Non Compressive Spinal Cord Diseases Flashcards
Non compressive myelopathies?
7
Inflammatory-TM
Demyelination-MS
Vascular disorders- anterior spinal A thrombosis
Vasculitis-SLE
nutritional-vit B12 def
Infections-HIV,syphilis
Degenerative-MND,syringomyelia
Causes of TM?
1.idiopathic
2.infections-EBV,CMV,VZVrabies,mycoplasma
3.post infectious(immune mediated)- influenza,measles,varizella
Features of TM?
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
Ix and Mx of TM?
Ix- MRI,LP(^ wbc)
Tx- steroids ,plasmaparesis ( if autoimmune)
Pathophysiology of MS?
Osmosis
Histology- inflammatory T cells,B cells,macrophages,demyelination amd axonal degeneration = demyelinating plaques
Clinical features of MS?
-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
Types of MS?
4
RRMS-most common
Primary progressive MS
SPMS
progressive relapsing MS
Diagnosis of MS?
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)
Tx of MS?
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
Prognosis and dermographics?
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
Blood supply of SC and clinical features of anterior spinal A thrombosis?
-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
Pathophysiology and other features of subacute combined degeneration of thr cord (vit B12 def)?
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
Clinical features of SACD (subacute combined cord degeneration )
5
Exaggerated knee jerk (UMN)
Absent ankle jerk (peripheral N)
Extensor planter response (UMN)
Stocking sensory loss (PN)
Loss of proprioception (dorsal column)
How to Ix a pt with a cord xd?
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
What is MND?
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
Classification of MND?
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)
Clinical features of ALS?
-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
Dx of ALS?
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
Ix and Mx of MND?
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
Distal spinal muscular atrophy?
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
