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IN PREVIOUS studies* a characteristic pattern of protein changes was observed both in the blood serum and in the cerebrospinal fluid in about 80% of the patients with typical multiple sclerosis. The changes in these biological fluids examined simultaneously were termed the "protein profile." The variations in serum proteins, as determined electrophoretically,1 consisted of significantly decreased albumin and A/G ratio values, markedly increased alpha-2 and beta globulin fractions, a slightly elevated (or normal) gamma globulin fraction, and a normal alpha-1 globulin fraction. Similarly, the cerebrospinal fluid proteins in the same patients showed markedly elevated total protein and gamma globulin values, as well as increased gamma globulin-total protein (G. G./T. P.) ratios.The present study was undertaken to enlarge our case material for purposes of statistical analysis and to investigate changes of the "protein profile" in comparison with the clinical findings, over extended periods of time. Furthermore, the use of

Since Babinski's first description of the big toe phenomenon as a sign of disturbed function of the pyramidal tracts, many theories as to its mechanism have been propounded. In the available literature we were unable to find any of these theories that would be satisfactory—at least to us. In reading Babinski's original papers, one is struck by his failure to offer any explanation for the mechanism of his phenomenon.In routine neurologic examinations of cases of disease of the central nervous system, great variations are frequently noted in the response of the big toe to plantar stimulation. Among these are: Dorsiflexion of the big toe without stimulation, i. e., spontaneously, which is observed in diseases included in the so-called amyostatic symptom-complex (extrapyramidal system), the so-called chronic Babinski sign; spontaneous, constant, vermicular, slow, rhythmic movement of the big toe in dorsiflexion simulating an athetoid movement—this type of reaction may be designated

While no specific therapeutic agents have been introduced to retard the inexorable progress of amaurotic familial idiocy (Tay-Sachs' disease), adjunct measures, such as the antibiotics, have led in recent years to a protracted clinical course of this uniformly fatal infantile disorder. Thus, whereas medical texts of a few decades past mention an average life span of somewhat less than 18 months,1-3 the 50 children in the presently reported series survived to an average age of 28 months. No deviation in the early disease phases of these cases was forthcoming to suggest any fundamental variant from the classical disease. All the children were normal at birth, displayed arrest of development before the 10th month of life, and followed a pattern of continued clinical deterioration, including macular degeneration, amaurosis, peripheral paralysis, and hyperacusis.It was during the protracted aspect of the disease that certain features emerged which had not been associated

A number of publications dealing with electrophoretic serum protein patterns and quantitative cerebrospinal fluid (C. S. F.) protein determinations of patients with various neurological diseases have appeared in the literature. However, most data have been confined to multiple sclerosis (M. S.) cases.* In our previous studies † of 43 patients with typical multiple sclerosis, simultaneous determinations of the serum and C. S. F. proteins were performed. Significant changes, based on group statistics, were found in the following electrophoretic serum protein fractions: decreased albumin and A/G ratio, increased α2- and β-globulins, with slightly elevated or normal γ-globulins (γ-G). Chemical analysis of the C. S. F. proteins ‡ disclosed statistically elevated γ-globulin and/or increased γ-globulin/total protein (γ-G/T. P.) ratios, in keeping with the finding of Kabat and associates.§ The combined protein changes in both serum and cerebrospinal fluid were termed by us the "protein profile." †It is well known that protein

Multiple sclerosis is a disease characterized by remissions and exacerbations of symptoms. Thus, any considerations of therapeutic influences on the symptomatology must be subjected to critical analysis because of the natural fluctuations in the course of the disease. Recently isoniazid was reported as favorably influencing multiple sclerosis. In their preliminary report, Kurtzke and Berlin1 did not claim unequivocal effectiveness of isoniazid but suggested that their results warranted further investigation. Their patients were, in the main, persons who had recent exacerbations of rather short duration.This communication presents the results of the use of isoniazid in patients with multiple sclerosis of long standing. It was felt that a study of the effect of the drug on patients with persistent disabling features of the disease would be of special value for the evaluation of its therapeutic efficacy. In addition, a group of patients with other neurological disorders was selected for the

Spastic pseudosclerosis was first recognized as a clinical entity by Creutzfeld1 and Jakob.2 Characteristic symptoms are those of pyramidal and extrapyramidal involvement, muscular atrophies and a slowly evolving mental state, usually regarded at the outset as belonging to the neuroses. Anxiety, irritability and depression are early symptoms, followed by definite changes in personality. Mild sensory disturbances have been described.Three cases reported here are of unusual interest because of the familial incidence. This group, consisting of the cases of 2 brothers and a sister—1 of whom was previously described by one of us (Davison)—presented in life the clinical picture of spastic pseudosclerosis. The diagnosis was confirmed histopathologically in 2 of the cases. The fourth case in this presentation was of interest because of the sensory disturbances. REPORT OF CASES FAMILIAL TYPE.— CASE 1.— The case of J. R., a man aged 32, a brother of S. R., was

As an aid in localizing cerebral lesions, symptoms and objective manifestations play an important rôle. These must, therefore, be meticulously evaluated, for when obscure they cause confusion and lead to errors in localization. From time to time new signs are described, and varying degrees of importance are attached to them. In recent years the frontal lobe has been assigned a new function.The so-called reflex grasping or forced grasping and the synonymous terms used to designate these motor phenomena, such as "tonic innervation," Zwangsgreifen, "groping" and "pointing," have created considerable discussion.It is not the purpose of this communication to give an exhaustive review of the literature. An analysis of some of the recorded data is, however, essential.Wilson and Walshe,1 in describing the phenomenon, called it "tonic innervation." Patients with hemiparesis showed, on voluntary flexion and extension of the involved arm, a considerable delay in relaxation associated with

Although mapharsen has been widely adopted in the treatment of syphilis since 1934, reports of fatalities from toxic effects following its use are few. In 1933 Cole and others1 reported the first case of death attributed to mapharsen; acute nephrosis was the primary cause of death. Two years later Simon and Iglauer2 reported a case in which a patient had died of anuria after five injections of mapharsen. Shortly afterward Rein and Wise3 added 2 more cases with fatal outcome. In 1 of these aplastic anemia had developed; in the other granulocytopenia. In 1941 Kirkham and Perlmutter4 reported a case in which death was due to aplastic anemia following twenty injections of mapharsen.The first report of mapharsen therapy followed by involvement of the central nervous system appeared in 1939 from India. Rajam and Rao5 described a case in which hemorrhagic encephalitis followed the use

We recently observed a series of patients of unusual interest. They uniformly presented rapid development of motor and sensory signs with kaleidoscopic improvement or recovery. There was involvement of the motor and sensory components, at times in the nerve roots, at other times in the spinal cord substance, and in some of the cases in both the nerve roots and the cord. The similarity between all these cases led us to the opinion that there is a common etiologic agent. That this may be an infectious agent is probable, for in almost all of the cases the patients gave a history of a preceding inflammation of the upper respiratory tract. Ordinarily, each case taken by itself could have and probably would have been designated as an infective myelitis, and individually would perhaps have created no special interest. None of the cases can properly be labeled as purely neuritic. The rapidity