Neel Golwala
Senior Grand
Rounds
1/22/2015
CENTRAL AND
PERIPHERAL NERVE
DISORDERS

 Differentiate common and uncommon nerve disorders
 Understand red flag symptoms
 Discuss management options in the ED
OBJECTIVES

 35 y/o F w/ BLE numbness
 Started in feet, ascended to calves and now thighs
 Today had difficulty emptying bladder
CASE 1

 Abd: slightly ttp lower abd w/o rebound or guarding, + lower
abd fullness
 Neuro: 3/5 strength LLE, 5/5 strength RLE
 Normal vibration/proprioception
 Decreased pain and temp T10 and below
 Deep tendon reflexes (DTRs) absent BLE
 R foot drop
CASE 1

 Foley: 1 L output
 DDx?
 Guillain-Barre syndrome
 Multiple sclerosis
 Spinal neoplasm vs. hematoma
 Transverse myelitis
CASE 1

CASE 1

 Interruption of ascending and descending pathways in the
transverse plane of spinal cord
 Autoimmune response or direct infection
 Ages 10-19, 30-39
TRANSVERSE MYELITIS

 Motor weakness, sensory abnormalities, bowel/bladder
dysfunction
 Usually bilateral
 Pain and temp lost below well-defined level (distinguishes
from peripheral lesions)
 MRI
 High-dose steroids (anecdotal), plasma exchange (best)
TRANSVERSE MYELITIS

 39 y/o M pmh HCV, heroin abuse
 Bilateral upper and lower weakness, neck pain
 Difficulty with urination
CASE 2

 Vitals normal
 HEENT, Pulm, Cards, Abd normal
 Neuro: BUE 3/5 strength, normal DTRs
 BLE 2/5 strength, absent DTRs
 C6 tender to palpation
CASE 2

CASE 2

 Infectious process
 Dorsal epidural space (rich vascular supply)
 Dura limits spread
 T-spine > L-spine >>> C-spine
 RFs: IVDA, DM, CKD, alcoholism, immunosuppression
EPIDURAL ABSCESS

 MC organisms: S. aureus (50%) >> Strep, E. coli,
Pseudomonas
 Classic triad: back pain, fevers, progressive neuro deficits
(rare)
 Untreated: bowel/bladder dysfunction  weakness 
paraplegia/quadriplegia, encephalopathy
EPIDURAL ABSCESS

 MRI w/ contrast, elevated WBC, elevated ESR/CRP
 Abx, decompression
 Outcome: dependent on speed of diagnosis, fatal in 18-23%
 Deficits rarely improve after 12-36 hrs
EPIDURAL ABSCESS

 Rare: (0.1/100,000)
 Trauma from LP, epidural anesthesia, spinal surgery
(spontaneous rare)
 RFs: anticoagulation, thrombocytopenia, liver disease
SPINAL EPIDURAL HEMATOMA

 Sudden, constant severe back pain w/ radicular component
 Progress to neurologic deficits (weakness, paresis, loss of
bowel/bladder)
 MRI
 Decompression
SPINAL EPIDURAL HEMATOMA

SPINAL EPIDURAL HEMATOMA

 60 y/o M w/ numbness and paresthesias of bilateral legs x 1
month
 Difficulty walking x 1 year
 New erectile dysfunction and occasional urinary incontinence
 Progressively blurry vision
 Multiple sexual partners
 Painless genital lesions 10 years ago
CASE 3

 Pupils constrict on accommodation, no response to light
 BUE normal strength/DTRs; BLE exam normal strength, absent
DTRs
 Joint position and vibration impaired below ASIS
 Ataxic gait; loss of balance when standing w/ eyes closed
CASE 3

 DDx?
 Labs?
 VDRL (serum, CSF), CBC, BMP, B12, RF, HIV, hepatitis
 VDRL strongly reactive
CASE 3

 Late manifestation of neurosyphilis
 Slow progressive degeneration of posterior columns
 Proprioceptive, vibratory and fine touch input
TABES DORSALIS

 Symptoms decades after initial infection
 Triad: unsteady gait, lightning-like pain, autonomic
dysfunction (urinary incontinence, erectile dysfunction)
 Seizures, HA, behavioral changes
 Argyll Robertson pupil, hyporeflexia, positive Romberg sign
TABES DORSALIS

 VDRL (serum, CSF), MRI
 Poor response to treatment (penicillin G iv)
TABES DORSALIS

