Tremor induced by toluene misuse successfully treated by a Vim thalamotomy

A 22 year old man developed a vigorous tremor of 5 Hz in his right hand, after a 7 year history of toluene misuse. T2 Weighted MRI depicted marked decreases in the signal intensity of the basal ganglia, red nucleus, and thalamus on both sides. The stereotactic coagulation of the left nucleus ventrointermedius (Vim) of the thalamus abolished the tremors in his right hand. This patient clearly exhibited the pathological involvement of rubral lesions in generation of a toluene induced tremor on MRI. Toluene induced tremor is an irreversible symptom which persists even after stopping toluene misuse, therefore in medically intractable cases, it should be positively treated by a Vim thalamotomy.     

Writer's cramp and left frontal meningioma

A 55 year-old, right-handed, man developed a progressive writer's cramp spreading to a right hemidystonia. He had a left paramedian frontal meningioma. The association of an hemidystonia with contralateral lesions in the basal ganglia has been often reported. Its occurrence with a frontal lesion appear to be much less frequent. This association suggests a possible disorder of frontostriatal connections.     

书写痉挛的立体定向治疗

目的回顾性分析9例书写痉挛患者的立体定向手术治疗的方法及疗效,探讨书写痉挛患者的治疗机制。方法运用脑深部微电极引导下的立体定位技术,对9例药物治疗无效及符合手术指证的书写痉挛患者行丘脑腹外侧核团损毁术,并分析其治疗效果。结果 9例患者即刻恢复书写功能,术后随访,无手术并发症,2³ y的随访中,1例患者复发,余8例患者随访疗效稳定。结论立体定向丘脑腹外侧核团损毁术是书写痉挛的安全有效的治疗手段。     

Writer's cramp: not associated with anxiety

Many authors have commented on an association between writer's cramp and anxiety and some view it as a form of anxiety disorder. This study describes 22 patients with writer's cramp who were recruited from a neurology clinic. Clinical examination found little evidence of generalized anxiety or of anxiety specific to the writing situation. Additionally, the subjects' scores on the Crown-Crisp Experiential Index, which contains several anxiety subscales, did not differ significantly from those of a matched normal control group. Writer's cramp differs from the anxiety disorders in two other respects: it has a later onset (mean in the 30s) and the male preponderance is much greater than in any anxiety disorder, even social phobia.     

Writer's cramp secondary to spinocerebellar ataxia type 7

Spinocerebellar ataxia type 7 (SCA 7) is a rare autosomal dominant neurodegenerative disorder (ADCA) caused by expansion of a highly unstable CAG repeat. Clinical features including progressive cerebellar, retinal degeneration and pyramidal signs. We report a patient with SCA 7 diagnosis revealed by progressive cerebellar ataxia and writer's cramp.     

Writer's cramp: treatment with botulinum toxin injections

Twelve patients with writer's cramp were treated with injections of botulinum toxin. The overactive muscles were identified by clinical observation of the subjects while they were writing. Repeated injections were given at 2-week intervals until the optimal response was obtained. Eleven patients reported some benefit, which was considered by seven to be significant. Eight of the 10 patients who had pain reported moderate to significant relief. Five patients had local complications, consisting of disabling weakness of target or neighbouring muscles. These preliminary results suggest that this treatment can be successfully applied to many patients with writer's cramp without performing complex electromyographic recordings while the patients are writing.     

立体定向丘脑腹中间核毁损术治疗书写痉挛的疗效评估

目的探讨丘脑腹中间(Vim)核毁损术治疗书写痉挛的有效性和安全性。方法采用微电极引导下左侧丘脑Vim核毁损治疗书写痉挛(WC)患者47例,其中男32例,女15例;年龄15~64岁,平均(28.9±10.7)岁;病程2~21年。在术前和术后,采用书写痉挛评分量表(the writer's cramp rating scale,WCRS)对患者进行评分。结果全部患者毁损手术当时症状就明显改善,肘部、腕部和手指痉挛明显缓解,写字的流利程度和速度明显提升。其中2例患者(4.2%)出现一过性的右手指尖麻木;另有3例患者(6.4%)出现一过性言语不流利;上述并发症在3个月内完全恢复,没有永久性并发症发生。术后对患者进行电话随访,随访时间6~36个月,平均23个月;结果显示,患者的症状改善稳定;其中有3例患者失随访。另外,有1例患者因症状反复,再次行丘脑Vim核毁损术,术后症状改善明显。结论丘脑Vim核毁损术治疗书写痉挛是一种有效、安全的手术方式。     

