Multiple sclerosis (MS) is one of the most common neurological diseases affecting young adults. The prevalence of MS in Alberta has been described as among the highest reported in the world, estimated at 217 per 100,000. Numerous anecdotal reports, and a few small empirical investigations have suggested that cannabis use may relieve the symptom experience of those with MS. The present study was undertaken to describe cannabis use by this patient group. Information on peoples’beliefs, practices and experiences related to use were investigated.

Aquestionnaire was mailed to a sample of 780 adults with MS in southern Alberta, Canada.

Completed questionnaires were returned by 420/673 eligible subjects (response rate 62%). Mean sample age was 48 years and 75% were women. Respondents ranged from mildly to severely impaired. The majority of respondents (96%) was aware cannabis was potentially therapeutically useful for MS and most (72%) supported legalization for medicinal purposes. Forty-three percent had tried cannabis at some point in their lives, 16% for medicinal purposes. Symptoms reported to be ameliorated included anxiety/depression, spasticity and chronic pain. Reasons given for not trying cannabis were the fact that it is an illegal substance, concern about side effects and lack of knowledge on how to obtain it.

Subjective improvements in symptom experience were reported by the majority of people with MS who currently use cannabis. Further evaluation of this substance is warranted.

To examine the longitudinal media reported rate of concussions in the National Hockey League (NHL) over the period 1986-87 to 2001-02.

All injury reports published in the weekly sports newspaper The Hockey News for the 16 seasons 1986-87 through 2001-02 were reviewed for reported concussions. The Hockey News reports are based on weekly injury reports released by the NHL, which derive from reports submitted to the league by individual team offices.

Adjusted for changes in the number of teams and games per season over the 16 year study period, and expressed as: number of concussions per 1000 games, results by season (starting with 1986-87) were 4, 8, 7, 7, 5, 5, 7, 7, 6, 8, 13, 20, 30, 27, 30, 25. Comparing each season with the prior season, significant increases were reported in 1997-98 and 1998-99 (p<0.05 and 0.025, respectively), with no change since 1998-99.

The reported concussion rate in the NHL during the last five years is more than triple that of the previous decade. Bigger, faster players, new equipment and harder boards and glass have all theoretically increased the risk of concussion in the NHL in recent years. However, the abrupt increase and subsequent plateau in concussion rate since 1997 suggests that increased recognition and reporting may be primarily responsible for the apparent increase in incidence.

Elevation of blood pressure (BP) is common in acute cerebral infarction, with several studies reporting a high plasma catecholamine level or previous hypertension as a contributory factor. However, more comprehensive studies on associated clinical parameters are lacking. Our main aim in undertaking this study was to correlate clinical variables associated with a BPelevation in acute ischemic stroke.

Consecutive patients who were admitted to the emergency room and diagnosed with an acute cerebral infarction within 24 hours after the onset of symptoms were investigated. A BP elevation was defined as a high systolic (³200mmHg) or diastolic (³110 mmHg) pressure. The mean systolic and diastolic BP were compared between the different stroke subtypes, lesion locations (carotid vs. vertebrobasilar), and hemispheric sides. The frequency of symptoms, risk factors, location of the infarct, stroke severity, vascular status and laboratory abnormalities were analyzed in order to build a regression model.

One hundred thirty-one patients were recruited (M:F=60:71, mean age 66±12 years) and an elevated BP was identified in 33 patients (25.2%). The mean systolic and diastolic BP did not differ significantly between the stroke subtypes, lesion locations, and hemispheric sides. According to univariate logistic regression, an elevated systolic BP correlated with headache (p=0.01) and underlying hypertension (p=0.02) while an elevated diastolic BP correlated with underlying hypertension (p=0.01). Multivariate logistic regression analysis revealed previous hypertension (OR 5.21, 95% CI 1.40-19.37) and headache (OR 4.09, 95% CI 1.44-11.66) to be independent predictors of an elevated systolic BP.

Headache itself is closely associated with severe systolic BP elevation in acute ischemic stroke. Whether treatment of elevated BP improves headache and clinical outcome is not yet known, necessitating future controlled studies.

P53 expression and increased MIB-1 proliferation index have been shown to correlate with invasive behavior in pituitary adenomas. The purpose of this study was to determine whether these indices could be used to predict a higher likelihood of recurrence in clinically nonfunctional pituitary adenomas and thus guide adjuvant therapy.

Fifty-one clinically nonfunctional pituitary adenomas were selected from the database at the Vancouver Hospital and Health Sciences Center between the years 1990-1998. Included were 32 nonrecurrent and 19 recurrent adenomas.

