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Flashcards in Neurosurgical DDx Deck (12)
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1
Q

DDx Myelopathy

A
  1. Congenital: Chiari malformation, Tethered cord, Syringomyelia,Neuroenteric cyst, mucopolysaccharidoses, Hereditary spastic paraplegia
  2. Acquired: Cervical or spinal stenosis, Traumatic (including spinal shock, hematomyelia, spinal epidural hematoma, barotrauma, electrical injuries, compression by bone fracture), Herniated intervertebral disc, Kyphosis, Extramedullary hematopoiesis,incompetence of odontoid process or transverse atlantal ligament, epidural lipomatosis, OPLL, arachnoiditis ossificans, vertebral Paget’s disease, idiopathic spinal cord herniation
  3. Neoplastic: Extradural primary tumors (neurofibromas, chordomas, osteoid osteomas, ABC, vertebral hemangioma), lymphoma, leukemic deposits (chloroma), epidural mets; Intradural-extramedullary (meningioma, neurofibroma); intradural-intramedulary (primary cord tumors: ependymoma, astrocytoma; rarely intradural mets); carcinomatosis meningitis; paraneoplastic syndrome
  4. Vascular: hematoma/hemorrhage, spinal cord infarction, spinal cord vascular malformation, radiation myelopathy, secondary to iodinated contrast material
  5. Autoimmune: post-viral or post-vaccination
  6. Demyelinating: acute transverse myelitis, MS, Devic syndrome
  7. Metabolic/toxic: subacute combined system disease (AKA subacute combined degeneration), toxins
  8. Infectious: (para) spinal abscess, vertebral osteitis/osteomyelitis, pyogenic discitis, HIV/AIDS related myelopathy, TB, spinal meningitis with pachymeningitis, viral (zoster, HSV-2, CMV), syphilis, parasitic cysts, CJD
  9. Peripheral neuromuscular disorder: GBS, chronic dysimmune neuropathies, myopathies
  10. Motor neuron disease: ALS, primary lateral sclerosis
2
Q

DDx Myelopathy: Congenital Etiologies

A
  • Chiari malformation (type I often presents in adulthood
  • Tethered cord (often doesn’t present until after trauma)
  • Syringomyelia: may be congenital or post-traumatic in some quadiplegics; usually presents with central cord syndrome or progressive myelopathy
  • Neuroenteric cyst
  • Cord compression that occurs with some mucopolysaccharidoses (e.g., Morquio syndrome due to atlanto-axial subluxation, or Hurler syndrome)
  • Hereditary spastic paraplegia: FHx key, this is a diagnosis of exclusion
3
Q

DDx Myelopathy: Acquired Etiologies

A
  • Cervical or spinal stenosis: often degenerative disease superimposed on congenitally narrow canal (e.g., achondroplastic dwarfs)
  • Traumatic: including spinal shock, hematomyelia, spinal epidural hematoma, barotrauma, electrical injuries, compression by bone fracture (may follow minor trauma in setting of canal stenosis)
  • Herniated intervertebral disc (myelopathy more common in thoracic region, radiculopathy more common in cervical region; long tract signs are rare with herniated cervical disc)
  • Kyphosis
  • Extramedullary hematopoiesis (hypertrophy of marrow causes cord compression; usually chronic anemias, eg, thalassemia major)
  • Incompetence of odontoid process or transverse atlantal ligament (may be congenital, traumatic, neoplastic, inflammatory, esp RA)
  • Epidural lipomatosis: hypertrophy of epidural fat, usually secondary to yrs of chronic steroid use
  • OPLL
  • Arachnoiditis ossificans: rare condition (43 case reports) involving calcification of arachnoid membrane
  • Idiopathic spinal cord herniation: rare condition where T-spinal cord herniated through anterior dural defect often causing Brown-Sequard syndrome or spastic paraparesis
4
Q

Arachnoiditis ossificans

A
  • Rare condition (43 case reports) involving calcification of arachnoid membrane
  • In T-spine, may occur as ossified plaques or in cylindrical form surrounding the spinal cord
  • May be difficult to detect on MRI and myelography
  • Plain unenhanced CT may be optimal for diagnosis
5
Q

DDx Myelopathy: Neoplastic causes

A

Extradural spine tumor (55%)
-Primary tumors (rare): neurofibromas, chordomas, osteoid osteoma, ABC, vertebral hemangioma
-If age > 40, suspect extradural lymphoma (primary or secondary) or leukemic deposit (chloroma), esp if preexisting Dx of heme or lymphatic disorder
-If age > 50, increasing incidence of epidural mets (occurs in 10% of cancer pts; 5-10% of malignancies have first presentation due to cord compression)
Intradural-Exctramedullary tumor (40%)
-Meningioma
-Neurofibroma
Intradural-intramedullary:
-Primary cord tumor: ependymoma, astrocytoma
-Rarely intramedullary mets
Carcinomatous meningitis: neuro deficit usually cannot be localized to single level
Paraneoplastic syndrome: including effects on spinal cord or peripheral nerves