Isaacs' syndrome successfully treated by immunoadsorption plasmapheresis

We report a 70-year-old woman with Isaacs' syndrome (acquired neuromyotonia) who showed a marked improvement after immunoadsorption plasmapheresis (IAP). She developed hyperhidrosis in her teens, and slowly progressive symptoms of neuromyotonia for over 50 years. An in vitro investigation of her serum with patch-clamp technique suggested the presence of antibodies against potassium channels. She was treated with IAP, which brought disappearance of her symptoms. Though the symptoms started to recur in 3 weeks, moderate improvement has been maintained by immunosuppressive drug treatment.     

Writer's cramp: Increased dorsal premotor activity during intended writing

Simple writer's cramp (WC) is a task‐specific form of dystonia, characterized by abnormal movements and postures of the hand during writing. It is extremely task‐specific, since dystonic symptoms can occur when a patient uses a pencil for writing, but not when it is used for sharpening. Maladaptive plasticity, loss of inhibition, and abnormal sensory processing are important pathophysiological elements of WC. However, it remains unclear how those elements can account for its task‐specificity. We used fMRI to isolate cerebral alterations associated with the task‐specificity of simple WC. Subjects (13 simple WC patients, 20 matched controls) imagined grasping a pencil to either write with it or sharpen it. On each trial, we manipulated the pencil's position and the number of imagined movements, while monitoring variations in motor output with electromyography. We show that simple WC is characterized by abnormally increased activity in the dorsal premotor cortex (PMd) when imagined actions are specifically related to writing. This cerebral effect was independent from the known deficits in dystonia in generating focal motor output and in processing somatosensory feedback. This abnormal activity of the PMd suggests that the task‐specific element of simple WC is primarily due to alterations at the planning level, in the computations that transform a desired action outcome into the motor commands leading to that action. These findings open the way for testing the therapeutic value of interventions that take into account the computational substrate of task‐specificity in simple WC, e.g. modulations of PMd activity during the planning phase of writing. Hum Brain Mapp, 2013. © 2011 Wiley Periodicals, Inc.     

Slit ventricle syndrome successfully treated by a lumboperitoneal shunt

The authors present the case of a 17-year-old man with slit ventricle syndrome, presenting as progressive neurological deterioration after head trauma. Serial computed tomography scans revealed slight ventricular enlargement, suggesting shunt malfunction. Communication between the lumbar subarachnoid space and the lateral ventricles was confirmed by computed tomography cisternography. He underwent a lumboperitoneal shunt, resulting in complete resolution of the symptoms. A lumboperitoneal shunt is considered to be a promising option for the treatment of slit ventricle syndrome. [Neurol Res 1995; 17; 440-442]     

Writer's cramp in an Australian pedigree with DYT1 dystonia.

Oppenheim's or DYT1 dystonia is a primary dystonia typically presenting in a limb at an early age and usually becoming generalised within 5 years. Over the last decade research into this debilitating disorder has progressed considerably, enabling the identification of a genetic lesion (a 3bp deletion in the DYT1 gene) now widely accepted as the cause of a majority of cases. This case report presents the first molecularly diagnosed pedigree of an Australian family with DYT1 dystonia, which presented as writer's cramp in the 15-year-old proband and two of his cousins.     

Primary writing tremor treated by stereotactic selective thalamotomy

Three cases with primary writing tremor were treated successfully by stereotactic selective thalamotomy centred mainly on the ventralis intermedius nucleus. They exhibited progressive coarse tremor of 5-7 Hz during writing, and Westphal's phenomenon on stretch, as the only neurological manifestations. Within the thalamus, a very high incidence of irregular burst discharges was recorded. These findings suggest that the writing tremor is an organic disorder.