Here we assess the ability of patients with cerebellar disease to execute a simple visually-guided movement task involving tracking of a target with the index finger.

Spontaneous microdisplacements in index finger position are compared in patients with cerebellar deficits (ischemia [n = 3], multiple sclerosis [n = 3], degenerative cerebellar disease [n = 3]) and age-matched healthy subjects. Subjects were required to maintain a constant finger position relative to a stationary baseline displayed on an oscilloscope.

Unusual transient abrupt movements (saccadic or myoclonic-like) directed with or against gravity were seen in patients whose neurological deficits were the most severe (7/9 patients). These abrupt myoclonic-like movements occurred independently of visual input, were not associated with clinically observable myoclonus, and were not detected previously in patients with Parkinson’s disease. These abrupt myoclonic-like movements were not associated with abnormalities in either physiological tremor, or oscillations in finger microdisplacements induced by insertion of a delay (300–1400 ms) into the visual feedback of this finger “holding” experiment. An unexpected finding is that the results obtained for patients with cerebellar deficits by insertion of an experimental delay are not significantly different from those obtained with their age-matched controls.

These observations suggest that abrupt myoclonic-like movements are a characteristic abnormality of patients with a variety of cerebellar deficits and emphasize the value of this simple motor tracking task for characterizing movement disorders.

Two cases of sudden unexpected deaths in patients with multiple sclerosis were investigated by the Office of The Chief Forensic Pathologist.

Case studies.

Marked similarities of the deaths, including circumstances, scene examination, and autopsy findings including a complete drug screen were recorded.

Exposure to high ambient temperature may pose a danger to patients with multiple sclerosis. Mechanisms of death and the potential dangers to patients with clinically widespread disease are discussed.

Etiologic hypotheses for Parkinson’s disease have implicated environmental factors, genetic factors, or a combination of the two.

Data from a survey of elderly Canadians (n = 10,263) with regard to their history of Parkinson’s disease and previous environmental exposures were analyzed. Exposure to various environmental factors was compared between 87 patients with Parkinson’s disease and 2070 elderly controls without Parkinson’s disease.

Exposure to plastic resins (OR (odd ratio) = 8.79), epoxy resins (OR = 6.94), glues (OR = 4.26), paints (OR = 3.84), and petroleum (OR = 2.30) products was significantly (p < 0.05) associated with Parkinson’s disease.

These substances deserve further exploration with respect to the possible development of parkinsonism.

Some patients with reflex sympathetic dystrophy (RD) develop intractable symptoms unresponsive to conventional therapy. Recently, intrathecal morphine therapy has been used with some success in such patients.

The clinical course of two patients with intractable reflex sympathetic dystrophy (RSD) is described. Both patients developed intractable leg pain, swelling and autonomic changes after a leg injury. Numerous medical treatments and surgical sympathectomies failed to provide long term relief.

Relatively satisfactory symptom control was achieved only with the use of long term intrathecal morphine therapy delivered by subcutaneously implanted infusion pumps. Exacerbations of the RSD continued to occur, at times in association with further leg trauma, but these could be controlled by a temporary escalation of the intrathecal morphine dose. Complications of morphine therapy were relatively minor. A red rash appearing over the pump site was the first sign that a drug catheter break had occurred, necessitating surgical catheter revision.

Long term intrathecal morphine therapy is a useful treatment option for patients with intractable severe RSD who have failed other therapies and remain markedly disabled.

In animals and in humans, stress is known to be accompanied by increased β-endorphin secretion.

Blood samples from 47 patients in a state of stress induced by multiple trauma were assessed for β-endorphin concentration by radioimmunoassays.

We show that there is a clearcut correlation (Spearman’s R = 0.72, P = 2.1 x 10”-6) between the level of consciousness evaluated with the Glasgow score and levels of circulating β-endorphin. In addition, β-endorphin levels are higher than normal in patients with Glasgow coma with scores higher than seven, and lower than normal in those with Glasgow coma scores of seven or less. Finally, in the complete absence of stress (shown by the lack of brain activity in six irreversible coma patients), there is a severe drop in the level of circulating β-endorphin.

β-endorphin serum levels correlate with the state of consciousness of multiple trauma patients.

Stereotactic insertion of catherters into deep-seated tumors or developmental cysts is easily accomplished, but connecting the catheter to an Ommaya reservoir while maintaining catheter position can be difficult. We describe a technique for easy placement of a catheter-Ommaya reservoir construct with one pass.

Standard stereotactic imaging is performed. The distance from the outer table of the skull to the target point is measured. A catheter-Ommaya reservoir construct is assembled to this length and directed to the target position with a standard Cosman-Robert-Wells (CRW) stereotactic frame.

Use of this technique placed catheters into tumor or developmental cysts accurately and with no surgical complications in 12 patients.

This technique is simple, safe, reliable, and requires no special equipment. It avoids the risk of dislodging the catheter when it is being connected to the Ommaya reservoir, reducing the chances of cyst leakage and collapse.

The evaluation and treatment of Jefferson fractures, a burst fracture of the ring of CI, has been well documented in the medical literature. Vertebral artery injury associated with a Jefferson fracture is very rare.

The case study technique was used to summarize the case. Review of the literature was performed to discuss the case. Retrospective chart review of the 174 patients with cervical fractures admitted to St. Michael’s Hospital from 1989–1994 was also performed.

The case of a patient with a Jefferson fracture, with bilateral lateral displacement of the lateral masses causing bilateral vertebral artery occlusions resulting in a lateral medullary and cerebellar infarction is reported. A review of the literature is provided.

A high index of suspicion for this injury is paramount, especially in patients with multiple trauma, where the diagnosis of Jefferson fractures can be delayed.

