A 17th century B.C. Surgical Papyrus known as “The Edwin Smith Papyrus” was published in facsimile and hieroglyphic transliteration with translation and commentary by James Henry Breasted in 1930. The Papyrus was acquired by Edwin Smith in Luxor, 1862. This document was conceived in the Pyramid Age (3000-2500 B.C.) and remains in material form from the 17th century B.C. It is of importance to the history of Neurology as it contains the earliest mention in oriental literature of (a) the brain and meninges (b) calvarial and cervical vertebral injuries in details of pathology, symptomatology, treatment and prognosis and (c) functional localization in the brain and spine. Most importantly, Papyrus Smith is a statement of the medical ethic of its time.

Thirty cases of Alzheimer's disease and 30 age-matched controls were studied to determine the incidence of cerebral amyloid angiopathy and its relationship to age, neuritic plaque formation, and amyloid plaque content. Cerebral amyloid angiopathy (CAA) was present in 86% of AD cases and 40% of age-matched controls. Its frequent occurrence in AD is not merely a reflection of the advancing age of this group: it was seen only in the presence of neuritic plaques, regardless of age, and represents an integral component of AD. Neuritic plaques however, did occur in the absence of CAA in 17% of all cases. The amount of vascular and plaque amyloid tended to be of comparable severity in many cases, but significant discrepancies were observed, with preferential deposition of amyloid in either plaque or vessel. Our results suggest that neuritic plaque formation and amyloid deposition are linked genetically or etiologically, but independently expressed, without a cause-and-effect relationship.

A 40-year-old female with a recurrent mixed astrocytoma/oligodendroglioma was treated with intraarterial BCNU at six week intervals. Phosphorus magnetic resonance spectroscopy was performed before, and on two occasions after her third treatment.

Before treatment, phosphodiesters were 25% less than normal and intracellular pH was 7.14 (normal 6.97 ± 0.02). Eight hours following treatment phosphocreatine and phosphodiesters were reduced by ∼40% and pHi increased to 7.24. Thirty-two hours after treatment, phosphocreatine and phosphodiesters had reversed their decline, but pHi had increased further to 7.35. MRI and x-ray CT scans did not show any change during this period.

This study demonstrates that chemical changes can be observed in a glioma by magnetic resonance spectroscopy shortly after chemotherapy in a clinical setting and before changes are observable by imaging modalities. This approach evidently offers a possible means of monitoring the acute metabolic response of tumours to chemotherapy or other forms of treatment by a non-invasive repeatable quantitative method.

Fifty-one patients were enrolled in a double-blind, parallel group, multicentre study conducted to assess short-term efficacy and tolerance of bromocriptine (Parlodel®) or L-DOPA/carbidopa (Sinemet®) in patients never treated with amantadine, ergot alkaloids or L-DOPA-based drugs. An attempt to use the lowest effective dose was made. The responder rate for each group was approximately 78%; the mean daily dose for responders was 22.5 mg of bromocriptine or 250 mg of L-DOPA/carbidopa. The overall clinical improvement in each group was 62% (bromocriptine) and 55% (L-DOPA/carbidopa) for neurological assessment and 36% (bromocriptine) and 31% (L-DOPA/carbidopa) for functional disability. Comparison between groups did not show any significant difference for both neurological and disability assessments. The most frequent side effect was nausea (L-DOPA, N = 3; bromocriptine, N=6).

L'observation d'une poly-neuropathie associée à une cryoglobuline et à un syndrome sec est rapportée chez une femme âgée de 76 ans. La biopsie de nerf montre une démyélinisation très importante. Une vascularité est mise en évidence sur le prélèvement musculaire. La discussion envisage les neuropathies périphériques dans le cadre du syndrome de Gougerot-Sjögren et dans celui des cryoglobulines. Les traitements doivent être entrepris sans tarder et surtout les plasmaphérèses.

