Current recommendations for treating elevated blood pressure (BP) in the acute stroke are based largely on expert opinion and vary with regard to treatment thresholds and choice of antihypertensive agents. In this study we investigate the influence of these recommendations by comparing the management of hypertension in acute stroke at a tertiary care hospital with current guidelines.

Retrospective chart review of patients admitted with acute stroke at The Ottawa Hospital-General Campus over six consecutive months. The use of antihypertensive medications (type, dose, routes of administration, BP recordings) in the first seven days after admission was noted.

Transdermal nitroglycerin paste was the most commonly used antihypertensive agent. In contrast to the 15% reduction in BP over 24 hours recommended for lowering BP in hypertensive patients with ischemic stroke, nitroglycerin caused a >15% reduction of BPover the first 24 hours on 60% of the occasions used. Furthermore, despite concerns about sublingual nifedipine, this was the second most commonly prescribed agent. Surprisingly, the mean time to first BP measurement following initiation of antihypertensive therapy was 117 ± 43 minutes in ischemic stroke and 88 ± 89 minutes in hemorrhagic strokes.

The current guidelines for management of acute poststroke hypertension appear to have little influence on prescribing patterns, leading to considerable variations in practice. Such variations, likely due to uncertainty caused by lack of evidence from randomised controlled trials, are intolerable as patients maybe submitted to nonstandardised, potentially harmful care such as inappropriate choice of antihypertensives and inadequate BP monitoring as observed in this study.

The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out those tumors involving the optic chiasm only (chiasmatic tumors) from those also involving the hypothalamus (chiasmatic/hypothalamic tumors). The purpose of this study was: (i) to analyze the outcomes of chiasmatic and chiasmatic/hypothalamic tumors separately; and (ii) to determine the appropriateness of recommending radical surgical resection for the chiasmatic/hypothalamic tumors.

A retrospective chart review of all newly diagnosed tumors involving the optic chiasm from 1982-1996 at British Columbia’s Children’s Hospital was performed.

There were 32 patients less than 16 years of age, 14 with chiasmatic and 18 with chiasmatic/hypothalamic astrocytomas, with an average duration of follow-up of 5.8 years and 6.3 years, respectively. Ten of the patients with chiasmatic tumors and none with chiasmatic/hypothalamic tumors had neurofibromatosis I. Thirteen of the 14 chiasmatic tumors were managed with observation only, and none had progression requiring active intervention. For the chiasmatic/hypothalamic tumors, eight patients had subtotal resections (>95% resection), six had partial resections (50-95%), three had limited resections (<50%), and one had no surgery. There were fewer complications associated with the limited resections, especially with respect to hypothalamic dysfunction. There was no correlation between the extent of resection (subtotal, partial, or limited) and the time to tumor progression (average 18 months).

In conclusion, chiasmatic and chiasmatic/hypothalamic tumors are different entities, which should be separated out for the purposes of any study. For the chiasmatic/hypothalamic tumors, there was more morbidity and no prolongation of time to progression when radical resections were compared to more limited resections. Therefore, if surgery is performed, it may be appropriate to do a surgical procedure that strives only to provide a tissue diagnosis and to decompress the optic apparatus and/or ventricular system.

La fréquence de la dépression au cours de la maladie de Parkinson (MP) conduit à s’interroger sur les difficultés du diagnostic et sur les liens entre la dépression et l’histoire naturelle de la maladie.

Une série consécutive de 135 sujets atteints d’une MPidiopathique a été examinée sur le plan psychiatrique (DSM-III-R, échelle de dépression de Goldberg), neurologique (en distinguant les signes “axiaux” des autres signes parkinsoniens), neuropsychologique ( et en particulier tests frontaux).

