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Despite the involvement of cerebellar ataxia in a large variety of conditions and its frequent association with other neurological symptoms, the quantification of the specific core of the cerebellar syndrome is possible and useful in Neurology. Recent studies have shown that cerebellar ataxia might be sensitive to various types of pharmacological agents, but the scales used for assessment were all different. With the long-term goal of double-blind controlled trials-multicentric and international-an ad hoc Committee of the World Federation of Neurology has worked to propose a one-hundred-point semi-quantitative International Cooperative Ataxia Rating Scale (ICARS). The scale proposed involves a compartimentalized quantification of postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders, in order that a subscore concerning these symptoms may be separately studied. The weight of each symptomatologic compartment has been carefully designed. The members of the Committee agreed upon precise definitions of the tests, to minimize interobserver variations. The validation of this scale is in progress.

The distribution of two calcium-binding proteins, calbindin D28k (CaBP) and parvalbumin (PV), was investigated by immunohistochemistry in the brains of three individuals dying of nonneurologic illness and three patients with spinocerebellar ataxia-1 (SCA-1). SCA-1 has recently been proven to be due to an unstable CAG repeat mutation on chromosome 6. In the cerebellum of control individuals the Purkinje cells showed strong immunoreactivity to CaBP. Other cells were CaBP-negative. Parvalbumin was highly localized to Purkinje, basket, stellate, and Golgi cells. All surviving Purkinje cells in SCA-1 were strongly immunoreactive to CaBP. The number of PV-immunoreactive Purkinje cells was markedly reduced in SCA-1. In addition, there was a significant decrease in the intensity of PV immunostaining within the individual Purkinje cells compared with controls. However, in the hippocampus, temporal cortex, and lateral geniculate scattered PV-positive neurons were seen in SCA-1 patients, similar to those in controls. The present results suggest that the decreased PV-immunoreactivity in the surviving Purkinje cells in SCA-1 may reflect biochemical alterations preceding Purkinje cell degeneration.NEUROLOGY 1996;47: 249-253

The dominantly inherited ataxias are characterized both by phenotypic variability (phenotypic heterogeneity) within the same genotype and overlapping phenotypes from different genotypes (genotypic heterogeneity). Therefore it is important to characterize specific clinical-neuropathologic phenotypes as precisely as possible at the genetic level. We describe a family with dominantly inherited ataxia of late adult onset with relatively "pure" cerebellar signs. Neuropathologic examination in two individuals from this family revealed findings consistent with cerebello-olivary atrophy, suggesting that this neuropathologic phenotype many run true within f families. Mutations at the spinocerebellar ataxia-I, Machado-Joseph disease, and dentatorubropallidoluysian atrophy loci were excluded by direct DNA analysis on the leukocytes of one living affected member. Thus we provide evidence that these mutations are not responsible for this particular phenotype of dominantly inherited ataxia.

We examined insulin-like growth factor I (IGF-I)-dependent phosphorylation and protein tyrosine kinase (PTK) activity in cerebellar cortex of normal humans, patients with olivopontocerebellar atrophy (OPCA) ("C" kindred) and in lurcher mutant mouse, a suggested animal model for OPCA. PTK activity and IGF-I-dependent protein tyrosine phosphorylation was significantly reduced in cerebellar cortex of human OPCA patients as compared to the normal controls. Immunoblot analysis also demonstrated a decrease in cerebellar 80 kDa phosphotyrosine protein in these patients. By autoradiography, IGF-I receptors were localized in the molecular layer of 30-day-old control and lurcher mutant mice cerebella. However, the lurcher mutant mice showed a decrease in [ 125 I]-IGF-I binding in the molecular layer as compared to the littermate controls. The IGF-I receptor autophosphorylation was also markedly reduced in 15-day- and 22-day-old lurcher cerebella. These results suggest that the process of cerebellar degeneration in human OPCA and lurcher mutant mouse may be associated with altered IGF-I receptor binding and protein tyrosine phosphorylation.