Progressive deterioration and ensuing death following a neurosurgical procedure often represents a diagnostic challenge to the team responsible for patient care. Many, but not all, causes are treatable if a diagnosis is made early.

A 69-year-old woman who died 6 weeks post-operatively following a meningioma resection is reported. An initial routine post-operative course became complicated by progressive neurological deterioration 3–4 weeks later. Despite extensive investigation she died 6 weeks post-operatively without a diagnosis.

Autopsy demonstrated extensive Candida meningitis. A review of the literature demonstrates this to be a reported complication in high risk patients, difficult to diagnose, but treatable when identified.

Fungal meningitis should be high in the differential diagnosis in the post-operative patient with delayed, unexplained neurological deterioration, especially when associated with negative CSF cultures.

Tibial mononeuropathy due to compression by a synovial cyst is uncommon with fewer than 10 cases reported in the literature.

Case Study.

Tibial motor mononeuropathy with focal conduction block between the popliteal fossa and ankle was identified by electrophysiological testing. Magnetic resonance imaging and surgical exploration identified a synovial cyst compressing the nerve just distal to the popliteal fossa.

The combination of electrophysiological localization and imaging in cases of unexplained tibial mononeuropathy may identify an unexpected structural cause.

We have searched for amyloid substance in 41 consecutive samples of tissue taken during laminectomies performed to relieve spinal canal stenoses. We also evaluated the relation between the occurrence of deposits and the age of patients.

Tissue sections were stained with Congo red; positive areas had to show green dichroism under polarized light.

Small amyloid deposits were found in fibrous and fibro-elastic (ligamentum flavum) tissues in 14 subjects (34%). After age 60, the frequency was close to 50%. It reached 58% in those aged 70 or over. Our 3 patients aged 80 or more all had deposits. There was also a relation between age and importance of the infiltration. Both sexes appeared evenly involved. Systemic amyloidosis (multiple myeloma) was demonstrated in a single patient. In this case infiltration was slightly more abundant and exceeded ligamentary structures, also involving vascular walls.

Small amyloid deposits were frequent in our material. Their presence and their abundance were closely related to the age of patients. Rarely, a more important infiltration, exceeding ligamentary structures will perhaps suggest a systemic form of amyloidosis. A few other rare amyloid conditions (amyloidosis associated with prolonged dialysis, amyloid tumour) have been reported to induce spinal canal stenosis. The occurrence of amyloid in vertebral ligaments of non-stenosed elderly persons is not documented; it may represent a new localization of senile amyloidosis.

Neuroleptic Malignant Syndrome (NMS) is an adverse reaction to dopamine receptor antagonists, characterised by hyperpyrexia, extrapyramidal rigidity and impaired autonomic function. It might result from central dopamine receptor blockade that causes severe muscle contraction.

Case Study.

High dose intravenous therapy with the anticholinergic drug, procyclidine hydrochloride, temporarily diminished the muscle rigidity and reversed most of the autonomic features in a patient with NMS occurring after a single intramuscular dose of the dopamine antagonist metoclopramide. Paradoxically, however, the heart rate decreased and bowel movements increased with this atropine-like drug.

Since the degree of tachypnoea, tachycardia, and bowel hypotonia closely paralleled the severity of the muscle rigidity, it is suggested that these autonomic features of NMS result from sustained muscle contraction rather than a direct effect of neuroleptic drugs on the central nervous system.

Ependymomas usually arise from the ventricular surface.

We report an 11-year-old female who presented with a supratentorial ectopic ependymoma.

The patient presented with a two-month-history of progressive headache, nausea and vomiting. Examination revealed papilledema, horizontal nystagmus, diplopia on upward gaze, and right pronator drift. CT scan showed an enhancing left precentral subcortical lesion measuring 3 cm in diameter with associated edema and mass effect. Its medial border was located 3 cm from the ependymal surface of the ventricle. A firm tumour was dissected from the centrum semiovale white matter, and removed in toto as confirmed on MRI. Pathological examination revealed histological, immuno-histochemical and electron microscopic features consistent with an ependymoma. Spine MRI and bone marrow aspirate, as well as lumbar puncture of cytology failed to show any dissemination.

From the literature review, this represents an exceptional ependymoma located at the distance from the ventricular system or cisterns. Different pathogenic alternatives are discussed.

Neurenteric cysts are rare spinal lesions of congenital origin. They usually present insidiously with a long history of local spinal pain, radiculopathy and myelopathy. We report a 14-year-old male with a high cervical neurenteric cyst who developed a progressive myelopathy after minor neck trauma. Full recovery followed a partial cyst excision and decompressive procedure.

The possible pathogenic mechanisms for this unusual presentation include hemorrhage into the cyst, sudden mechanical compression from abnormal spinal movement of a chronically distorted and compressed spinal cord, or an increase in the size of the cyst secondary to accumulation of cyst fluid. In this case a small increase in the cyst size may have resulted in increased mechanical distortion and spinal cord dysfunction on a compressive and ischemic basis.

Herpes simplex encephalitis usually has a progressive cause. Sudden neurological deficits are unusual.

Case study.

A 17-year-old girl presented with an acute onset focal neurological deficit followed one week later by the more classical feature of altered level of consciousness, fever and focal seizures. The diagnosis of hepetic encephalitis was made by magnetic resonance imaging (MRI) and by the significant increase in cerebrospinal fluid titres of antibodies against herpes simplex type I.

Herpetic encephalitis should be considered in the differential diagnosis of acute stroke in young patients even in the absence of encephalitic features, if common etiological factors such as embolization and intracerebral bleed are excluded.