Plus de la moitié des sujets ont une dépression qui apparaît plus fréquente dans les formes akinétiques et les formes fluctuantes de la maladie. Les sujets déprimés n’ont pas de déficit cognitif plus marqué mais leurs scores aux tests frontaux sont plus élevés. En outre les signes axiaux de la maladie (instabilité posturale, rigidité axiale) sont plus marqués chez les parkinsoniens déprimés, suggérant un lien entre la dépression et les lésions non dopaminergiques de la maladie. Alors que le ralentissement, les troubles de l’appétit et du sommeil, la fatigue peuvent être observés même chez des parkinsoniens non dépressifs, la séparation de la population parkinsonienne en plusieurs groupes montre que certains symptômes ne sont jamais rencontrés chez les parkinsoniens non dépressifs: il en est ainsi apparu que “l’impression que la vie ne vaut pas la peine d’être vécue”, “la perte d’espoir pour l’avenir”, “l’impression de ne pas être à la hauteur, de ne rien faire de bien”, “la baisse d’énergie”, “la tristesse matinale” caractérisent la dépression parkinsonienne. La dépression parkinsonienne revêt deux grandes formes cliniques. La première est la plus riche en manifestations somatiques: troubles du sommeil, fatigue matinale; elle correspond aux dépressions les plus sévères avec une absence d’espoir pour l’avenir , une perte de confiance en soi. La seconde est pauvre en manifestations somatiques alors que l’apathie y est très fréquente ainsi que le ralentissement.

Cette étude permet ainsi de préciser les symptômes de la dépression parkinsonienne qui doit être mieux reconnue pour être plus souvent traitée. Les liens entre la dépression et les signes axiaux de la maladie expliquent sans doute pourquoi la L-dopa et les agonistes dopaminergiques améliorent les signes moteurs de la dépression sans agir le plus souvent sur les manifestations dépressives.

Primary central nervous system lymphoma (PCL) is more frequently encountered by neurosurgeons given the increasing incidence among both nonimmunocompromised and immunocompromised patients. The most frequent surgery is stereotactic biopsy. Historically, radiation therapy has been the standard treatment modality for this disease and median survival was in the 15-month range. More recently, multi-modality therapy combining radiation therapy with chemotherapy (systemic, intrathecal, and/or intra-arterial) have resulted in longer survivals. We reviewed survival data for our series of patients treated for PCL over the last decade.

Thirty-four patients with histologically confirmed PCL were treated at our center. Multivariate Cox regression analysis was performed to determine which factor(s) (age, gender, HIV status, Karnofsky Performance Scale, chemotherapy, single modality therapy, histology, location, number of lesions, surgical resection) had a significant impact on survival.

The overall median survival was 19 months. Patients receiving multi-modality therapy (n=17) (chemotherapy and radiation) had a median survival of 34 months compared to four months for patients receiving single modality therapy (n=17 including seven HIV positive patients). Multi-modality therapy was the only significant factor affecting survival in this multivariate analysis (p<0.0001).

Chemotherapy plus radiotherapy significantly enhances survival over patients treated with single modality therapy alone. Quality of life issues should be addressed on a case by case basis as additional treatment modalities are initiated.

Six/second spike waves, 14 and 6/second positive spikes and small sharp spikes are apiculate paroxysmal rhythms in the electroencephalogram, thought to be of no diagnostic importance. The cortical origin of these discharges is documented in this report.

These waveforms were assessed in recordings from the surface of the cerebral cortex using implanted subdural electrodes in 61 patients monitored for possible epilepsy surgery.

Eight patients had 6/second spike wave, four had 14 and 6/second positive spikes and 3 had small sharp spikes. The 6/second spike waves were localised to the posterior cingulate gyrus, a more restricted region than would be predicted from scalp recordings and the 14 and 6/second spikes to the posterior mesial temporal cortex. Small sharp spikes were more widely distributed but also predominated in the posterior mesial temporal area. None of the discharges were congruent with the focus of seizure origin and no interaction with other interictal spikes was found.

These benign paroxysmal rhythms can appear incidentally in the electrocorticograms of patients with focal epilepsy and should not be confused with focal pathological spike activity or considered to be of any value in localizing the seizure focus.