The mean initial labelling index for p53 in nonrecurrent tumours was 0.38% (0-1.58%), while it was 0.46% (0-3.65%) for recurrent adenomas. The mean initial MIB-1 index for nonrecurrent tumours was 1.63% (0.08-9.36%), while for recurrent tumours it was 1.92% (0-7.76%). The percentage of p53 positive adenomas was 66% for nonrecurrent tumours and 68% for recurrent tumours. None of the differences in the labelling indices between the recurrent and nonrecurrent groups was statistically significant. As 12 patients (38%) in the nonrecurrent group had undergone radiotherapy as initial adjuvant therapy after surgery and none of the recurrent group had done so, patients who did not receive radiotherapy in the nonrecurrent group were analyzed separately. Again, none of the differences in the labelling indices between the recurrent and nonrecurrent groups was statistically significant when the effect of radiotherapy was removed from the analysis.

The results demonstrate no statistical difference in the p53 or MIB-1 labelling indices between recurrent and nonrecurrent nonfunctional pituitary adenomas. Concern should be raised in attaching too much clinical significance to these labelling indices, especially with respect to p53 as a predictor of the clinical behavior of nonfunctional pituitary adenomas.

Trigeminal neuralgia (TN) has a higher incidence among patients with multiple sclerosis (MS) than in the general population. This cohort of MS patients with TN presents a series of management challenges including poor tolerance of antineuralgic medications and occasional bilateral presentation. We analyzed our surgical series of MS patients presenting with TN who were treated with percutaneous radiofrequency rhizotomy to estimate the success, failure and recurrence rate of this procedure for those patients.

Surgical reports were retrospectively reviewed between the years 1996-2000. Patients with MS and TN who received a percutaneous rhizotomy during that time were included in the study and followed until the end of 2002. Data regarding age, sex, duration of MS and pain, response to medical treatment, pain distribution and surgical outcome were evaluated.

There were thirteen patients with MS and medically refractory TN treated with percutaneous radiofrequency rhizotomy. The average age at diagnosis for MS was 41 with TN beginning an average of eight years later. Following rhizotomy, complete pain relief without the need for any medication was achieved in 81% of the patients. The addition of medications resulted in pain control in the remaining patients. During a mean follow-up period of 52 months, there was a 50% recurrence rate. There were no complications related to the procedure and the associated facial numbness was well-tolerated.

Percutaneous radiofrequency rhizotomy is a safe and effective method for the treatment of TN in patients with MS. The unique susceptibility of this cohort to the side effects of antineuralgic medications may require early consideration of rhizotomy.

An altered breathing pattern in sleep, over two to three weeks, reported by the parents of a child on Vagal Nerve Stimulation (VNS) therapy for refractory epilepsy, prompted a sleep study in him. His polysomnography (PSG) revealed respiratory irregularity concordant with VNS activation. Dyspnoea is a well recognised and reported side effect of the VNS. However there are only a few studies looking at respiration in sleep with VNS. We therefore undertook PSGs in seven other children on VNS.

Sleep studies were undertaken, in accordance with standard clinical practice. Sleep and apnoeas and hypopneas were scored in accordance with conventional criteria. Respiratory pattern changes in sleep (RPCS) with VNS were looked for.

Respiratory pattern changes in sleep were seen during PSG in seven of eight children on VNS for refractory epilepsy. Decreased effort and tidal volume occurred in seven children, concordant with VNS activation. In one child, this was associated with a fall in respiratory rate, in the other six children with an increase. No study showed an apnoea/hypopnoea index in the abnormal range. The RPCS were not associated with significant hypoxia or hypercapnoea.

Our results suggest that RPCS occur in most children with VNS. This is not surprising in view of the significant influence vagal afferents have on respiratory control centres. The RPCS did not appear to have a clinical impact in our group. However further investigations are suggested to explore this phenomenon, especially in patients with sleep apnoea syndromes or compromised respiratory function.

To report on five patients with temporal lobe epilepsy (TLE) as the unique manifestation of multiple sclerosis (MS).

Among 350 consecutive MS patients, we identified 16/350 (4.6%) who also had epileptic seizures. Here, we review their electrophysiological and clinical features.

Five of these 16 patients (four female, one male; mean age 34.2 years; range 31 to 38) with MS and epileptic seizures had an extremely homogeneous clinical picture characterized by TLE as the unique manifestation of MS, even at long follow-up (mean: five years; range 4 to 10). In all patients, seizures started in the second or third decade. Brain MRI revealed at least one juxta-cortical lesion within the temporal region. Antiepileptic medication was always effective.

