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A young man with weakness and paresthesias
Charles T. Allred, M.D.
06/25/2010
History
• 37 y.o. previously healthy male.– 2 weeks prior developed burning pain in left
shoulder followed by weakness, ? due to pain.– Seen in ER – muscle relaxant and pain med.– Cont. pain and again went to ER. No change.– 1 week prior, right facial weakness, some
difficulty with speech. Seen in ER. Dx. Bell’s palsy with prednisone and acyclovir prescribed.
HX. Cont.’
• Finally seen in Dr.’s office. Neg. HIV, RPR, hepatitis testing, CBC, lead level and CMP.
• 2 day history of left facial numbness, difficulty feeling urination and defecation but no incontinence.
• 1 day history of leg weakness and paresthesias from abd. to knees, difficulty with walking, worse left arm weakness.
Social, F. Hx., ROS
• Smokes ½ ppd.
• No ETOH or drug use.
• Employed as pipe bender.
• Heterosexual and in monogamous rel.
• Aunt has MS.
• Brother has lymphoma.
• ROS + for viral URI 4 weeks prior.
EXAM
• VS – afebrile, P – 99, BP – 164/117• Eyes – normal fundi.• Neuro –
– right facial weakness (lower motor neuron type); – subjective numbness left face; – unable to abduct at left shoulder but only sl.
decreased grip strength; – able to stand and walk but abn. gait with proximal
muscle weakness in legs;
EXAM cont.’
• Neuro – – Subjective decrease in sensation lower abd.
and thighs.– Reflexes – negative Babinski bilaterally.
Decreased reflexes in brachioradialis, absent otherwise.
– Rectal – normal sphincter tone.
Lab and x ray
• CT of head – normal.
• CBC, CMP, sed rate all normal.
Differential Diagnosis
Differential Dx.
• Guillain-Barre’ syndrome• Lyme disease• Acute arsenic poisoning• Glue sniffing neuropathy• Acute spinal cord disease
– – Transverse myelitis– Trauma to spinal cord– Tumor– Multiple sclerosis
• Tick paralysis• Painful, paralytic
porphyria• Myasthenia gravis• Botulism• Polyneuropathy of critical
illness• Lambert Eaton syndrome
Diagnosis of GB syndrome
• Clinical history of progressive, fairly symmetric muscle weakness accompanied by absent or depressed DTRs.
• Cerebrospinal fluid - < 10 cells and elevated protein (90%). May be normal first few days of illness.
• Abnormal nerve conduction and EMG studies. Abnormal within a few days of onset.
Pathophysiology
• Illness triggered by a number of factors:– Viral illness most common.
• Includes Epstein-Barr, cytomegalovirus, HIV, influenza, lots of others.
– Bacterial illness, especially Campylobacter jejuni.– Trauma, surgery, bone marrow transplant, SLE, and
more.– Immunizations.
• Increased association after 1976 swine influenza vaccine and again when 1992 and 1993 seasons were combined.
• Menactra – cases reported after vaccine.
Pathophysiology cont.’
• Infection (or other event) provokes immune response.
• Cross reaction with peripheral nerve components due to molecular mimicry.
• Results in acute polyneuropathy.
• Immune response can be against myelin or the axon of nerves (more severe case.)
Clinical Features
• Incidence 1 in 100,000 per year.– Females > males.– Age - bimodal peaks in young adults and the
elderly. Range in one series 8 months to 81 years.
– Worldwide but a severe variant more common in Japan and China after C. jejuni.
• Mortality 5% even with modern treatment.
Clinical Features
• Any peripheral nerve can be effected, so:– Motor:
• classically ascending weakness (Landry ascending paralysis). 90% weakness begins in legs, 10% arms or face.
• Respiratory weakness leads to needing ventilator support in up to 30% of cases.
• Facial and oropharyngeal weakness develops in 50%.
• Oculomotor weakness in 15%.
Clinical Features
• Any nerve:– Sensory:
• Mild numbness in fingers and toes common initially.
• Most sensory findings are mild.• Pain in low back and/or proximal legs in > 50%.
– Lumbar disc disease a common mistaken dx. early.
Clinical Features
• Any nerve:– Autonomic dysfunction:
• Some finding in 70% of patients.• Severe in 20% mostly in those with severe
weakness.• Manifested by
– Tachycardia and other rhythm problems.– Hypertension alternating with hypotension.– Orthostatic hypotension.– Urinary retention and ileus.– Too much sweat or inability to sweat.
Clinical Features
• Remember:– Progressive over 1 to 4 weeks.– Fairly symmetric muscle weakness.
–Decreased or absent DTR’s – ankle within the first week.
–Elevated CSF protein without increase in WBCs.
Management
• Two pronged – supportive and disease modifying.
• Supportive:– Respiratory failure (up to 30%)
• Measure vital capacity and negative inspiratory force every 4 hours.
• Intubate if forced vital capacity < 20 ml/kg or neg. inspiratory force < 30 cmH2O.
Management
• Respiratory failure – – Predictors of failure:
• Time of onset to admission < 7 days.• Inability to cough.• Inability to stand.• Inability to lift elbows.• Inability to lift head.• Elevated LFTs.
Management
• Supportive:– Autonomic dysfunction:
• Telemetry to monitor for rhythm problems. • Blood pressure q 4 hours or >.
– May need short acting pressors or lowering agents.
• Don’t sit a paralyzed pt. up without checking orthostatic BP first!
• Follow bladder scans for post void residual and listen to bowel sounds daily.
– Pain control. • Careful with narcotics.• Gabapentin, carbamazepine considerations.
Management
• Supportive:– Prevent secondary problems:
• DVT prophylaxis.• Think about pneumonia, UTI’s, decub problems.• Depression and emotional distress.
– Rehab with recovery.
Management
• Disease modifying:– Plasmapheresis.
• Believed to remove the offending antibodies from serum.
• 4 to 6 treatments QOD or QD if needed.
– Intravenous immune globulin.• ? Binds the antibodies.• 0.4 mg/kg per day x 5 days.
– Both equally effective. Onset to recovery shortened by 50%.
Prognosis
• Predictors of poor prognosis:– Older age.– Rapid onset (less than 7 days) prior to
presentation.– Need for ventilatory support.– Motor response amplitude <20% of normal.– Preceding diarrheal illness (C. jejuni).
Prognosis
• At 6 months, 65% walk independently.• Overall, 80% recover completely or with minor
deficits.• 5 – 10% of pts have a prolonged course over
several months with ventilator dependency and delayed and incomplete recovery.
• 5% die. Causes include sepsis, PE, unexplained cardiac arrest, ARDS.
• Relapses occur in 10% and require repeat tx.
Our patient
• CSF – RBCs – 0.– WBCs – 7.– Differential – 49% lymphocytes, 44%
monocytes.– Glucose – 67.– Protein – 176.
Our patient
• Received IVIG daily x 5 days. By second day was feeling better with less pain and better strength. Never had any respiratory problems. BP improved, remained tachy in low 100s. Dismissed day 6 walking without difficulty.
• 6 week follow up, requested release to go back to work. Estimated shoulder was 50% of normal and legs 80%. No further FU since.
Summary
• Weird, neurological stuff doesn’t always mean your pt. is crazy, Joe.
• Progressive, ascending paralysis with absent reflexes, think GBS.
• Tap. High protein and low cells – give IVIG or call Henry.