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.