The incidence of primary intracranial tumors in Manitoba, Canada was reviewed. From 1980 through 1985,657 tumors were diagnosed. The crude incidence rates were 10.2/100,000 for males and 10.8/100,000 for females. The three most common tumors were: astrocytoma 281 (43%), meningioma 145 (22%), and pituitary adenoma 111 (17%). Average annual incidence rates for all tumors showed a bimodal distribution with one peak in the 0-4 age group (4.2/100,000), and the other in the 60-69 age group (27.2/100,000). For malignant astrocytoma, the age-specific annual incidence rate increased to the seventh decade where it reached a peak of 14.3/100,000. The incidence of benign astrocytoma remained relatively constant with age at 1.1/100,000. The annual incidence of meningioma increased with age up to the eighth decade reaching 7.2/100,000. Of the 145 meningiomas, 56 (39%) were meningotheliomatous, 48 (33%) transitional, 10 (7%) malignant, 7 (5%) fibroblastic, 6 (4%) psammomatous, 3 (2%) angioblastic, and 15(10%) lacked pathologic diagnosis. The annual incidence of pituitary adenoma showed two peaks, the first occurring in the third decade (2.6/100,000) and the second in the eighth decade (3.2/100,000). Although the incidence of meningioma was relatively high, the clinical features and pathologic patterns of these tumors were not unlike those previously reported in the literature.

We report the case of a 26 year old obese woman who presented with intermittent headaches and blurred vision in her left eye (OS) and on clinical examination had an enlarged visual field blind spot OS with OS disc edema. After an extensive neurologic work up including two nondiagnostic lumbar punctures, a clinical diagnosis of OS anterior ischemic optic neuropathy was made. Gradual progression of visual field loss OS prompted reassessment of the diagnosis and intracranial pressure was confirmed to be markedly elevated by usage of a subarachnoid monitoring bolt, thus establishing the diagnosis of pseudotumour cerebri. An optic nerve sheath fenestration was performed OS with subsequent reversal of the progressive visual field loss.

The neuropsychological findings in twin brothers with familial fatal Parkinsonism are reported. Post-mortem examination had shown extensive pathology in basal ganglia and brainstem, but not in the cerebral cortex. Although both showed average intelligence three months prior to death, they had impairment on a sorting task and in serial attention span. Some possible neural mechanisms are discussed.

A 60 year old man with chronic lymphocytic leukemia (CLL) developed a subacute neurological illness associated with multiple contrast enhancing lesions on CT scan. At autopsy large demyelinating lesions characteristic of progressive multifocal leukoencephalopathy (PML) were found in the right cerebral hemisphere surrounded by a dense leukemic infiltrate. The areas of contrast enhancement, highly unusual for PML, coincided with the CLL infiltrate.

Atypical forms of progressive multifocal leukoencephalopathy (PML) may simulate other disorders. A previously healthy 70-year-old female developed unsteadiness of gait, dysarthria, dementia and weakness leading to inanition and death from bronchopneumonia over a 43 month period. The diagnosis of PML was not suspected prior to death.

Neuropathologic examination of the brain disclosed characteristic findings of PML-deep bilateral cerebral demyelinative foci with enlarged gemistocytic astrocytes and swollen oligodendrocytes containing intranuclear inclusions. Electron microscopy identified papova virus particles within these inclusions. An underlying source of immunosuppression was not identified either premortem nor at the time of autopsy.

The prolonged clinical course, simulating that of a primary degenerative disease, and the lack of apparent immunocompromise are unusual features of PML and lend credence to the suggestions that variations in its expression and course are to be expected.

A 35-year-old woman who died after a long history of an illness consistent with Shwachman-Diamond Syndrome, was found to have extensive calcified necrotizing lesions confined to the pontocerebellar fibers of the basis pontis. The possible relationship of this recently described lesion to the patient's immunosuppressed state and/or other systemic metabolic disturbances is discussed.

We report the experience at a large teaching hospital over a 10 year period with Miller Fisher Syndrome, facial diplegia, and multiple cranial nerve palsies. In these patients, absence of drowsiness on examination, normal cranial CT scans, albumino-cytological dissociation on CSF examination and slowing of nerve conduction, all suggest that a peripheral nerve dysfunction is the underlying mechanism. Pertinent literature is reviewed, in an attempt to separate these probable variants of Guillain-Barré Syndrome from brainstem encephalitis, with which they may be confused.

Recent studies have reported that in abetalipoproteinaemia patients high dose vitamin E therapy may arrest or improve the neurological syndrome. Five patients with abetalipoproteinaemia have been followed since 1982, when all were started on high dose vitamin E therapy. Auditory brainstem responses (ABR), visual evoked potentials (VEP), and sensory evoked potentials (SEP) were recorded every six to twelve months. The vitamin E levels stayed below normal range in all patients; the neurological status remained relatively stable in most of the patients over the four years. The ABRs were consistently normal in all patients over the period of study. The VEPs improved in one patient with introduction of vitamin E therapy, and remained stable in the others, one of whom always had abnormal VEPs. The cortical SEPs were abnormal in all but the least affected patient and fluctuated in the two patients who also demonstrated some deterioration in neurological status. These results suggest that serial assessments combining neurological and neurophysiological studies provide important information in the follow-up of patients with abetalipoproteinaemia and that the SEP is the evoked potential best suited for the detection of the neurological changes in this disorder.

