A 32-year-old man is brought to the emergency department due to difficulty walking and frequent falls. One week ago he had numbness in his toes and fingertips that progressed to lower-extremity weakness. He had an upper respiratory tract infection 4 weeks ago that resolved spontaneously. The patient has no bowel or bladder complaints. His past medical history is unremarkable. His temperature is 36.9° C (98.5° F), blood pressure is 130/70 mm Hg supine and 100/62 mm Hg standing, pulse is 102/min, and respirations are 18/min. Physical examination shows muscle weakness in both lower extremities and absent knee and ankle reflexes bilaterally. The sensory examination is unremarkable. Lumbar puncture is performed and cerebrospinal fluid analysis results are as follows:
Leukocytes 3/mm3 Glucose 70 mg/dL Protein 120 mg/dL Gram stain No organisms
What is the most appropriate next step in management of this patient?
Guillain-Barré syndrome | |
Pathophysiology |
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Clinical features |
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Diagnosis |
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Management |
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This patient with symmetric, ascending muscle weakness and absent deep-tendon reflexes after recent infectious illness most likely has Guillain-Barré syndrome (GBS). Patients with GBS may also present with bulbar symptoms (eg, dysarthria) and respiratory compromise.
Although GBS is primarily a motor polyneuropathy with ascending weakness (eg, starting in lower extremities), mild sensory symptoms such as paresthesias and sensory ataxia may also occur at different sites (eg, fingertip numbness). GBS patients are at risk of developing dysautonomia (eg, orthostatic hypotension, arrythmia, urinary retention, ileus, lack of sweating) due to autonomic nervous system involvement. About two-thirds of patients also have severe pain in the back or extremities.
Diagnosis is made clinically (eg, consistent symptoms, recent infection) as well as with lumbar puncture (LP) and neurophysiology studies (eg, electromyogram). LP shows elevated cerebrospinal fluid protein with normal white blood cell count (albuminocytologic dissociation).
Treatment of GBS includes supportive care (eg, monitoring of autonomic and respiratory functions) and intravenous immunoglobulin (IVIG) or plasmapheresis. Both IVIG and plasmapheresis are equally beneficial and the choice of treatment depends on patient-specific risk factors and availability.
(Choices A and E) Cyclosporine and pyridostigmine are used to treat myasthenia gravis (MG). MG usually presents with ocular symptoms (eg, ptosis, diplopia) and fluctuating muscle weakness that is worse late in the day. MG is usually not associated with an antecedent illness and rapidly ascending weakness. Deep-tendon reflexes are also normal in MG.
(Choice B) Intravenous acyclovir is used to treat herpes simplex encephalitis, which presents with fever, altered mental status, focal neurologic deficits, and seizures. Cerebrospinal fluid usually shows lymphocytic pleocytosis. This patient's presentation is not suggestive of herpes simplex virus encephalitis.
(Choice D) Glucocorticoids were previously used in the treatment of GBS. However, studies have shown that they are not beneficial and so are no longer recommended.
(Choice F) Riluzole is used in the treatment of amyotrophic lateral sclerosis, which presents with both upper and lower motor neuron signs. Acute presentation is uncommon as the disease is slowly progressive over a few years.
Educational objective:
Guillain-Barré syndrome (GBS) is characterized by ascending weakness, bulbar symptoms (eg, dysarthria) and respiratory compromise after antecedent illness such as respiratory or gastrointestinal infection (especially Campylobacter jejuni). Cerebrospinal fluid analysis shows albuminocytologic dissociation. Treatment of GBS includes intravenous immunoglobulin or plasmapheresis.