To compare mild vs. severe non-lesional temporal lobe epilepsy (TLE).

Data from 104 consecutive patients with non-lesional TLE were reviewed. Seventy-three of the 104 fulfilled the criteria for inclusion in this study of a follow-up period longer than three years at our Institute. Patients were considered to have a mild TLE if they were seizure free for at least three years after appropriate antiepileptic medication, or had rare (≤ 2/year) complex partial or secondarily generalized seizures for at least three years with or without appropriate antiepileptic therapy. Clinical, EEG and MRI data of mild vs. severe non-lesional TLE patients were compared on the basis of a cross-sectional study design.

Of the 73 patients with non-lesional TLE included in the study, 43 (59%) had mild TLE, and 30 (41%) had severe TLE. Duration of epilepsy was significantly shorter (mean 15.2 ± 10.5 years vs. 26.4 ± 13.2 years) and age at onset was significantly higher (mean 34.3 ± 15.3 years vs. 7.8 ± 6.8 years) in mild than in severe TLE group. Patients with mild TLE had also a significantly higher prevalence of positive family history of epilepsy (37.2% vs. 10%), and a significantly lower occurrence rate of febrile convulsions (FC) (4.7% vs. 33.3%), mesial temporal sclerosis (MTS) (6.9% vs. 36.7%), and intelligence deficiency (0% vs. 20%). In mild TLE there was also a significantly high rate (58.1% vs. 0%) of delayed diagnosis (from 1 to 28 years), because of misdiagnosis (39.5%) or no medical counseling (18.6%).

Mild non-lesional TLE is a common, unrecognized disorder mainly characterized by both onset in adulthood and high prevalence of familial history of epilepsy. The present findings suggest that mild non-lesional TLE may represent a clinical entity different from severe non-lesional TLE.

Unexpected exacerbation of seizures may occur following initiation of treatment with carbamazepine (CBZ). We reviewed the occurrence of such reactions in our patient population at a tertiary care children's hospital.

A retrospective analysis of our clinic database identified 129/691 (18.6%) patients with epilepsy treated with CBZ, as monotherapy. 38/129 children were later switched to another drug. In 11/38 (28.5 %) clinical and/or EEG deterioration was observed. Two patients identified at another institution with similar exacerbation were also included in our analysis. We report on the findings in these 13 cases.

Two groups were identified: Group I - 6 patients with normal neurological exam, normal EEG background, and a diagnosis of idiopathic generalized epilepsy. Group II - 7 patients with an abnormal neurological exam and/or abnormal EEG background. Following introduction of CBZ therapy, worsening of preexisting seizures, appearance of new seizure types, behavioral regression, and accompanying EEG deterioration were reported in both groups. Dramatic improvement in seizure control occurred, following withdrawal of CBZ and substitution of another anticonvulsant.

Physicians treating epilepsy must be aware that CBZ can exacerbate seizures, and cause developmental regression in children. Careful patient selection, when choosing CBZ as treatment, and prompt recognition of clinical deterioration and intervention, may help avoid or reverse these paradoxical reactions.

To evaluate the safety and tolerability of subcutaneous (s.c.) cladribine therapy in patients with chronic progressive multiple sclerosis (CPMS), and to evaluate the effects on lymphocyte subsets.

Cladribine, a synthetic antineoplastic agent with immunosuppressive effects, may favourably affect the course of CPMS. However results of a previous reported clinical trial showed significant myelosuppression in some patients.

19 patients with severe (mean extended disability status score [EDSS] = 6.7) CPMS were treated on a compassionate basis with cladribine 0.07 mg/kg/ day s.c. for 5 days per cycle, repeated every 4 weeks for a total of 6 cycles. Patients underwent clinical evaluation, EDSS, and hematologic analysis before, during, and following therapy.

The treatment was very well tolerated with no clinically significant side effects observed. Between baseline and the end of cycle 6, mean decreases were noted in absolute lymphocyte count from 1697 to 463 (p = 0.000012), CD4 count from 865 to 187 (p = 0.0000008), CD8 from 418 to 165 (p = 0.005) and CD19 from 197 to 26 (p = 0.000002). Platelet, granulocyte and RBC counts were unaffected. Approximately one year after completion of therapy, some recovery of CD4 and CD8 counts had occurred although both counts remained suppressed compared to baseline (302 and 227 respectively); the CD19 count had recovered essentially to normal by one year. EDSS scores post-therapy revealed some deterioration in 8 patients and stable scores in the remaining 11. Global patient evaluations of the treatment were mixed.

Cladribine therapy, at lower doses than previously reported, was remarkably well tolerated in CPMS, with no significant myelosuppression. Profound effects occurred in total lymphocyte count and CD4, CD8 and CD19 subsets.

To evaluate, in a double-blind fashion, the efficacy of deep brain stimulation of the internal segment of the globus pallidus (GPi) contralateral to a previous unilateral medial pallidotomy (MP).