Dominantly inherited ataxias resulting from different gene mutations are difficult to distinguish based on clinical phenotypes. We believe the phenotypic variability within families can be a clue to clinical diagnosis. We illustrate the range of phenotypes extending from levodopa-responsive extrapyramidal disease to more purely ataxic syndromes seen in two families with molecularly proven Machado-Joseph disease.

Epidemiological data have not yet enabled physicians to look beyond age and race to identify men at increased risk for prostate cancer. We conducted a hospital-based case-control study of familial patterns of prostate cancer with self-reported data from a risk-factor questionnaire. There were 385 patients with histologically confirmed prostate cancer, and 385 race and age-matched (+/- 5 years) controls with other cancers. Family history, available for 378 patients and 383 controls, was positive for prostate cancer in 13.0% versus 5.7%, respectively. The difference was significant at p = 0.01. The over-all age-adjusted risk estimate for men with a first-degree relative with prostate cancer was significantly elevated (odds ratio of 2.41), as were the individual risk estimates for having a father or brother with prostate cancer (odds ratio of 2.24 and 2.66). Having a second-degree relative (grandfather or uncle) with prostate cancer also conferred elevated but not statistically significant risk. These data accord well with the few previously published case-control studies of familiarity of prostate cancer. On the basis of these findings, one should consider recommending participation in early detection programs for prostate cancer in a man whose father or brother has had the disease.

To the Editor: The editorial by Rosenberg [1] is a superb analysis of the current genotype situation in the field of the hereditary ataxias. Rosenberg is quite right in his comments that the genotype has great importance in clarifying the issue of correlation …

We describe a progressive neurologic disorder in three sisters characterized clinically by palatal myoclonus, spastic weakness, hyperreflexia, mild cerebellar dysfunction, and ocular motor abnormalities.Postmortem examination of one patient demonstrated widespread Rosenthal fiber deposition associated with demyelination. The father previously was reported to have similar pathologic findings and carried a clinical diagnosis of multiple sclerosis. These clinical and pathologic findings describe a rare familial leukodystrophy that corresponds most closely to cases reported as adult Alexander's disease. Although similar pathologically to the well-characterized infantile variant of Alexander's disease, it is not known whether this adult variant represents the same disease process.NEUROLOGY 1995;45: 2266-2271

How cana medication work on one side of the Rhine and not the other? The articles by Trouillas et al 1 and Wessel et al 2 in this issue of theArchivesillustrate in many ways the causes and types of disagreement about the effect of an experimental drug on a constellation of symptoms. The levorotatory form of hydroxytryptophan (l-5-hydroxytryptophan), a serotonin precursor, has been used for a decade or more by Trouillas et al 1 and others in an attempt to treat ataxia of various causes. Studies have evolved from early unblinded attempts to these two double-blinded studies. 1,2 Trouillas and coworkers originally found the most benefit in treating those patients with noninherited ataxia. In the present study, 1 they concentrated on 19 persons with Friedreich's ataxia from 11 centers, whereas the study by Wessel and coworkers 2 included 39 patients from three centers: 19 with Friedreich's

I heldup a tablet from my breakfast plate and asked Marilyn what it was. "A new brand of vitamin," she replied. I commented that the drug firm should fire their marketing staff for putting a black vitamin pill on the market. She glanced at me, "It's not black, it's dark red." The instruction on the handle of my weed eater says in yellow print, "Before removing weeds wound around the shaft, - - - - - the motor." Thinking that a strange instruction I looked more closely and with better light. The words "turn off" in dark red against the black background were nearly invisible. Small blinking red lights on equipment often escape my attention, so I burn out fuses on my radio transmitter tuner and fail to push the button on the gas pump. It was in the first or second grade that I lost my purple crayon in the dark depths of

