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Published on: November 9, 2017
Anti-N-methyl-D-aspartate-glutamic-receptor encephalitis presenting as paroxysmal exercise-induced foot weakness
Angelo Labate1, Aldo Quattrone, Josep Dalmau
1Institute of Neurology University Magna Graecia, Catanzaro, Italy.
Background:
Patients with anti-N-methyl-D-aspartate-glutamic-receptors (NMDAR) encephalitis often develop complex abnormal movements that may manifest early in the course of illness.
Methods/Results:
A 17-year-old boy developed paroxysmal exercise-induced right foot weakness, without behavioral change. Two weeks later, he had hyperthermia and convulsive status epilepticus. Intravenous valproate resolved the status, but the paroxysmal episodes of right foot weakness persisted. They recurred any time he began running, without any refractory period or other triggering factors. Neurological examination between attacks was normal. Ictal video-polygraphy showed transient loss of lower limb muscle activity without any EEG changes. Results of brain MRI and extensive laboratory tests were normal. NMDAR antibodies were present in liquor and serum. No tumor was found. He was treated with intravenous methylprednisolone plus immunoglobulins with resolution of his symptoms that have not recurred at 8-month follow-up.
Conclusions:
Our case enlarges the spectrum of abnormal movements as presentation of oligosymptomatic anti-NMDAR encephalitis.
Insights
Anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis can present with unusual movement disorders. This case highlights exercise-induced weakness as an early symptom of oligosymptomatic NMDAR encephalitis.
Area of Science:
- Neurology
- Immunology
Background:
- Anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is an autoimmune condition.
- Patients often exhibit complex abnormal movements early in the disease course.
Purpose of the Study:
- To describe a unique presentation of anti-NMDAR encephalitis.
- To expand the understanding of movement disorders associated with this condition.
Main Methods:
- Case report of a 17-year-old male.
- Clinical presentation, neurological examination, ictal video-polygraphy, MRI, and laboratory tests including NMDAR antibody detection.
- Treatment with immunotherapy (methylprednisolone and immunoglobulins).
Main Results:
- The patient presented with paroxysmal exercise-induced right foot weakness, followed by hyperthermia and status epilepticus.
- Normal neurological examination between attacks and normal EEG during episodes.
- Positive NMDAR antibodies in serum and cerebrospinal fluid.
Conclusions:
- Exercise-induced weakness can be an early, oligosymptomatic manifestation of anti-NMDAR encephalitis.
- This case broadens the spectrum of movement abnormalities seen in NMDAR encephalitis.
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