 18 y/o F w/ BLE paresthesias
 Progressive weakness and difficulty walking
 URI 3 weeks ago
CASE 4

 BUE 5/5 strength, normal DTRs, sensory intact
 BLE 3/5 strength, absent DTRs
 Sensory intact, normal anal sphincter tone
CASE 4

 DDx?
 Guillain-Barre syndrome
 Lyme disease
 Botulism
 Multiple sclerosis
 Labs?
 CBC, BMP, ESR, CRP, LP
 LP: elevated CSF protein, normal WBC
CASE 4

 Acute inflammatory demyelinating polyneuropathy
 Progressive, symmetric distal weakness
 Days to weeks after URI or GI illness
 Usually worse in LE, partial or complete loss of DTRs, variable
sensory findings
GUILLAIN-BARRE SYNDROME

 MCC: C. jejuni, CMV, EBV, M. pneumoniae
 High concern for respiratory compromise
 Dx: LP (CSF w/ markedly elevated protein w/ normal WBC)
GUILLAIN-BARRE SYNDROME

 Always check FVC and negative inspiratory force
• FVC < 20 ml/kg or NIF < 30 cm H2O impending respiratory
compromise: intubate
• If ABG shows alveolar hypoventilation (elevated pCO2)  intubate
GUILLAIN-BARRE SYNDROME

 Treatment: IVIG, plasma exchange
 No proven benefit to steroids
GUILLAIN-BARRE SYNDROME

 42 y/o M w/ HA, dizziness, myalgias, malaise x 2 days
 Worsening weakness in BLE and now has gait instability
 Hiking in Colorado 5 days prior
CASE 5

 BUE 5/5 strength, normal DTRs, sensory intact
 BLE 3/5 strength, absent DTRs, sensory intact
 L medial thigh
CASE 5

 Rocky Mountain states, Pacific Northwest
 6 main species, including Ixodes and Dermacentor
 Neurotoxin inhibits presynaptic ACh release at NMJ
TICK PARALYSIS

 Prodrome: fatigue, restlessness, irritability, nausea
 Acute ascending flaccid paralysis and weakness
 Cranial nerve involvement
 Normal sensation
TICK PARALYSIS

 Death from respiratory muscle paralysis
 Treatment: removal of tick, supportive care, intubation as
necessary
TICK PARALYSIS

 3 month old F w/ decreased activity, poor feeding,
constipation
 Weak cry, decreased wet diapers
 Previously healthy, vaccines up-to-date
CASE 6

 BP 98/64, P 114, T 37.0C, weight 5.3 kg (75th %), height 57
cm (50%)
 Awake, no distress, weak cry
 Poor head control
 Decreased pupillary reflexes, absent corneal reflexes,
bilateral ptosis
CASE 6

 Weak suck and gag reflexes, increased oral secretions
 Abd soft, non-tender, no HSM, decreased bowel sounds
throughout
 Decreased muscle tone, decreased DTRs throughout
 No rashes or petechiae
CASE 6

 DDx?
 Sepsis
 Meningitis
 Encephalitis
 Hypothyroidism
 Polio
 Toxins
 Botulism
CASE 6

 C. botulinum: anaerobic spore-forming bacterium
 Types A, B, E, F cause human disease
 Preformed toxin  irreversible inhibition of ACh release at NMJ
 Descending, symmetric, flaccid paralysis 6-48 hrs post-
ingestion
BOTULISM

 CN and bulbar muscles affected first
 Anticholinergic Sx
 Pupils dilated, unresponsive to light (differentiates from
myasthenia gravis)
 Normal or diminished DTRs
BOTULISM

 Infants especially susceptible (higher gut pH)
 Spores survive in honey
 Lethargy, poor feeding, weak cry, constipation
 Diagnosis: clinical, stool/serum assay (usually send-out)
INFANTILE BOTULISM

 Treatment: human botulinum immune globulin (BabyBIG),
intubate as necessary
 Single dose reduces average hospital length from 5.5 wks to
2.5 wks and decreases intubation rate by 2/3
INFANTILE BOTULISM

QUESTIONS?

 The more vague the complaint, the more thorough the H&P
 Do a complete neuro exam
 Always ask social and travel history
TAKE HOME POINTS

 Lower extremity weakness
 High stepped gate, foot drop
 Frequent trips/falls
 Charcot-Marie-Tooth
ONE LAST THING