Electromyography (EMG) is a widely used diagnostic technique for disorders of the nervous system. The Canadian Society of Clinical Neurophysiologists (CSCN) promotes the education, evaluation and standards of EMG in Canada. A statement of practice standards was needed to clarify the position of the CSCN on several issues relevant to the practice of EMG.

A subcommittee of the CSCN reviewed current patterns of practice and established guidelines for review by the CSCN. The guidelines developed by the subcommittee were reviewed by the CSCN and adopted as recommendations for EMG practice. The subcommittee was charged with formulation of a document for publication.

This document deals with minimum standards for electromyographer education, laboratory operation, equipment and a variety of special circumstances relevant to the practice of EMG. The standards can be adopted by EMG laboratories to guide quality assurance.

Internal carotid artery dissection has been extensively studied and well-described. Although there has been a recent increase in the number of reported cases of vertebral artery (VA) dissection, the clinical variety of presentation and the early warning symptoms have not been well-described before. Our objectives in this study include: (1) To determine the early symptoms and warning signs which may help the clinician in the early identification and treatment of patients with VAdissection. (2) To explore the variety of clinical presentation of VA dissection and its relation to prognosis.

Retrospective analysis of hospital records in a tertiary academic centre for the period 1989-1999.

Twenty-six patients were identified (13 men and 13 women). The mean age was 48. Possible precipitating factors were identified in 14 patients (53%). Sporting activity and chiropractic manipulations were the most common (15% and 11% respectively). Headache and/or neck pain was the prominent feature in 88% of patients and was a warning sign in 53%, preceding onset of stroke by up to 14 days. The most common clinical features included vertigo (57%), unilateral facial paresthesia (46%), cerebellar signs (33%), lateral medullary signs (26%) and visual field defects (15%). Bilateral VA dissection presented in six patients (24%). The most common region of dissection was the C1-C2 level (16 arteries, 51%). Intracranial VAdissection was found in eight arteries (25%). The majority of patients (83%) had favorable outcome. Poor prognosis was associated with (1) bilateral dissection; (2) intracranial VA dissection accompanied by subarachnoid hemorrhage. Only two patients reported stroke recurrence.

Our findings show that VA dissection affects mainly middle age persons and involves both sexes equally. Headache and/or neck pain followed by vertigo or unilateral facial paresthesia is an important warning sign that may precede onset of stroke by several days. Although the majority of patients will have excellent prognosis, this was less likely in patients presenting with subarachnoid hemorrhage or bilateral VA dissection. Recurrence rate was low.

Traditionally, odontoid fractures have been treated with different bracing techniques resulting in variable degrees of successful healing. Surgical intervention is becoming more widely practiced as a primary intervention. The purpose of this report was to survey our recent experience in southern Alberta to determine potential outcome differences in management strategies.

We retrospectively reviewed the charts of 520 patients diagnosed with cervical spine fractures over a six-year period from January 1990, through December 1996. Patients were identified through the medical records database of the two Level 1 trauma facilities, on the basis of ICD-9 diagnostic coding.

Ninety-three fractures of the odontoid process were identified, of which 85 were acute and eight were chronic. There were 57 Type II (67%) and 27 Type III (32%) acute odontoid fractures. Of these, 64 were managed conservatively (bracing), whereas 20 were treated surgically. Thirteen patients underwent anterior screw fixation, seven patients had posterior cervical fusion. Eleven patients died in the acute phase, two as a result of their high cervical cord injury and nine from unrelated medical causes. Fifty-six of the remaining 82 patients (68%) were located with a minimum of three months follow-up (range three months to eight years). Satisfactory results were obtained in 76% of all acute patients treated by bracing, but only 50% in those over the age of 65. In the surgically managed group, all patients (100%) went on to develop stable fusions.

Our results indicate that while conservative management of odontoid fractures with external bracing results in fracture healing in most cases, surgical fusion may provide superior rates of bony union with acceptable morbidity. This difference in outcome lends itself to formal investigation through a prospective randomized trial.

To assess the feasibility and the toxicity of adjuvant high dose tamoxifen (TAM) and postoperative brain irradiation for patients with newly-diagnosed glioblastoma multiforme (GBM).

Twelve patients with histopathologically confirmed GBM entered the study. There were nine males and three females, with median age of 48.8 years (range 30-75 years). Karnofsky performance status (KPS) was 60-70% for four patients and 80-100% for eight patients. Based on the Radiation Therapy Oncology Group recursive partition analysis, there were three class III patients, six class IV, one class V, and two class VI. Eleven patients underwent partial surgical tumor resection and one patient had a near complete resection. Two weeks post surgery, the patients were started on high dose TAM (120mg/m2 P.O. BID for three months). Two weeks from date of starting TAM, external beam radiotherapy (RT) was given at a dose of 59.4 Gy/33 qd fractions/6.5 weeks. Patients were assessed weekly for toxicity during treatment. Imaging studies were done at the end of two weeks of TAM, then monthly.