6
Q

DDx Myelopathy: Vascular etiologies

A

Hematoma/Hemorrhage
-Spinal epidural hematoma: usually associated with anticoagulation; traumatic after LP or epidural anesthesia; spontaneous rare, includes hemorrhage from spinal cord AVM or from vertebral hemangioma
-Spinal SAH: may be post-traumatic or secondary to spinal cord AVM
-Hematomyelia
Spinal cord infarction: uncommon after eliminating syphilitic endarteritis; usually in territory of ASA which spares posterior columns; usually T4 level (watershed zone)
-Atherosclerosis of radicular artery in elderly patient with hypotension is now major cause of this rare condition
-Clamping aorta during surgery (eg for AAA)
-Hypotension (relative or absolute) during surgery in sitting position in presence of spinal stenosis (may be improved by avoiding absolute hypotension, using awake fiber-optic intubation and positioning, intraoperative SSEP monitoring and inducing hypertension if changes occur with positioning, avoidance of sitting position, and avoiding hyperflexion, hyperextension, and traction)
-Aortic dissection
-Embolization of spinal arteries
Spinal cord vascular malformations: 10-20% present as sudden onset of myelopathy usually pts 100 ml of CSF via LP and replace with equal amount saline over 30 min)

7
Q

DDx Myelopathy: Autoimmune etiologies

A

Post-viral (or post vaccination): may be etiology of auto-immune process (eg transverse myelitis); viral prodrome present in ~37% of cases of acute transverse myelitis
-Viral infection is usually most damaging to gray matter (eg poliomyelitis)

8
Q

DDx Myelopathy: Demyelinating etiologies

A
Acute (idiopathic) transverse myelitis: peak incidence in first 2 decades of life; abrupt onset LE weakness, sensory loss, back pain, sphincter disturbance indistinguishable from spinal cord compression; thoracic region most common; CT and myelogram are normal; MRI may show pathology; CSF show pleocytosis and hyperproteinemia
MS: diagnosed in only ~7% of pts presenting as acute transverse myelitis; more common in young adults, but can occur at any age, usually insidious and incomplete (some sparing), affects myelin thus sparing gray matter; abdominal cutaneous reflexes almost always absent in MS
Devic syndrome (neuromyelitis optica): variant of MS with acute bilateral optic neuritis and transverse myelitis extending 3 levels or more often causing cervical myelopathy; spinal cord edema may become so severe as to cause complete block on myelogram; more common in Asia and India than US or Europe; compared to classic MS, myelopathy tends to be more severe (more necrosis as opposed to incomplete demyelination) and with less chance of recovery; distinct serum IgG antibodies (NMO-IgG) may help differentiate from MS
9
Q

DDx Myelopathy: Metabolic/toxic etiologies

A

Subacute combined degeneration: B12 deficiency (dietary, pernicious anemia, or other gastric disorders such as low gastric pH in ZES which can inhibit attachment of intrinsic factor to ileal receptors)
Toxins: local anesthetics used for spinal anesthesia rarely cause myelopathy

10
Q

DDx Myelopathy: Infectious

A

(Para) spinal abscess (AKA spinal epidural abscess) or epidural empyema: often Hx of staph infection, usually skin furuncle; vertebral osteomyelitis often accompanies; produces local tenderness, back, pain, fever, elevated ESR
Vertebral osteitis/osteomyelitis
Pyogenic discitis: spontaneous or following procedures
HIV/AIDS related myelopathy: similar to B12 deficiency; spastic weakness and ataxia; can cause vacuolization of spinal cord; tropical (spastic) paraparesis of AIDS also seen in HTLV-I infection
TB: Pott’s disease
Spinal meningitis with pachymeningitis
Viral: Herpes varicella-zoster (rarely causes necrotizing myelopathy); HSV-2 (may cause ascending myelitis); CMV (may cause transverse myelitis)
Syphilis: may cause tabes dorsalis, syphilitic meningomyelitis, or spinal vascular syphilis; Dx by serum and CSF serology
Parasitic cysts
Form of CJD with predominant initial muscle wasting may mimic spinal cord disease or ALS

11
Q

DDx Myelopathy: peripheral neuromuscular disorder

A

Guillain-Barre syndrome: rapidly ascending weakness (mimics cord compression) with areflexia and near normal sensation
Chronic dysimmune neuropathies (presumed to be immune mediated): chronic immune demyelinating polyradiculoneuropathy (CIDP), similar to GBS but can progress over longer period of time; Multifocal motor neuropathy: characterized by asymmetric muscle wasting, cramping, and LE twitching, may mimic ALS but is treatable (with IVIG or immunosuppression)
Myopathies: including steroid myopathy (usually affects proximal > distal muscles)

12
Q

DDx Myelopathy: motor neuron diseases

A

ALS: upper and lower motor neuron disease; slight spasticity of LEs (extreme spasticity rare), atrophic weakness of hands and forearms, fasciculations in UEs, absence of sensory changes (including lack of pain), sphincter control usually perserved
Primary lateral sclerosis: age > 50, no LMN signs, slower progression than ALS (yrs to decades), pseudobulbar palsy is common