We report a patient with chronic, untreated idiopathic hypoparathyroidism who presented with papilledema and progressive deterioration of visual function. The papilledema resolved with treatment of the hypocalcemia. Visual acuity progressively improved as the serum calcium rose during treatment with vitamin D and calcium supplements. Lumbar puncture may also have contributed to the normalization of cerebrospinal fluid pressure and recovery of vision in this patient. The association of hypoparathyroidism and pseudotumor cerebri is rare, and a retrospective review of 41 patients with hypoparathyroidism admitted to two local general hospitals revealed no other cases.

This uncommon paroxysmal movement disorder featuring attacks of dystonic spasm with athetoid posturing is usually of idiopathic nature, often familial and starting in childhood. There are a few reported examples due to cerebral pathology, and only four previous cases due to brain trauma. We report here a 21 year old man with left-sided motor spasms, choreoathetoid in type, which were clearly caused by a right frontal penetrating injury with contusion and haemorrhage. The attacks were relieved by phenytoin therapy. In this case, it seems that the post-traumatic paroxysmal kinesigenic choreoathetosis may be a form of reflex epilepsy.

We report two patients with dyskinesia responding to antidepressants. The first is a 70-year-old man with depression, Parkinsonism and neuroleptic-induced tardive dyskinesia who presented with hysterical mutism. After recovery from the mutism, he was started on desipramine for depression. One week later the dyskinesia improved markedly. The second patient is a 61-year-old man with Parkinson's disease, dementia, depression and L-dopa-induced oro-lingual-facial dyskinesias. He was taking levodopa, trihexyphenydil and bromocriptine. The depression was treated first with desipramine and later with trazodone. The dyskinesia improved significantly on both drugs. The response of the dyskinesias to antidepressant medication may be due to the fact that antidepressants decrease beta-adrenoreceptor sensitivity and density which in turn may result in a diminished release of dopamine since beta-adrenoceptors mediate the noradrenaline-stimulated release of dopamine.

The anatomical substrate of eosinophilic fasciitis (EF) was studied in 15 muscle biopsy specimens of this disease, six of which included the dermis and subcutaneous tissue. As controls, 94 postmortem muscle specimens from patients dying of non-muscular diseases were used. Of these 94 specimens, 22 (23.4%) showed practically no deep fascia and 72 specimens showed a single dense bundle of collagen with no distinction between deep fascia and epimysium. The 15 specimens of EF showed thickening and inflammatory infiltration of varying degrees in the deep fascia, epimysium, perimysium, endomysium and also in muscle. We conclude that the anatomical substrate of EF is not confined to the deep fascia, but involves other structures including mysia and muscle itself. Most reported cases of EF in the literature do not even describe muscle. A comparative study of 15 biopsy specimens of polymyositis and dermatomyositis with those of EF revealed only quantitative differences in the histopathological changes of muscle and mysia, inflammatory infiltrate and eosinophilia. We suggest that the diseases are more closely related than previously recognized.

We report the occurrence of cerebral aneurysms and subarachnoid hemorrhage (SAH) in a family with Graves' disease (GD). Nine affected individuals across three generations have GD alone; three in the same generation have SAH without GD; and three others in the same sibship have both conditions. The familial association of GD and SAH has not been previously described. The occurrence of these disorders in individuals of the same family and their coexistence in the same individuals suggests a possible genetic determination for some cerebral aneurysms and may imply a genetic locus related to that of Graves' disease.

We recorded cerebral evoked potentials, back and forward averaged from the EMG onset of the responding muscle, in three reaction time tasks, each requiring an identical motor response to an identical stimulus but differing in the nature of the sensory discrimination required. Two types of stimuli were presented: a rare one to which the subject responded with finger-extension, and a frequent one to which no response was required. We found a close but variable relationship between the cerebral events associated with performance of a task and the timing of the motor response. As completion of the discrimination process was delayed relative to stimulus occurrence, EMG activity began later relative to the cerebral potentials. Moreover, we were able to record these cerebral events only from the response to the rare (unexpected) stimulus and not when subjects were required to respond to the frequent stimulus, suggesting that the sensory discrimination, in these experiments, is an event that occurred only in the processing of the unexpected stimulus.