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Successful immune moderation treatment for progressive encephalomyelitis with rigidity and myoclonus
Shinichi Ueno1, Nobukazu Miyamoto, Hideki Shimura
1Department of Neurology, Juntendo University Urayasu Hospital, Japan.
Abstract:
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disease. PERM consists of the same symptoms as stiff person syndrome, in addition to sensory, brainstem and autonomic features. We herein report a case of PERM in a 48-year-old woman who initially presented with spasticity of the lower limbs and subsequently developed upper limb spasticity, perioral myoclonus and restlessness after three months. The onset of potentially fatal dysautonomia was observed at the peak of the disease. Treatment with high-dose immunoglobulin (400 mg/kg, 5 days), levetiracetam and azathioprine resulted in a drastic and sustained improvement of these symptoms. This is an interesting case of PERM in which the patient showed a dramatic improvement following immune moderation.
Insights
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disorder. This case report highlights successful treatment of PERM with immunoglobulin therapy, leading to significant symptom improvement.
Area of Science:
- Neurology
- Immunology
Background:
- Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare autoimmune disorder.
- PERM shares symptoms with stiff person syndrome but includes sensory, brainstem, and autonomic dysfunction.
Observation:
- A 48-year-old woman presented with progressive lower limb spasticity, evolving to upper limb spasticity, perioral myoclonus, and restlessness.
- The patient developed potentially fatal dysautonomia at the disease's peak.
Findings:
- Treatment with high-dose immunoglobulin, levetiracetam, and azathioprine led to drastic and sustained symptom improvement.
- Immune modulation was effective in managing severe PERM symptoms.
Implications:
- This case demonstrates the efficacy of immune-based therapies in treating PERM.
- Early and aggressive treatment may prevent severe neurological and autonomic complications in PERM patients.
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