Cervical dystonia (CD) is characterized by sustained contractions of the neck musculature, resulting in abnormal head postures. The Cervical Dystonia Severity Scale (CDSS) was developed to provide a reliable measure of treatment response in patients with CD. The CDSS uses a protractor and wall chart to rate the severity of the head's deviation from neutral in each of three planes of motion (rotation, laterocollis, anterocollis/retrocollis), which is then scored in 5 degree intervals (1 degree to 5 degrees deviation = 1; 86 degree to 90 degrees deviation = 18). To test the reliability of the CDSS, four centers, each with two independent examiners, evaluated 42 patients with CD. At each site, each of the two examiners used the CDSS to evaluate the head position of each patient twice, on the same day, for a total of four evaluations. The kappa value for intra-examiner agreement was 0.94 (95% confidence limit of 0.900-0.972), indicating excellent intra-examiner reliability. The kappa value for interexaminer reliability was 0.79 for the first evaluation and 0.86 for the second evaluation (95% confidence limits of 0.668-0.920 and 0.790-0.920) indicating excellent interexaminer reliability. Thus, the CDSS was highly reliable in both intra-examiner and interexaminer scoring comparisons.
SUMMARY Arachnoid cysts which develop in relation to the cerebral hemispheres are usually found in the middle cranial fossa. These cysts are usually asymptomatic but can produce symptoms if there is haemorrhage into the cyst or the development of an associated subdural hematoma. Recent publications have emphasised the association of arachnoid cysts of the middle fossa with subdural haematomas. This report describes a case of an asymptomatic arachnoid cyst which ruptured into the subdural space. This event was followed by the development of symptoms despite the lack of haemorrhage.The advent of computed tomography (CT scan) has increased the recognition and facilitated the evaluation of arachnoid cysts of the middle cranial fossa.' It has been suggested that the presence of an excessive contralateral shift of the midline structures and/or collapse of the ipsilateral ventricular system in a CT scan showing an arachnoid cyst of the middle cranial fossa is compatible with the presence of an associated subdural hematoma.2 This complication is believed to result from tearing of blood vessels draped over the cyst following minor head trauma or rupture of the cyst.3Case reportAn 11-year-old boy was admitted to hospital for evaluation of headaches of one month's duration. The headaches started after he had been swimming, but there was no history of head trauma. The first headache was bilateral, diffuse and throbbing in nature and lasted about 24 hours. Subsequent headaches were left-sided, lasted several hours, and occurred at least once a day. They were often present upon awakening and occasionally awoke him from sleep. Their onset was sometimes heralded by a brief flash of red light which was not localised to a particular visual field. There was usually no nausea or vomiting, however, one episode of projectile vomiting, without nausea, occurred just after admission. There was no photophobia. The headaches were relieved by paracetamol and codeine. The
Botulinum toxin (BTX) injection is considered the treatment of choice for patients with cervical dystonia (torticollis). We conducted a pilot, open-label, dose-escalation study with BTX type B in 12 patients who no longer responded clinically to injections with BTX type A. At the doses tested, BTX type B was safe and well tolerated without evidence of dose-limiting toxicity in this patient population. Mild-to-moderate adverse events generally resolved quickly and included asthenia, pain, nausea, dysphagia, hypertonia, and tremor. No serious adverse events or antibodies to type-B treatment were reported. Low-dosing-session (100-899 units) and high-dosing-session (900-1,500 units) groups were defined based on units administered per dosing session. Toronto Western Spasmodic Torticollis Rating Scale-Severity Scale (TWSTRS-Severity), Patient Analogue Pain Scale, and Physician and Patient Global Assessment Scales were measured during this study. The TWSTRS-Severity mean maximum percent improvement from baseline demonstrated a 9.9% versus 28.8% difference between the low-dose and high-dose groups, respectively. EFfectiveness was noted for the high-dose group on the Patient Analogue Pain Scale but not on the Global Assessment Scales.
A 41 year old male presented with headache, lethargy, and ataxia and found to have a left temporal lobe mass and a leukoerythroblastic peripheral blood smear. The latter prompted an iliac crest bone marrow biopsy on which a diagnosis of metastatic glioma was made and verified by immunohistologic characterization. The patient was treated with cranial irradiation and simultaneous systemic BCNU (bis-dichloroethylnitrosurea) with complete response. This case with diffuse bone marrow involvement demonstrates that a glioblastoma is capable of extracranial metastases without previous intervention. From a review of reported cases of gliomas of extraneural metastasis, it is concluded that untreated gliomas are capable of vascular spread although less frequently than previously manipulated tumors.
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