To determine whether the cognitive status of patients with dominantly inherited spinocerebellar ataxia (DSCA) might be related to neurologic severity, we administered a comprehensive neuropsychological test battery to 43 patients with DSCA, ranging in ataxia severity from mild to end-stage. As compared with the controls, the mildly ataxic patients scored normally or close to normal as a group on all of the neuropsychological tests. In contrast, approximately one-half of the moderately and all of the severely ataxic patients showed poor performance, independent of age, Hamilton Rating Scale for Depression score, or education, on the Wisconsin Card Sorting Test, suggesting impaired executive system function. In addition, a subgroup of these patients had a neuropsychological profile suggestive of mild generalized cognitive impairment. We conclude that DSCA is not a homogeneous group of disorders with respect to cognitive status and that the neurologic severity of the disorder is a major factor. Impaired executive system function could be explained by damage to olivopontocerebellar system control over cerebral cortical function or to damage to other neuronal systems (especially cholinergic) that degenerate in parallel with the olivopontocerebellar system.

An 18-month double-blind treatment of multiple sclerosis with low dose oral methotrexate showed it to be well tolerated and suggested effectiveness in exacerbating-remitting MS but not in the exacerbating progressive and chronic progressive stages.

We examined protein kinase C (PKC) activity and inositol 1,4,5-trisphosphate (InsP3) binding in frontal cortex (FC) and cerebellar cortex (CC) of normal humans, patients with dominant ataxia (“C” kindred) and in Lurcher mutant mouse brain (LMB), a suggested animal model for olivopontocerebellar atrophy (OPCA). PKC activity and [3H]InsP3 binding were decreased in CC of human OPCA by 70% and 90% respectively. The decreases were specific to CC as there were no changes in FC. PKC activity and [3H]InsP3 binding in cerebellum (CB) of LMB were similarly decreased as compared to normal littermate controls. The LMB decrease of PKC and [3H]InsP3 binding was evident on the 15th day of age, the day of onset of ataxia. InsP3-mediated calcium release was also decreased significantly in the cerebellar microsomes of 25-day-old LMB and human OPCA when compared with their respective controls. These data indicate that the decrease of second messenger linked PKC activity and InsP3 receptor binding in CB may be a biochemical marker that reflects neuronal degeneration in dominant cerebellar ataxia.

• James Parkinson, a general physician, was interested in all facets of medical care, and wrote two books for the lay public that contained comments on diverse things. He may have been one of the first physicians to publicly describe child abuse. Some of his advice to the public was of a neurologic nature, and included directions on the diagnosis and treatment of pseudoseizures, epilepsy, drowning, and stroke.

Parkinson's introductory paragraph, perhaps the most famous disease description in neurology reads: "Involuntary tremulous motion, with lessened muscular power, in parts not in action and even when supported; with a propensity to bend the trunk forwards, and to pass from walking to a running pace: the senses and intellects being uninjured." 1 Most great ideas come from somewhere or someone. What or who started James Parkinson's thinking about the difference between tremor with motion and tremor at rest? Do the references in his essay tell us? Did he receive his stimulus from those he quoted, the Dutchman, Franciscus Sylvius de le Boe, or the Frenchman, Francois Boissier de la Croix de Sauvages, or from Galen, a Greco-Roman, all three ofwhom he read in Latin? Let us look at Parkinson's quotes. Sylvius de le Boe (1614-1672), a perceptive Dutchman who gained fame for introducing bedside teaching in Leiden, the Netherlands, wrote

Trouillas et al 1,2 have reported beneficial effects in the treatment of ataxia of varied origins with the dextrolevorotatory form of hydroxytryptophan (dl-5 hydroxytryptophan) (DL-5HTP) and the levorotatory form of hydroxytryptophan (l-5 hydroxytryptophan), (L-5HTP), serotonin precursors. The ataxias lack effective therapy. 3 We treated six patients with hereditary ataxia using the L-5HTP for 1 year. Of the six patients, three were affected with Friedreich's ataxia, two patients with adult-onset dominant ataxia (Marie's, olivopontocerebellar atrophy), and one patient with adultonset recessive ataxia with retained reflexes. 4 The duration of disease in the four males and two females, aged 18 to 50 years, ranged from 3 to 20 years. Four patients were ambulatory and two, both with Friedreich's ataxia, were in wheelchairs. See also pages 449, 451, and 456 After approval by the Food and Drug Administration (Washington, DC) and the Institutional Research Board and with the patients' agreement, treatment

