CONGENITAL PATHOGENESIS

Spina bifida occulta

Meningocele

Meningomyelocele

Cystic cavity (syrinx) within central canal of spinal cord resulting in damage
Syringomyelia
to anterior white commissure (spinothalamic tract) first

INFECTIOUS

Poliovirus (fecal-oral transmission); replicates in oropharynx and small

Poliomyelitis intestine before spreading to bloodstream to CNS. Infection causes
destruction of cells in anterior horn of spinal cord (LMN death)

3° Syphilis; results from degeneration (demyelination) of dorsal columns and

Neurosyphilis (Tabes
roots, results in progressive sensory ataxia --> impaired proprioception -->
Dorsalis)
poor coordination

Pott's Disease (TB) Disseminated TB

Herpes Zoster, HSV1&2, EBV, CMV, Rabies

Bacteria are less likely cause (usually abscess) but Listeria, Borrelia,
Acute Infectious Myelitis
Treponema and Mycoplasma
Schistosomiasis: inflammatory and granulomatosis

Retrovirus-associated
Human T-cell Lympotropic Virus (HTLV-1) (Tropical spastic paraparesis)
Myelopathy

AUTOIMMUNE

Amyotrophic Lateral
Sclerosis

Multiple Sclerosis Autoimmine; demyelinating myelopathy

Neuromyelitis Optica Autoimmine; demyelinating myelopathy

VASCULAR

3 arteries supply the cord: 1 Anterior; 2 Posterior; systemic hypotension

Spinal cord infarction
causes cord infarction in watershed zone (T3-T4)

Anterior fed by vertebral arteries, radicular vessels (C6), T1 and T11 (artery
of Ademkiewicz); Anterior supplies anterior 2/3 of cross section of spinal
Complete occlusion of
cord with watershed zone between T1 and art. Of Adem.
anterior spinal artery
Etiology: aortic atherosclerosis, aortic dissection, vertebral occlusion,
profound hypotension

TRAUMA

Tract damage

Brown-Sequard
Syndrome
(UMN + LMN): Saddle anesthesia (S3-5), bladder dysfunction and
Conus Medullaris
impotence. Muscles okay
Low back and radicular pain, asymmetric leg weakness and sensory loss,
Cauda Equina Syndrome variable areflexia in the lower extremities, and relative sparing of bowel and
bladder function.
Cord Compression

Compression Fracture

Osteophyte (Bone spur)

Cervical spine and lumbrosacral spine due to narrowing around the spinal
Spinal Stenosis
cord due to various of causes (ex. ligamentous hypertrophy, tumors, etc.)

Impingement of bone and soft tissue overgrowth on nerve roots associated

Spondylitic Myelopathy with radicular pain, most common C5/C6

Herniation

MALIGNANCY
Ependymomas

Astrocytomas

Meningioma

Medulloblastoma "drop metastases" to the spinal cord

Metastases

METABOLIC

Vitamin B12 Deficiency

(Subacute Combined Dorsal column damage
Degeneration)

Hypocupric Myelopathy Low copper

Metachromatic
leukodystrophy

Krabbe Disease

Vitamin E

GENETIC

Werdnig-Hoffmann Congenital degeneration of anterior horns of spinal cord; LMN lesion only;
Disease autosomal recessive inheritance

Familial Spastic
Variable inheritance (AD, AR, XLR)
Paraplegia

X-linked; variant of adrenoleukodystrophy; mutation in peroxisome

Adreno-myeloneuropathy
membrane transporter

Fredrich's ataxia
Exam: look for sensory level on trunk! Look for UMN (increased tone,
reflexes, toes up)/LMN symptoms

PRESENTATION DIAGNOSIS

"Cape-like" b/l loss of pain and temperature sensation in UE

Floppy baby with marked hypotonia and tongue fasciculations; asymmetric

weakness (Poliomyelitis is restricted to gray matter of cord)

Cardinal signs: Argyll Roberson Pupil; decreased DTR in legs; impaired position
sense (ataxia of legs and gait); Romberg sign
Fleeting and repetitive lancinating pains in legs
Paresthesia, bladder disturbances; visceral crisis (acute abdominal pain with
vomiting)

HSV-2: Recurrent sacral myelitis associated with recurrences of genital herpes

Slowly progressive asymmetric spastic, variable sensory and bladder distrubance; HTLV-1 Ab in serum by ELISA;
50%: mild back pain or leg pain; unable to walk w/in 10 years western blot
Optic neuritis and myelopathy Serum anti-aquaporin 4 Abs

Anterior Cord Syndrome: loss of everything by spared vibration sense;

progressive over a few hours (unlike hemispheric stroke)
Spinal shock: areflexia, hyperreflexia, spasticity

Sciatica, +SLR

Neurogenic claudication (pseudoclaudication); not vascular; positional;

"shopping cart sign"; multiple L-S radiculopathies
Cervical stenosis: Generally insidious, may spot on PE with brisk DTRs and up-
pointing toes with some form of urgency or incontinence; follow closely to see if
symptoms get worse which requires surgery

Neck and shoulder pain with stiffness; compression of cord in 1/3 of cases can
produce spastic paraparesis MRI or myelography
Loss of vibration, touch and position sense in hands & feet; paresthesias; ataxia
Blood smear (macroovalocytes,
(spinocerebellar tract damage); spastic paralysis and +Babinski signs
hypersegmented PMNs); increased
(corticospinal tract damage); flaccid paralysis late with peripheral motor neuron
homosysteine and MMA
involvement; Optic atrophy
Low serum copper and serum
Presentation similar as SCD
ceruloplasmin

Similar to poliomyelitis except symmetric weakness

Onset 1st year to middle adulthood; Progressive spasticity + weakness in legs,

usually symmetrical; sensory symptoms are absent or mild; can have nystagmus, Symptomatic
ataxia, optic atrophy

Adrenal insufficiency beginning in childhood, progressive spastic or ataxic

Increased VLCFA in plasma
paraperesis beginning in early adulthood; mild peripheral neuropathy
TREATMENT PROGNOSIS

Acyclovir for suspected viral

Gancyclovir + Foscarnet if CMV

symptomatic

Steroids, Anti-CD20 antibodies for

NMO
Cervical collar, surgical
decompression by laminectomy
1000g of IM B12 repeated or oral
B12

Oral supplementation