit B12 def)?

Answer 12

Vit B12 is a coenzyme for transmethylation in DNA synthesis and myelin synthesis
Affect dorsal column and lateral white colums,peripheral N

-macrocytic megaloblastic anemia
-dementia
-optic atrophy

Question 13

Clinical features of SACD (subacute combined cord degeneration )

Answer 13

5
Exaggerated knee jerk (UMN)
Absent ankle jerk (peripheral N)
Extensor planter response (UMN)
Stocking sensory loss (PN)
Loss of proprioception (dorsal column)

Question 14

How to Ix a pt with a cord xd?

Answer 14

1.exclude compressive lesion with MRI of the relevant area first
2.if MRI N,
-CSF Ex
-virus screen for TM
-MRI brain if MS suspect
-vasculitis screen (ESR,CRP,ANA)
-HIV ,VDRL
-vit B12 levels,FBC,blood picture
-neurophysiology

Question 15

What is MND?

Answer 15

Selective loss of function of the motor neurones innervating voluntary muscles
-limbs
-bulbar
-respiratory muscles

No sensory signs
Bladder and bowel not affected
Extrapyramidal signs are absent
Cerebellar signs do not occur
Occular muscles not affected
Cognitive functions are usually normal

Question 16

Classification of MND?

Answer 16

1.combined UMN N LMN
-amyotrophic lateral sclerosis (ALS)
2.pure LMN
-acute infantile form (werdnig hoffman) AR
-chronic childhood (kugelberg welander) AR
-distal spinal muscular atrophy (DSMA)
3.pure LMN
-primary lateral sclerosis (PLS)

Question 17

Clinical features of ALS?

Answer 17

-Commonest form of MND
-Median age of onset 60 yrs
-largely sporadic
-5%-AD
-neurodegenerative disease( progressive loss of motor neurons)
-resentlessly progressive

-early symptoms- cramps ,fatigue
-wasting (lmn)
-weakness (lmn)
-fasciculation (lmn) not confined to wasted muscles
-exaggerated tendon reflexes (umn)

Bulbar involvement
-dysphagia
-dysarthria
-wasted tongue with fasciculations
-spastic tongue with fasciculations
-brisk jaw jerk
-emotional lability

Question 18

Dx of ALS?

Answer 18

1.clinical and electrophysiological demonstration of UMN and LMN signs in bulbar,arm and leg muscles
2.notorious progressive
3.exclusion of other causes for UMN + LMN lesiond
-compressive lesions in yhr spinal cord
-cervical spondylitic tadiculomyelopathy
-tumours
-Syringomyelia

Question 19

Ix and Mx of MND?

Answer 19

Ix- 2
EMG (degenerative changes,not confined to clinically affected muscles)
MRI cervical spine (to exclude treatable causes)

Mx- 5
Breaking bad news
Nutrition (NG OR PEG)
ventilatory support (non invasive)
End of life decisions
Riluzole

Question 20

Distal spinal muscular atrophy?

Answer 20

6
Sporadic or familial (AD or AR) 1:1
Age 15-25
Predominately male
Wasting and weakness of the arms or legs (wont go beyond elbows)
UL onset or asymmetrical
spontaneous arrest within 3-7 yrs