Franklin Robinson

Necrotizing encephalomyelopathy of childhood

AMONG THE LARGE GROUP of debiIitating diseases of the central nervous system occurring in children is a disorder of relatively recent description referred to as infantile, subacute, necrotizing encephalomyelopatfiy14 or encephalopathy.5-10 The etiology and pathogenesis of this disorder are unknown and, for reasons which we will indicate later, we prefer the designation necrotizing encephalomyelopathy of childhood, The diagnosis has been primarly an anatomical one, depending entirely on postmortem observations. Because of a certain resemblance of the lesions encountered to those seen in Wernicke’s disease, attention has been directed by some11J2 toward exploring a possible defect in thiamine metabolism as an explanation of the disorder. The purpose of this paper is to draw further attention to this disease, which we believe represents a definite clinicopathological entity, and to offer suggestions to obtain information helpful in elucidating etiology and pathogenesis of the disease. Two cases are presented with detailed neuropathological observations.

Cingulate-cerebellar mechanisms in the physiological pathogenesis of epilepsy☆

Abstract 1. 1. The effects of electrical stimulation of the anterior cingulate gyrus are described. 2. 2. Respiratory, motor and electroencephalographic effects are observed and include suppression and facilitation in each category. Respiratory effects consist of arrest and hyperpnoea. Motor effects consist of arrest of spontaneous motor movement and facilitation in the form of a jerk of the muscles of the face. With increasing intensity of stimulation, the jerking involves progressively the muscles of the neck, forelimbs, trunk and hindlimbs, in that order. 3. 3. Electroencephalographic suppression has been reported in detail previously. An after-discharge may also be produced by stimulation of the anterior cingulate gyrus but not of the pre- or sub-callosal areas nor of the posterior cingulate gyrus. Optimum parameters of stimulation are: frequency — 30 per sec., pulse duration — 11 to 16 msec. and intensity — 2 to 3 volts. The stimulus is applied for 10 sec. and the after-discharge persists 5 to 30 sec. after cessation of stimulation. It usually shows a characteristic 3 per sec. spike and wave configuration, although the rate may be 12 to 18 per sec. It is transmitted preferentially to the cerebellum where it may be amplified and at a faster rate. It is seen less often in the lateral cerebral hemispheres, has been recorded from the hippocampal formation, or may remain localized. 4. 4. Electrical stimulation of the uncus elicits local after-discharge which spreads preferentially to the temporal tip and posterior orbito-frontal areas, but may involve the anterior cingulate gyrus and cerebellum as well. 5. 5. Electrical stimulation of the cerebellum does not elicit local or transmitted after-discharge. 6. 6. Dilantin elevates the threshold of motor cortex to the point of unresponsiveness at maximal stimulation but does not alter the threshold of the anterior cingulate gyrus. Tridione elevates the treshold of the motor cortex and of anterior cingulate gyrus slightly. 7. 7. The similarities are reviewed between the effects of stimulation of the anterior cingulate gyrus and the clinical seizures of the petit mal triad. It is suggested that petit mal triad seizures are attributable to discharge in a system which includes the anterior cingulate gyrus and cerebellum as well as the anterior and mid-line thalamic nuclei. 8. 8. A functional integration in this system of the hippocampal formation and entorhinal areas afferent to it is suggested.

Dorsal Root Ganglionectomy for Intractable Monoradicular Sciatica

Dorsal root ganglionectomy was introduced in 1975 as a procedure with theoretical advantages over dorsal rhizotomy for the treatment of intractable radicular pain. Results of the few clinical reports have been generally favorable. The authors report the largest and most comprehensively studied series to date. 61 patients (33 men, 28 women) underwent dorsal root ganglionectomy for intractable sciatica, most often monoradicular. Patients were selected for surgery only if the clinical history, physical examination, radiologic studies, and diagnostic radicular block all predicted a favorable outcome. Sciatica was markedly reduced or eliminated in 36 patients (59%). Dysesthesia following ganglionectomy was observed frequently, but not invariably (approximately 60% of cases). Dysesthesia was generally of mild or moderate severity, self-limited, and responsive to treatment.

Controlled fractional pneumoencephalography; with particular reference to technic and the anatomy of the subarachnoid cisterns.

There has been no real standardization of pneumoencephalography in this country; this is true both of the roentgen technic and the method of gasfluid replacement. In most clinics, varying amounts of cerebrospinal fluid are drained from the lumbar subarachnoid space prior to the introduction of air or other gases. The total amount of gas injected may vary from 50–100 c.c, to complete replacement of the cerebrospinal fluid reservoir. In view of the relatively uncontrolled nature of many of these studies, Falk has aptly remarked that encephalographic examinations often reveal more about the examiner than they do about the patient (3). Two traditional objections to the use of encephalography in the diagnosis of expanding intracranial lesions have been (a) the risk of herniation of the cerebellar tonsils due to a rise in intracranial pressure and concomitant fall in lumbar pressure and (b) the frequent failure of ventricular filling, resulting in an unsatisfactory study. These objections are rightfully indictm...

Experimentally Induced Vascular Lesions of the Brain

The previous attempts to produce selective injury of the arterial wall by allylamine, utilizing intraarterial catheterization technics, have been limited to the study of the coronary and renal arteries (1–4). The present report represents the preliminary studies on selective injection of allylamine into the carotid and vertebral arterial systems. The ensuing allyl-amine-induced arterial lesions closely resemble some atherosclerotic lesions and are basically similar to those occurring in certain diseases of man. The plan of the investigation is to observe the neurologic effects in the experimental animal following selective intra-arterial injection of allylamine into the brain circulation. The progressive manifestations characterizing the allylamine-induced vascular lesions are evaluated by carotid or vertebral angiography and correlated with histopathologic studies. Material and Methods Six adult mongrel dogs of either sex with an average weight of 12–15 kg were given intravenous anesthesia of pentobarbit...

Chronic non-specific spinal epidural granuloma

The entity of chronic non-specific spinal epidural granuloma is presented. A brief review of the literature is appended together with personal experience with a series of four cases. The various radiological techniques and findings helpful in establishing a correct preoperative diagnosis are discussed.