This pilot study involved 4 patients with a previous MP and one previously unoperated patient unable to tolerate any antiparkinsonian drugs. One of the patients with a prior unilateral MP had two electrodes implanted in the contralateral side, one in GPi and one in Vim thalamus. Detailed neurologic assessments were performed after overnight drug withdrawal and in the drug “on” state at baseline, 1 week and 3 months (in all), and 9 and 12 months (one) with patients and evaluators blinded to the status of stimulation.

GPi stimulation resulted in improvements in “off-period” contralateral bradykinesia, rigidity and tremor in all patients. Dyskinesias and freezing episodes were ameliorated in one patient each but dyskinesias were transiently induced in another. The patient with GPi+Vim electrodes had complete resolution of contralateral tremor with thalamic stimulation but less benefit from acute GPi stimulation. One patient experienced a single seizure one week post-op and no other surgical complications were observed.

Deep brain stimulation can be applied safely and effectively in patients who have already had a pallidotomy on the contralateral side. The effect of stimulation at different sites on different symptom profiles and levodopa-induced dyskinesias requires further evaluation.

Constructional impairment following left vs. right hemisphere damage has been extensively studied using drawing tasks. A confounding factor in these studies is that right-handed patients with left hemisphere damage (LHD) are often forced by weakness to use their non-dominant (left) hand or hemiparetic dominant hand. Qualitative differences in the drawing characteristics of left and right hand drawings by normal subjects have not previously been characterized. The present study was undertaken to determine the qualitative differences between left and right hand drawings of normal subjects.

Thirty right-handed, elderly subjects without a history of neurological disease were asked to draw, from memory, seven objects using the right and left hand. Half of the subjects were randomly assigned to draw with the left hand first, and half the right hand first. Right and left hand drawings were compared using a standardized scoring system utilized in several previous studies of drawing in focal and diffuse neurological disease. Each drawing was scored on eighteen criteria. Right and left hand drawing scores were then compared using the t-test for paired samples or the Wilcoxon matched-pairs test

Drawings made using the left hand were found to be significantly simpler, more tremulous and of poorer overall quality than drawings made by the same subjects using the right hand.

The deficits found in left versus right hand drawings of normals are similar to those found in patients with LHD, suggesting that much of the drawing impairment seen following LHD is due to an elementary motor disturbance related to use of the non-dominant hand.

Dissection of the internal carotid and vertebral arteries is a well recognized cause of stroke, especially in the middle-aged. The exact etiology of this condition is controversial. According to one theory there is an underlying vasculopathy originating from disturbed development of the neural crest. The neural crest gives rise to several tissues, including the tunica media of large cervical arteries and the outflow tract of the heart. We attempted to test the theory that developmental abnormality at the level of the neural crest may play a role in dissection of the large cervical arteries.

We designed a retrospective case control study. By means of transthoracic echocardiography we measured the aortic root diameter in a group of patients with radiographically determined dissection of at least one large artery in the neck. The results were compared to a control group.

In comparison to age matched controls, male patients were found to have a significantly larger aortic root. Although a similar trend was apparent in females, the difference between the patient and control group of females was not statistically significant.

Patients with cervical artery dissections may have other abnormalities in organs arising from the neural crest. A larger prospective clinical study and further research are needed to establish a firm link between dissection of the cervical arteries and abnormalities in other organs.

Segmental hyperhidrosis is an uncommon finding which is usually associated with irritation or infiltration of pre-ganglionic sympathetic fibres or the sympathetic chain.

We report two cases of segmental hyperhidrosis with striking clinical features.

In one case, a mesothelioma produced ipsilateral simultaneous underactivity and overactivity of sympathetic outflow and in the other case a thoracic central disc herniation was probably responsible for a band of sweating which clearly extended beyond the segmental level of injury.

Segmental hyperhidrosis should trigger a search for structural disease in the spinal and paraspinal region.

Since few studies have examined the effectiveness of therapies for subcortical vascular dementia, treatment guidelines are not available. Current patterns in the treatment of such dementias have not been studied.

To determine the practice patterns of Canadian specialists for the treatment of subcortical vascular dementia, and to survey their opinions regarding issues which are important in the design of a randomized controlled trial (RCT) in this field.

National survey of all specialists certified in Neurology or Geriatric Medicine.

Of responding physicians (78%) prescribed antithrombotic therapy for patients with vascular dementia. Most begin treatment with aspirin 325 mg daily (64%). The next three most common initial treatments were; no pharmacotherapy (12%), aspirin 650 mg daily (11%), and aspirin 1300 mg daily (11%). If the dementia continued to progress despite initial therapy, the treatment options were more varied. Most specialists (69%) believed that an RCT to assess the efficacy of aspirin in vascular dementia is warranted. The majority (69%) also felt that serial neuroimaging would be required for participants in such a trial, with magnetic resonance imaging being cited most frequently (41%). The majority of specialists considered three years as the minimum durationb for such a trial.

Specialist physician practice patterns vary significantly for the treatment of patients with subcortical vascular dementia. Most physicians believe that an RCT testing the efficacy of aspirin in this condition is required. However, before such a trial can be conducted, many methodological difficulties need to be addressed.