Median follow-up was 40 weeks (range 22-84 weeks). In one patient, TAM was associated with significant vomiting, necessitating the TAM dose to be decreased at three weeks and then stopped at two months. One other patient had bilateral deep venous thrombosis after 51/2 weeks on TAM, although the relationship to TAM was not firmly established. There were no radiological responses after two weeks of TAM or at the end of RT. The median time to progression was 17.7 weeks (range 5.1- 43.8 weeks). Median survival time was 33.4 weeks (range 10-79.7). Actuarial survival at 48 and 74 weeks was 40% and 15%, respectively.

Our study shows that adjuvant high dose TAM is feasible and relatively well-tolerated. Furthermore, the combined use of high dose TAM and RT postoperatively was not associated with any significant increase in radiation-induced neurological toxicity. However, high dose TAM does not appear to improve treatment results.

Familial autoimmune myasthenia gravis (MG) is rare, although a genetic role for the development of autoimmune MG is suggested by concordance in monozygotic twins and the increased frequency of other autoimmune diseases in family members of myasthenics.

A patient with a family history of MG was evaluated in hospital. Relatives were interviewed and medical records examined for details regarding the diagnosis of MG in three other family members.

The index case first experienced symptoms of MG at age 75 years. She developed generalized MG and required corticosteroids and immunosuppressive therapy to control her disease. Her father developed predominantly bulbar symptoms of MG at age 75 years. He died of complications experienced following a gastrostomy placed for continued difficulty swallowing. His brother developed similar symptoms of MG in his early 60s and died shortly after thymectomy. A 46-year-old nephew of the index case is also beginning to exhibit signs of generalized MG. Acetylcholine receptor antibodies were strongly positive in the index case and her nephew. (The assay was not available for her father and uncle).

Four individuals in three successive generations had diagnoses of autoimmune MG. Study of familial cases such as these may clarify the contribution of genetic factors to the development of this disease.

Various investigators have developed complex quantitative instrumental procedures for objective assessment of parkinsonian motor impairment, since drawbacks of rating scales are interrater variability, subjective impression, and insensitivity to subtle modifications. Objectives: To determine whether performance of inserting of pegs and tapping (i) correlates with each other (ii) differentiates between parkinsonian subjects and healthy controls and (iii) reflects severity of Parkinson's disease (PD). Subjects and

In 157 previously untreated idiopathic parkinsonian patients and healthy controls, we measured (i) the total time taken to insert 25 pegs from a rack into a series of appropriate holes in a Purdue pegboard-like apparatus and (ii) the number of taps on a contact board with a contact pencil for a period of 32 seconds for assessment of fine motor skills.

Results of both tests correlated with each other, differed between parkinsonian subjects and controls and reflected scored severity of PD. Better correlation with intensity of PD was noted with the Purdue pegboard-like task.

Both tapping and inserting of pegs represent useful tools for objective evaluation of severity of PD. Peg insertion correlated better with disease severity. Both approaches may be useful in future clinical studies.

Anti-Purkinje cell antibodies have been reported in cerebellar ataxia following Epstein-Barr virus (EBV) infection. We investigated autoantibody responses, including anti-Purkinje cell antibodies, and the clinical course in eight children who developed post-varicella ataxia, five of their siblings with uncomplicated varicella, one child with post-EBV ataxia, two children with acute disseminated encephalomyelitis (ADEM) and one with neuroblastoma associated ataxia, and in age and gender matched controls.

Autoantibodies were tested by indirect immunofluorescence (IIF) on cryopreserved cerebrum and cerebellum sections. Other autoantibodies were measured by conventional IIF protocols using HEp-2 cells as a substrate. Antibodies to myelin associated glycoprotein (MAG), asialo-GM1, b2 glycoprotein 1, cardiolipin and myelin basic protein (MBP) were measured by ELISA.

Three of eight children with acute post-varicella ataxia, one child with post-EBV ataxia, one child with ADEM and one child with uncomplicated varicella, had high titer autoantibodies (<1/160) that reacted with cerebrum and cerebellar tissue. This reactivity was not seen in one child with ADEM, in one with neuroblastoma and ataxia, in the remainder of the children with uncomplicated varicella or age and gender matched controls. Autoantibodies were not seen in CSF from two children with post-varicella ataxia. The punctate staining seen on cerebrum and cerebellum sections co-localized with rabbit antibodies to the centrosome protein pericentrin. All patients with strong reactivity with cerebrum and cerebellar tissue by IIF had elevated levels of anti-MAG that was not confirmed by absorption assay. No reactivity was seen with asialo-GM1, MBP, b2 glycoprotein 1 or cardiolipin. None of the sera had autoantibodies directed against endosomes, the Golgi complex, or the paraneoplastic autoantigens Hu and Yo.

Some children with post-viral ataxia develop antibodies that have strong reactivity with cerebral and cerebellar tissue. Some of the antigenic reactivity co-localized with the centrosome protein pericentrin.

Electroencephalogram recordings are requested for the assessment of many childhood disorders. To assess the utility of the EEG in children, we studied how often routine EEG results can be correctly predicted from the EEG requisition.