Metachromatic leukodystrophy (MLD) is a genetic neurodegenerative disorder resulting from a deficiency of arylsulfatase A. Late onset forms are relatively rare. Central nervous system (CNS) involvement is characteristic at all ages.

A patient in her late 40s with peripheral neuropathy was assessed by EEG, evoked potentials, CTand nerve conduction studies. Nerve and muscle biopsy samples were investigated by electron microscopy. Arylsulfatase A activity in leukocytes and excreted cerebroside sulfate were determined. The arylsulfatase A gene was investigated for mutations using polymerase chain reaction (PCR) and DNAsequencing. The identified mutation was expressed transiently in African green monkey kidney (COS) cells to determine the effect of the mutation on arylsulfatase A activity.

Central nervous system functions were normal. Nerve conduction velocities were decreased. Sural nerve biopsy showed inclusions typical of MLD. Arylsulfatase A was less than 5% of normal. A homozygous mutation thr286pro was identified in the arylsulfatase A gene and demonstrated to be deleterious through transient expression studies.

Our patient has a progressive peripheral neuropathy but has apparently intact CNS function at her present age of 57 years. Biochemical, physiological and pathological findings are consistent with a diagnosis of MLD. A homozygous mutation, thr286pro, found in her arylsulfatase A gene, decreased enzyme activity to a level consistent with a late onset form of MLD.

Aging impacts microvessels in a number of tissue beds. Vasopressin acts as a vasoconstrictor in most blood vessels but may also cause vasodilation. We evaluated the role of aging and vasopressin in the regulation of nerve blood flow (NBF) in rat peripheral nerve.

We undertook a dose-response study to examine the impact of aging on resting NBF and its vasoreactivity to vasopressin. Nerve blood flow was measured using microelectrode hydrogen polarography. Argininevasopressin was administered both intra-arterially and topically.

In young adult rats (two months old) topical epineurial application of arginine-vasopressin produced a concentration-dependent reduction of NBF (ED50= 3.8 X 10-5 mol/L). Intra-arterial arginine-vasopressin also reduced NBF. Nerve blood flow was lower in aged rats (12 months old) and less responsive to topically applied vasopressin. The aging group had significantly higher concentrations of vasopressin in plasma than did the younger group.

The results suggest that vasopressin constricts vessels in peripheral nerve and that there is an age related decline in the vasoconstrictive response to vasopressin. There may be a reduction in receptor sensitivity in vascular smooth muscle cells in peripheral nerve with increasing age.

The pathogenesis of symptoms in normal pressure hydrocephalus (NPH) is unclear. One theory is that in the presence of enlarged lateral ventricles, the ventricular fluid exerts increased force on the ventricular walls, the cerebrospinal fluid pressure remaining normal. This is in accordance with Pascal’s principle of F = PxA. It has not been possible to obtain direct evidence for this view.

The opportunity to gain insight into the matter arose when it was observed that two patients with symptomatic NPH were relieved of their symptoms when they developed bilateral subdural collections, one after head trauma, the other after ventriculo-peritoneal shunting for NPH.

In each case, concomitant with the subdural collections and the reversal of symptoms, the ventricular system became smaller. In the first patient, symptoms returned when the subdural collections were resorbed. In the second patient, symptoms returned when the subdural collections became excessive.

Although documentation was less thorough than desired, it was concluded that relief of symptoms was related to the decrease in the ventricular size rather than lowering of the intraventricular pressure, thus providing evidence for operation of the principle F = PxA as the mechanism of symptoms in NPH.

Carotid angioplasty and stenting is gaining popularity as an alternative to carotid endarterectomy for the treatment of carotid bifurcation stenosis. The major concern with the procedure is the risk of embolic stroke which may be initiated by balloon angioplasty of friable atherosclerotic plaque. Elimination of angioplasty may result in a lower incidence of embolic complications.

We describe a case in which a self-expanding stent alone, without balloon angioplasty, was used to successfully dilate an atherosclerotic stenosis of the carotid bifurcation.

