Painful tonic spasms caused by putaminal infarction.

Abstract

SUMMARY Lacunar infarcts in the basal ganglia are known to cause various movement disorders, such as chorea, focal dystonia, and hemlchorea-hemiballlsmus. We report here a case of putaminal lacunar infarction which presented with "painful tonic spasms" of the contralateral limbs. This consisted of paroxysmal brief, painful, flexor contractures of the upper, and occasionally the lower limb. These were not focal seizures but were controlled with carbamazepine, which has been used for the "painful tonic spasms" wellassociated with multiple sclerosis. The putaminal infarct we describe is probably related to a lupus anticoagulant and systemic lupus erythematosus.Stroke Vol 17, No 6, 1986PAINFUL TONIC SPASMS are a distinct, descriptive clinical entity. We believe this is the first case of known association with a contralateral putaminal infarct. These movements consist of a sudden, vigorous muscle spasm, preceded or accompanied by pain in the same limb(s). Usually unilateral, the arm is affected oftener than the leg, although both may simultaneously be involved. Facial grimacing can occur directly from facial spasm, or be reactionary to severe limb pain. Spasms last seconds to 1-2 minutes, and recur in stereotyped fashion daily, or even several times per hour. Temporary remissions may occur. Attacks can be triggered by hyperventilation, anxiety, physical activity or sensory stimuli, but usually are spontaneous. A classic "tetanic" posture can be assumed during a spasm, with arm adduction at the shoulder; elbow, wrist, and metacarpophalangeal flexion; and, interphalangeal extension. Variant postures with extension, fisting or clawing also exist. Flexion or extension occurs in the lower limb, usually with inversion of the foot. "* Case ReportThis 33-year-old woman was healthy until ten years ago when a prolonged bleeding time was discovered during evaluation for tonsillectomy. Neither patient nor family had a clinical bleeding disorder. The patient had recurrent bouts of pyelonephritis and keratoconjunctivitis. Progressive thrombocytopenia, resistant to steroid therapy, improved after splenectomy seven years ago.Soon thereafter, retrobulbar neuritis of the right eye occured, followed by five recurrences of retrobulbar neuritis, in either eye, over the ensuing seven years. Typically, her vision would painlessly diminish to "finger counting" at five feet, with quadrantic or altitudinal visual deficits, central scotomas, and afferent pupillary defect. The optic disc would pale without papilledema or vascular abnormalities. Her vision repeatedly responded to steroids, so she was kept on a chronic daily dose of 15 mg. prednisone.During the second episode of retrobulbar neuritis, further evaluation included a normal EEG, CT Scan, and temporal artery biopsy. Lumbar puncture revealed 1 RBC, 7 WBC (all lymphocytes), glucose = 68 mg/dl, protein = 48 mg./dl (normal electrophoresis, 9% gamma globulin), with cultures and VDRL negative.The patient was felt to have systemic lupus erythematosus with lupus anticoagulant, leading to prol...

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