Amongst the many patients with persisting neck pain and headache following cervical injuries are a small number in whom the mechanism is compression of the second cervical nerve root and ganglion. This paper describes the clinical features in 14 patients seen by the author. The main features are unilateral pain in the upper cervical and occipital region, tenderness in the suboccipital region, and diminished sensation in the C2 dermatome. The anatomical basis for this syndrome is discussed and illustrated with dissections from a cadaver.

In 1949, amphetamine sulfate was replaced by propylhexedrine in the nasal decongestant agent Benzedrex® because of psychosis, sudden death, and widespread abuse. Propylhexedrine is not without risks, and reported cases of psychosis, myocardial infarction, pulmonary vascular disease and pulmonary hypertension, and sudden death are well documented in the medical literature. We are reporting 2 cases of definite brainstem dysfunction and 5 cases of transient diplopia secondary to IV abuse of Benzedrex®. This widely abused drug is prepared by heating Benzedrex® and hydrochloric acid, and the resulting crystals are dissolved in water for injection. This agent is called “stove-top speed”. All 7 patients had transient diplopia, within seconds after injection. One patient had evidence of a right-internuclear opthalmoplegia, and another had a depressed right gag reflex and paralysis of the right half of the tongue. The deficits in these two patients, persisted for many months. In young adults with history of drug abuse, the IV use of Benzedrex® should be considered in the differential diagnosis of transient or permanent focal brainstem deficits.

We report a 74 year old lady who presented with an exceedingly rare combination of an internal carotid artery aneurysm which was almost entirely intrasellar and associated with hypopituitarism but no neurological deficits. Such a lesion could be misdiagnosed as a pituitary tumour with serious consequences, if surgery is attempted without prior carotid angiography.

Patients with hereditary long QT disorders — Romano-Ward Syndrome and Jervell Lange-Nielsen Syndrome — sometimes present with seizures due to cardiac tachyarrhythmias. Two such patients are presented, emphasizing diagnostic clues — syncope, seizures with onset in early life, precipitation of attacks by emotional or physical stress, positive family history of sudden death and/or seizures, normal EEG and prolonged QT interval on ECG. Treatment is usually with adequate doses of sympathetic betablockers.

This paper describes select results of a longitudinal study of 62 mild to moderate Alzheimer's disease (AD) patients, in comparison to 60 age-matched healthy controls. Initial neurologic, radiologic, psychiatric, laboratory and cognitive examinations, required two full days, followed by one-day examinations at annual intervals. Of the total original sample, 31 AD patients and 39 controls could actually be followed for three annual examinations. Cognitive examination data confirmed cross-sectional (group discriminative) validity of memory and language measures, and showed the expected longitudinal deterioration in the AD sample, with controls maintaining consistent performance over the three years. However, those measures showing largest group differences at initial examination were not the best for tracking patient deterioration over time. Implications of these results for the selection of cognitive assessment measures are discussed.

A 14 year old boy with scapuloperoneal muscular atrophy, pes cavus, areflexia and distal sensory loss (Davidenkow syndrome) is described. Nerve conduction velocities were diminished. Sural nerve biopsy demonstrated a reduction in the number of myelinated fibers and early “onion-bulb” formation. These observations support the hypothesis that the scapuloperoneal amyotrophy associated with distal sensory loss may represent a variant of type I hereditary motor sensory neuropathy.

The study of Alzheimer's disease is hampered by insufficient knowledge of its cause. It can best be described as a syndrome whose clinical and pathological features, and their associations over time, need to be more carefully examined. Issues which impede our understanding of this syndrome include the lack of: (a) a singular “gold standard” for its identification; (b) longitudinal studies with appropriate comparison groups and neuropathological follow-up; and (c) standardized multifaceted clinical assessment procedures. Our awareness of the significance of these issues has led us to undertake a large-scale prospective, longitudinal investigation of 399 dementing and normal individuals at Sunnybrook Medical Centre. As a result of problems identified, it is proposed that research efforts across various Canadian centres be coordinated to best utilize available resources and expertise.

