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 dif ficulty 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 l ight

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, l ightning-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 dif ficulty 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 il lness

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 (75 th %), 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 (dif ferentiates 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