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[Abdominal pain in progressive encephalomyelitis with rigidity].

J Seguier¹, J Serratrice¹, A Lachaud¹

¹Service de médecine interne, hôpital de la Timone, 264, rue Saint-Pierre, 13385 Marseille cedex 05, France.

La Revue De Medecine Interne

|January 28, 2014

Summary

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disorder. Early diagnosis is crucial, aided by antineuropile antibodies and electromyogram findings, despite varied presentations.

Keywords:

Abdominal pain Auto immunité Autoimmunity Douleur abdominale Encephalomyelitis Encéphalomyélite Rigidity Rigidité

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Area of Science:

Neurology
Immunology

Background:

Stiff-person syndrome is a rare neurological disorder characterized by trunk rigidity and painful muscle spasms.
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a clinical variant of stiff-person syndrome, encompassing neurological and cognitive disturbances.

Observation:

A 73-year-old woman presented with abdominal pain, anorexia, and significant weight loss.
The patient was diagnosed with progressive encephalomyelitis with rigidity and myoclonus (PERM).

Findings:

Diagnosis of PERM can be delayed due to diverse clinical presentations, sometimes lacking overt neurological signs.
The presence of antineuropile antibodies combined with electromyogram-confirmed muscular spasms strongly supports a PERM diagnosis.

Implications:

Recognizing the varied presentations of PERM is essential for timely diagnosis and management.
Antineuropile antibodies and EMG are key diagnostic biomarkers for PERM, improving patient outcomes.