Gamma knife thalamotomy (GKT) has been used as a therapeutic option for patients with disabling tremor refractory to medications. Impressive improvement of tremor has been reported in the neurosurgical literature, but the reliability of such data has been questioned.
OBJECTIVE:
To describe and provide audiovisual documentation of a syndrome of polymyoclonus, laryngospasm, and cerebellar ataxia associated with adenocarcinoma and multiple neural cation channel autoantibodies.
DESIGN:
Case report with video.
SETTING:
University hospitals. Patient A 69-year-old woman presented with subacute onset of whole-body tremulousness and laryngospasm attributed to gastroesophageal reflux.
RESULTS:
Further evaluation revealed polymyoclonus, cerebellar ataxia, and laryngospasm suspicious of an underlying malignant neoplasm. Surface electromyography of multiple limb muscles confirmed the presence of polymyoclonus. The patient was seropositive for P/Q-type voltage-gated calcium channel antibody; subsequently, whole-body fluorine 18 fluorodeoxyglucose positron emission tomography and cervical lymph node biopsy revealed widespread metastatic adenocarcinoma. Follow-up serologic evaluation revealed calcium channel antibodies (P/Q type and N type) and potassium channel antibody.
CONCLUSIONS:
We highlight the importance of recognizing polymyoclonus. To our knowledge, this is also the first description of a syndrome of polymyoclonus, laryngospasm, and ataxia associated with adenocarcinoma and these cation channel antibodies.
In 1885, Gilles de la Tourette described 9 patients who suffered from a disorder characterized by involuntary movements, echolalia, echopraxia, coprolalia, and strange, uncontrollable sounds. In his article, Gilles de la Tourette presented some earlier descriptions of this disorder. To appreciate what first led Gilles de la Tourette to Tourette syndrome, however, it is necessary to turn to an article that he published a year earlier. In his 1884 article, Gilles de la Tourette cited several movement disorders that he thought were similar to each other, yet different from true chorea. After describing these disorders, namely, "jumping" of Maine, latah of Malaysia, and miryachit of Siberia, he briefly mentioned a boy in Charcot's ward in Paris, France, who seemed to exhibit the same condition. In an addendum, he then said that other cases were now surfacing in Paris and that he would write an additional article describing these individuals. To achieve a more thorough understanding of the events that led Gilles de la Tourette to his 1885 description of the disorder that now bears his name, we herein present an English-language translation of his 1884 article along with a commentary.
Thirty consecutive patients from peninsular Malaysia with clinically definite multiple sclerosis were studied; 80% were ethnic Chinese, with a female-male ratio of 5:1. The average age at onset was 29.7 years, with one relapse average every 1.9 years. Optic-spinal recurrence was the most common clinical pattern of the disease, accounting for 63.3% (19/30) of the cases. All the patients had spinal cord involvement sometime during the course of the illness. The mortality was high at 36.7% (11/30), with an average duration of symptoms of 7.6 years. There was characteristic severe residual visual and motor disability. At the time of the last examination, 12 patients had bilateral optic atrophy with blindness or severe visual acuity impairment. Sixteen patients were bedridden or confined to a wheelchair. The severe motor disability reflected the severe spinal cord involvement. It was the main factor that accounted for the high mortality. The cerebral, cerebellar, and brain-stem involvements were, however, generally transient. None of the patients' had a family history of similar illness despite the average sibling size of six. There was no example of Devic's disease. The clinical pattern was closest to those patients who presented from Taiwan.