Five hundred consecutive initial EEG requests from the IWK Grace Health Centre from two time epochs were examined. All EEGs were 16 channel (10-20 electrode system). Based only on the requisition (patient demographics, referring physician, and reason for EEG), we coded our prediction of the result and then the actual result. When results were discordant from prediction, a judgment was made about the potential importance of the result.

Overall, EEG results were correctly predicted in 81%. Prediction for all nonepilepsy reasons was accurate in 91% (n=320) and 96% for paroxysmal nonepileptic events (n=158) but only 59% for epileptic disorders (n=141) (p<0.0001). Neurologists ordered 45% of EEGs, pediatricians 32%, and GP's 17%. Predictions were least accurate for neurologists'requests (p<0.006) however, neurologists were more likely to request EEG for epileptic disorders (p<0.0001). Age of the child and urban versus rural address did not affect the accuracy of prediction.

Results of routine pediatric EEG for most nonepilepsy reasons appear highly predictable and therefore, possibly of little value to an experienced clinician. When requested for epilepsy, this “ancient” test remains full of surprises.

A 52-year-old woman with metastases in brain and bone had clinical and radiological response to therapy but died about 10 weeks after diagnosis. General autopsy failed to identify a primary neoplasm or an anatomic cause of death. Investigation of sudden respiratory cessation was a consideration when undertaking an anatomic study of the brain.

Review of patient records and careful examination of the brain following autopsy were carried out.

The patient had terminal episodes of hypersomnia but episodes of sleep apnea were not observed. She received no respiratory support and no respiratory difficulties were recorded until she was pronounced dead at 7 a.m. Autopsy revealed metastatic adenocarcinoma in a pattern suggestive of a primary pulmonary neoplasm, including multiple cerebral metastases, although no significant pulmonary lesions of any type were found. A 0.2 cm metastatic adenocarcinoma was found in the nucleus of the tractus solitarius (NTS). No other tumor was present in the brain stem.

Unilateral destruction of the NTS in the medulla would have severely disturbed the most critical point of convergence of autonomic and voluntary respiratory control and of cardiocirculatory reflexes in the central autonomic network. It is postulated that this caused respiratory arrest during a state transition from sleeping to waking. Few metastases to the medulla are reported, most are relatively large, and several have caused respiratory symptoms before death. The very small metastasis in our patient could be the direct anatomic cause of death, and as such it is an unusual complication of metastatic disease of which clinicians should be aware. It is speculated that dysfunction of direct NTS connections to the pons or of connections passing close to the metastatic deposit resulted in terminal hypersomnia.

We report the treatment and follow-up, including MRI, of two patients with idiopathic hypertrophic pachymeningitis and review the English language literature, with emphasis on management and outcome in this rare disorder.

The files of two patients were reviewed, with relevant histopathology and imaging (MRI). The first patient has been followed for sixteen years (the longest MRI-documented postoperative course reported for this condition) and the second for two years. The English language literature was reviewed, including a summary of all reported patients that have been followed with MRI or CT imaging.

Despite extensive investigation, no underlying etiology was determined in either patient. Histopathological studies revealed a chronic inflammatory dural infiltrate in both patients, with granulomas in the first but not the second patient. The first patient underwent surgery twice and has remained stable for sixteen years, despite persistent neurologic deficits. The second patient was managed with dexamethasone after a surgical biopsy, and experienced complete resolution of all neurological deficits and abnormalities seen with MRI.

Although prompt and extensive surgery has been recommended for this condition, the results from our second patient indicate that complete remission can be achieved in some patients with biopsy and steroid therapy. This also supports the view that autoimmune mechanisms underlie idiopathic hypertrophic pachymeningitis. The first patient illustrates that extensive laminectomies may be an effective therapeutic option but chronic discomfort may result. If extensive surgery must be performed, laminoplasty should be done because of the potential for reduced pain and improved long-term spinal stability.

Familial adenomatous polyposis (FAP) is associated with malignant tumors of the central nervous system, predominantly medulloblastomas and glioblastoma multiforme (Turcot's syndrome) and with craniofacial osteomas (Gardner's syndrome). This report details the occurrence of benign, intracranial tumors in two related individuals with Gardner's syndrome, an asso ciation not previously described.

A 57-year-old woman (the propositus), her sister, two of her nieces and one of her grandnephews were previously diagnosed with Gardner's syndrome. The propositus came to neurosurgical attention because of vertigo associated with what proved to be an epidermoid cyst of the cerebellopontine angle. Her unaffected children and her relatives with Gardner's syndrome were examined and underwent computed tomography or magnetic resonance imaging.

A 39-year-old woman with Gardner's syndrome, the niece of the propositus, was found to harbor an asymptomatic left frontal meningioma.

Familial adenomatous polyposis, Gardner's syndrome, and that variant of Turcot's syndrome in which medulloblastoma predominate, are associated with a mutation of the adenomatous polyposis coli gene. The demonstration that patients with Gardner's syndrome can also have benign, nonneuroglial, intracranial tumors adds to the previously known extracolonic lesions associated with FAP. The molecular characterization of our patients should reveal if benign intracranial tumors represent a pleiotropic manifestation of the adenomatous polyposis coli gene mutation or if other genes are implicated.