Jerking stiff-man syndrome.
نویسندگان
چکیده
A female patient had permanent axial muscular rigidity similar to the "stiff-man syndrome", together with axial myoclonus triggered by stretch reflexes and by supramaximal stimulation of the supraorbital nerve. The disorder responded to treatment with diazepam and baclofen. This disorder bore a marked similarity to the so-called "jerking stiff-man syndrome".
منابع مشابه
Stiff man syndrome and related conditions.
The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous musc...
متن کاملA patient with reflex myoclonus and muscle rigidity: "jerking stiff-man syndrome".
A patient with progressive muscular rigidity associated with reflex myoclonus is described. The muscular rigidity was predominantly axial, and the myoclonic jerks affected axial and leg muscles. Jerks occurred either spontaneously, or in response to touch to the perioral region, or to stretch of head and neck muscles. Physiological investigations suggested that the myoclonus originated in the m...
متن کاملStiff leg syndrome and myelitis with anti-amphiphysin antibodies: a common physiopathology?
culty in walking. The symptoms had first appeared 2 weeks earlier. Her medical history included type 2 diabetes treated with metformin and a thyroid goiter treated with surgery and levothyroxine. On clinical examination, she presented permanent hypertonia in the lower limb and bilateral dystonia of the extensor hallucis longus muscles. Intermittent spasms were associated with hypertonia. The sp...
متن کاملStiff-person syndrome--DEEPER lOOK
Contributors Publication dates Originally released September 2, 1994; last updated June 7, 2010; expires June 7, 2013 Synonyms Stiff-man syndrome Key points • Stiff-person syndrome is a rare disorder that causes continuous muscle contraction with spasm, abnormal postures, and progressive disability. • Stiff-person syndrome is often associated with other autoimmune signs and symptoms as well as ...
متن کاملSpecific phobia is a frequent non-motor feature in stiff man syndrome.
OBJECTIVE To investigate systematically the rate and type of phobia in stiff man syndrome and its variants, and to compare patients with stiff man syndrome with and without phobia for sociodemographic and neurological characteristics. METHODS 43 consecutive patients with stiff man syndrome referred to a university department of neurology were assessed using the anxiety disorders interview sch...
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ورودعنوان ژورنال:
- Journal of neurology, neurosurgery, and psychiatry
دوره 45 12 شماره
صفحات -
تاریخ انتشار 1982