levetiracetam and Muscle-Rigidity

We studied the effects of oral levetiracetam (LEV) (500 mg twice daily) in three women with stiff-person syndrome in a single-blind, placebo-controlled study. The severity of muscle rigidity and of paroxysmal symptoms was assessed by EMG and clinically by a rating scale of 0-4 and by the Patients Global Impressions Scale. LEV was well tolerated. On active treatment all patients improved as assessed by any of the objective or subjective outcome measures. No response was noticed on placebo. Our data indicate that in patients with SPS, LEV is well tolerated and has a therapeutic role in the management of both muscle stiffness and life-threatening paroxysmal respiratory spasms.

Topics: Aged; Anticonvulsants; Electromyography; Female; Humans; Levetiracetam; Middle Aged; Muscle Rigidity; Muscle, Skeletal; Neurologic Examination; Piracetam; Respiration; Single-Blind Method; Spasm; Stiff-Person Syndrome

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.

Topics: Azathioprine; Drug Therapy, Combination; Encephalomyelitis; Female; Humans; Immunoglobulins; Immunosuppressive Agents; Levetiracetam; Middle Aged; Muscle Rigidity; Piracetam