The present study provides the first evidence of a peculiar form of MS characterized by TLE as the unique manifestation of the disease with no disability or MS relapses at long-term follow-up.

Gerstmann-Sträussler-Scheinker disease is an autosomal dominant prion disease. The clinical features include ataxia, dementia, spastic paraparesis and extrapyramidal signs.

We report a new large Italian family affected by Gerstmann-Sträussler-Scheinker disease.

The four generation pedigree includes 11 patients. The mean age at onset ± SD was 41.4 ± 16.2 years. Mean disease duration to death in four patients was 5.5 ± 1.7 years. Two clinical patterns were evident: cognitive impairment with scarce neurological features or ataxia followed by cognitive impairment. Molecular analysis showed P102L mutation in PRNP gene.

Three Italian families have been reported to date. The variable phenotype has already been reported, and does not appear related to the codon 129 polymorphism.

The Consortium to Investigate Vascular Impairment of Cognition (CIVIC) is a Canadian, multi-centre, clinic-based prospective cohort study of patients with Vascular Cognitive Impairment (VCI). We report its organization and the impact of diagnostic criteria on the study of VCI.

Nine memory disability clinics enrolled patients and recorded their usual investigations and care. A case report form included all vascular dementia (VaD) individual criteria for each of four sets (National Institute of Neurological Disorders and Stroke (NINDS-AIREN), Alzheimer’s Disease Diagnostic Treatment Centers (ADDTC), the ICD-10 Classification of Mental and Behavioural Disorders (ICD-10), and the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV)) of consensus-based diagnostic criteria and for the Hachinski Ischemia Score (HIS). Investigators, having completed the case report form, were asked to make a clinical judgement about the cognitive diagnosis based on the best available information, including neuroimaging.

Of 1,347 patients (mean age 72 years; 56% women), 846 (63%) were diagnosed with dementia and 324 (24%) were diagnosed with VCI. The proportion of patients diagnosed with VaD by the diagnostic criteria was: 23.9% (n=322) by DSM-IV, 10.2% (n=137) by HIS, 4.3% (n=58) by ICD-10, 3.8% (n=51) by ADTCC, and 3.6% (n=48) by NINDS-AIREN. Judged against a clinical diagnosis of VaD, the sensitivity/specificity of each was: DSM-IV (0.77/0.80); HIS (0.41/0.92); ICD-10 (0.29/0.98); ADTCC (0.24/0.98); NINDS-AIREN (0.42/0.995). Compared with a clinical diagnosis of VCI, sensitivities were lower for the diagnostic criteria, reflecting the exclusion of patients who did not have dementia.

Consensus-based criteria for VaD omit patients who do not meet dementia criteria that are modeled on Alzheimer’s disease. Even for patients who do, the proportion identified with VaD varies widely. Criteria based on empirical analyses need to be developed and validated.

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by progressive ptosis, dysphagia and proximal limb weakness. The autosomal dominant form of this disease is caused by short expansions of a (GCG)6 repeat to (GCG)8-13 in the PABPN1 gene. The mutations lead to the expansion of a polyalanine stretch from 10 to 12-17 alanines in the N-terminus of PABPN1. The mutated PABPN1 (mPABPN1) induces the formation of intranuclear filamentous inclusions that sequester poly(A) RNA and are associated with cell death.

Human fetal brain cDNA library was used to look for PABPN1 binding proteins using yeast two-hybrid screen. The protein interaction was confirmed by GST pull-down and co-immunoprecipitation assays. Oculopharyngeal muscular dystrophy cellular model and OPMD patient muscle tissue were used to check whether the PABPN1 binding proteins were involved in the formation of OPMD intranuclear inclusions.

We identify two PABPN1 interacting proteins, hnRNP A1 and hnRNP A/B. When co-expressed with mPABPN1 in COS-7 cells, predominantly nuclear protein hnRNP A1 and A/B co-localize with mPABPN1 in the insoluble intranuclear aggregates. Patient studies showed that hnRNP A1 is sequestered in OPMD nuclear inclusions.

The hnRNP proteins are involved in mRNA processing and mRNA nucleocytoplasmic export, sequestering of hnRNPs in OPMD intranuclear aggregates supports the view that OPMD intranuclear inclusions are “poly(A) RNA traps”, which would interfere with RNA export, and cause muscle cell death.