A moderate increase in vessel diameter, from 75% to 50%, was immediately observed after stent placement alone. No embolic complications were observed and follow-up plain film and ultrasound examinations showed progressive stent enlargement with excellent anatomic and hemodynamic results.

In this case of severe carotid stenosis, the use of a self-expanding stent alone, without balloon angioplasty, resulted in excellent anatomic and hemodynamic improvement.

Ifosfamide (IFX), an alkylating agent and isomer of cyclophosphamide, is used as a single agent or a component of multi-agent chemotherapy in the treatment of ovarian, testicular, head and neck cancers, sarcomas and lymphomas. Encephalopathy is manifested by cerebellar ataxia, confusional state, complex visual hallucinations, extrapyramidal signs, seizures, and mutism.

We report two patients with non-Hodgkin’s lymphoma presenting with mutism and confusional state after IFX infusion. Nonconvulsive status epilepticus (NCSE) as the cause of confusion was diagnosed on the basis of EEG pattern and the apparent improvement following intravenous administration of diazepam.

Electroencephalogram abnormalities during IFX treatment have been described but recordings are only available in six cases. In three of them, paroxysmal alterations warranted the diagnosis of NCSE; however, most cases of IFX encephalopathy might have associated NCSE.

This paper highlights the hemiparkinsonism-hemiatrophy (HPHA) syndrome as a unique presentation of the parkinsonian state. Clinically relevant diagnostic and treatment aspects are reviewed.

We report a case of HPHA, in a 21-year-old, otherwise healthy, woman. Clinical and radiographic features of our case are presented. We also review the current literature on the clinical, radiological and pathophysiological mechanisms of HPHA.

In our case, despite the lack of benefit from anticholinergics and dopamine agonists (the patient declined treatment with levodopa), the patient showed a dramatic improvement with subthalamic nucleus stimulator (STN) implantation. There are no reported cases of use of STN stimulator in HPHA.

Hemiparkinsonism-hemiatrophy is a distinct entity that may be clinically and pathogenetically different from idiopathic Parkinson’s disease; hence, HPHA needs to be considered as a possible syndrome in patients that have persistent unilateral parkinsonism. As medications are reported to be unhelpful in HPHA cases, early surgical intervention may be an option, such as in our case.

There are two case reports of patients who had proximal myotonic myopathy (PROMM) / myotonic dystrophy (DM) Type 1 and parkinsonism. The combination of myotonic myopathy and parkinsonism is so rare that it may appear to be just a coincidence. However, previous neuropathological examinations of patients who had myotonic dystrophy showed that there were intracytoplasmic inclusion bodies in the nigra and striatum, which raises the possibility that myotonic myopathy may be associated with parkinsonism. In this report we describe a patient with PROMM and a clinically definite parkinsonism to highlight this possibility.

A 65-year-old man developed proximal muscle weakness, myotonia and atrophy around the age of 55 and was diagnosed as having PROMM at the age of 62. Needle electromyography and muscle biopsy supported the diagnosis. A gene study of the DM Type 1 showed a normal CTG repeat length. At age 63, he developed rest tremor, bradykinesia, hypomimia, stooped posture, and gait disturbance. The postural instability worsened rapidly. The tremor and rigidity were much worse in his right side, where myotonia was more severe. Levodopa therapy was only partially effective.

This is a case report of a patient with PROMM that shows an association with a rapidly progressive form of parkinsonism. We suggest that this may be a novel form of a neurodegenerative disorder, which we name ‘Parkinsonism- Myotonic Myopathy-Complex’.

To describe a patient who presented with a hypoglossal nerve palsy caused by a cavernous malformation, review the literature on cavernous malformations associated with cranial nerves and the differential diagnosis of hypoglossal palsy.

Partial resection of the lesion was achieved and the diagnosis of cavernous malformation proven histologically.

Involvement of a cranial nerve by a cavernous malformation is very uncommon and the facial nerve is the example most frequently reported. This case report adds another possible site for this rare occurrence.