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.