Treadmill training is used for promoting rhythmical vigorous walking and for task-related training in patients with stroke. The neurological impact of treadmill training has not been established. The present investigation is aimed at (1) examining neurological changes over a four-week period after middle cerebral artery occlusion (MCAO) in rats and (2) assessing the impact of one-week, two-week and four-week treadmill training in MCAO rats.

Male Sprague-Dawley rats were subjected to 60-minute right MCAO. All rats were randomly assigned to one of seven groups. Infarct volume and neurological score were measured.

Rats sacrificed 24 hours post MCAO had the largest infarct volumes (171.4 ± 14.4 mm3) and the highest neurological score (median: 2, range: 1-3). We noted that without treadmill training, infarct sizes and neurological score diminished with time. Treadmill training for at least one week further reduced infarct volume and significantly improved neurologic function in MCAO rats.

Treadmill training after focal cerebral ischemia significantly improves neurological outcome in MCAO rats. Treadmill training may be beneficial for ischemic brain recovery.

Blockade of nonselective cation channels is a potential therapeutic approach that has not been attempted in cerebral ischemia, in spite of the ability of these channels to allow cellular calcium influx into neurons. Fenamates are a class of molecules that block these channels, and many congeners are also anti-inflammatory and free radical scavenging. These three mechanisms may contribute to brain damage in ischemia.

Pretreatment or posttreatment with mefenamate (30 mg/kg) was evaluated in a temperature-controlled rat transient focal ischemia model. Quantitative histopathology on 26 coronal sections allowed determination of tissue necrosis and tissue atrophy at one week survival.

Neither pre- nor postischemic administration of a dose previously shown effective in preventing epileptic neuronal necrosis was found to reduce necrosis in cortex, nor in any subcortical structures.

We conclude that nonselective cation channel blockade with mefenamate affords no neuroprotection in this model. Publication bias against negative studies exists in the literature, but we here report negative findings due to the multiple potentially positive actions of the drug. Closer examination of the effects of the molecule, however, reveals several potentially negative effects as well. We conclude there may be inherent weakness in pharmacologic monotherapy, even with molecules having protean potentially beneficial effects. This conclusion seems to have been borne out by the results of recent clinical trials.

Multiple brain abscesses are serious neurological problems with high mortality and disabling morbidity. The frequency is rising as a result of AIDS and the increasing number of immunocompromised patients.

A 59-year-old woman developed signs and symptoms of diffuse brain dysfunction including fever and neck stiffness. A brain CT scan demonstrated nine contrast-enhancing ring-shaped lesions. Analysis of the cerebrospinal fluid using PCR-technique revealed DNA of Fusobacterium nucleatum. Conservative treatment with antibiotics was successful. The patient recovered with only mild cognitive deficits.

The experience of our patient and the review of the literature indicate that multiple brain abscesses due to Fusobacterium nucleatum are rare. The most probable source is oral infection.

Multiple brain abscesses may be caused by Fusobacterium nucleatum. Cerebrospinal fluid analysis using PCR technique is helpful with diagnosis. Conservative management can be successful.

Spontaneous tumour regression in small cell lung cancer has previously been suggested in patients with paraneoplastic neurologic syndromes. Rare documentation of this event has occurred in the literature.

The authors report a patient with anti-Hu associated paraneoplastic sensory neuronopathy who had a spontaneous regression of her small cell lung cancer.

This case supports the hypothesis that anti-Hu neurologic syndromes are the consequence of a misdirected immune response to small cell tumours.

Dural cavernous angiomas are uncommon benign vascular malformations which may present intraoperative difficulties in hemostasis when the diagnosis is not suspected preoperatively. Preoperative diagnosis can be difficult when angiomas show atypical features and share imaging characteristics with other entities.

A patient presented with a radiographically aggressive lesion, subsequently identified as a dural cavernous angioma. The lesion is reviewed and its clinical, radiographic, and pathological features are compared with other vascular malformations.

A 40-year-old man presented with new onset seizures and an enhancing lesion infiltrating the floor of the right middle cranial fossa. Due to its aggressive radiographic appearance, initial considerations included chondrosarcoma, meningioma or metastasis. Pathological examination, however, revealed the lesion to be a cavernous angioma of dura.

This uncommon lesion may present a diagnostic challenge with significant intraoperative implications. T2 sequence hyperintensity in a relevant lesion should raise suspicion of an hemangioma. It is important to be aware of this entity and its potential to mimic other entities on radiographic grounds.