Bilateral facial weakness, diminished hearing and dysphagia developed in a patient with a large overdose of ethylene glycol. Our case and two others previously reported draw attention to the fact that cranial nerve dysfunction, especially bilateral facial palsy, may occur in addition to encephalopathy, coma and renal failure with ethylene glycol ingestion.

Two cases of malignant intracranial fibrous histiocytoma are presented. In Case 1 the tumour arose from the meninges and showed a disseminated spread throughout the neuroaxis. In the second case the tumour appeared to arise from within the brain substance. In this case surgical intervention and radiotherapy appeared to have achieved a cure, since no residual tumour was found at autopsy.

The tumours were examined using ultrastructural and immunohistochemical techniques, which appeared advantageous in delineating this rare tumour from other intracranial neoplasms.

Peripheral ischemia, secondary to ergotamine tartrate and caffeine suppositories is reported in a 48-year-old female. Lower extremity pre-gangrenous changes were unresponsive to surgical sympathectomy, calcium channel blockade, intra-arterial vasodilators and systemic anticoagulation. A dramatic clinical and radiological reversal of the vasospasm was obtained with intravenous sodium nitroprusside when surgical amputation appeared inevitable.

Scrapie affected brains exhibit a number of pathological features in common with the human neurodegenerative condition, Alzheimer's disease. The present report describes studies on chromatin structure seen in these two disease processes.

Chromatin associated proteins influence transcriptional activity of DNA through an effect upon chromatin structure. We examined chromatin structure by: (1) measuring the capacity of the enzyme micrococcal nuclease to release mono- and dinucleosomes from isolated nuclei and (2) measuring DN A-histone interactions by examining the effect of ambient tonicity upon the release of chromatin proteins.

In two strains of mice infected with two strains of scrapie agent there was reduced accessibility to micrococcal nuclease and an increased content on dinucleosomes of the histone HI and Hl° types. These changes precede clinical signs of scrapie and resemble those found in the human conditions of Alzheimer's and Pick's disease. Scrapie mouse brain differs from Alzheimer brain in that scrapie does not alter histone-DNA interactions as monitored by ionically induced histone release from chromatin. Despite similarities, the scrapie agent appears to operate upon different molecular mechanisms than those found in Alzheimer's disease.

We report a case of primary malignant schwannoma of the trigeminal nerve, and review the 5 previously reported cases. Clinical presentation, along with radiological and pathological features are discussed.

Swallow syncope is an often misdiagnosed rare disorder due to enhanced vagal tone during eating in patients with underlying esophageal and/or cardiac abnormalities. We present three cases of this disorder, one related to digitalis toxicity and the other two with diffuse esophageal spasm. The investigation, differential diagnosis, prognosis and management of swallow syncope are discussed.

A nine-year-old girl presented with right hemifacial spasm (HFS) of 27 months duration. Stimulation of the right supraorbital nerve produced synkinesis of the ipsilateral facial muscles. Latency and amplitude of the blink reflex were equal bilaterally. Other investigations including computerized tomography (CT) were negative. Treatment with carbamazepine was not helpful. This is the third reported case of HFS in childhood.

Studies of families located through a proband with dementia of the Alzheimer type have demonstrated transmission of the disorder within families, probably through shared genes. Increasing closeness of genetic relationship and increasing severity of illness are both associated with increasing risk to relatives. Families a.t high risk are especially valuable for studies of the biology of dementing illness including, in particular, their molecular genetics. Associations at several levels between Down's syndrome and Alzheimer's dementia provide important clues for molecular hypotheses.

Two French-Canadian families with seven cases of hydrocephalus in two generations are presented. The pattern of inheritance is consistent with an X-linked recessive transmission. The clinical and pathologic characteristics of this entity are reviewed. The anomaly of adducted thumbs was present in one patient and its cause is considered. The hypothesis of primary hydrocephalus and secondary compression of the aqueduct as the mechanism for aqueductal stenosis is discussed.

A 23-year old woman came to the emergency room with an isolated oculomotor nerve palsy (including pupillary dilatation) of rapid onset. Investigations and history revealed no cause. The subsequent course of events indicated a diagnosis of multiple sclerosis. While the third nerve has been shown to be involved during the course of multiple sclerosis, this is the first report of a case presenting as an isolated oculomotor nerve paralysis.