The fact that five books on ataxia have appeared in the last year and a half must mean something, but what? And what does the ataxia researcher clinician do faced with $900 worth of new books? Well, one or two of the five decisions are made easier by the focus of the volumes. The book on serotonin is for those who are interested in that particular neurotransmitter and its possible use as a treatment for ataxia via a precursor L5-hydroxytryptophane (L5HTP). It is a report of the 1991 Lyon, France, conference on serotonin and contains a thorough study of the raphe nuclei, serotonin, and the cerebellum and the rationale of L5HTP treatment of ataxia. This book will not, however, solve the question of the effectiveness of that treatment, since the last word remains to be said. But for those interested in brain-stem and cerebellar neurotransmitters, the book is a must.

Having survived for 29 years as the chief or chairman of neurology in one location, which may be at this moment the record among active chairmen, and appreciating that there is not a great clamor for positions of departmental leadership in neurology, especially in small medical schools, and realizing that the worries of McKhann 1 at The Johns Hopkins University School of Medicine, Baltimore, Md, and Ringel 2 at the University of Colorado School of Medicine, Denver, are different from mine in Mississippi, the urge to comment as I retire is hard to resist. In the first place, boys and girls, it is not too hard. All you need is the skin of a rhinoceros, the patience of an ant colony, the optimism of Pollyanna, the balance of a Wallenda, the willingness of Hercules, the morals of a vestal virgin, and the humor of Rodney Dangerfield. You have to convince

Reply from the Authors: We appreciate the comments of Drs. Honnorat and Trouillas and the opportunity to study and to be aware of their very unusual and interesting familial cases of adult-onset Alexander's disease …

In the 1960s a belief no longer current suggested that measles might be a cause of multiple sclerosis (MS). 1,2 After measles vaccination began in the United States in 1963 the incidence and prevalence of measles dropped precipitously, with a decrease in the incidence rate from 200 per 100 000 in 1963 to 10 in 1967 and a decrease in measles deaths from 700 in 1963 to 25 in 1968. 3 Has MS decreased as a result? Since the median age of onset of MS in our clinic is 28 to 30 years, now would be a useful time to study the birth dates of our patient group with MS. Over a 34-month period (April 1992-February 1995) 198 consecutive individuals seen by one of us (R.D.C.) at the University of Mississippi Multiple Sclerosis Clinic, Jackson, were questioned about date of birth and history of measles and measles vaccination. Verification of

Three cases of bilateral ptosis with relatively normal ocular motility are presented. In two of the patients, neuroimaging demonstrated lesions in the region of the third cranial nerve subnuclei. These unusual clinical presentations are due to isolated involvement of the central caudal nucleus supplying the bilateral levator muscles, with, virtually complete sparing of other third cranial nerve structures.

We report clinical and pathologic findings from two kindreds afflicted with a familial form of progressive subcortical gliosis. The disorder segregated as an autosomal dominant trait. Onset was in the presenium and the course was slowly progressive. Affected individuals initially manifested personality change, degeneration of social ability, disinhibition, psychotic symptoms, memory impairment, or depression. Later, all developed progressive dementia, frequently associated with verbal stereotypy, decreased speech output, echolalia, or manifestations of the human Klüver-Bucy syndrome. Terminal clinical manifestations included profound dementia, frequently with mutism, dysphagia, and extrapyramidal signs. Autopsy of seven end-stage patients revealed generalized cerebral atrophy, predominantly involving the white matter of the frontal and temporal lobes. Microscopically, prominent fibrillary astrocytosis was present in the subcortical white matter and in the subpial and deep layers of the overlying cerebral cortex. These changes were most pronounced in the frontal and temporal lobes, especially in the cingulate gyri and insulae. Mild cortical neuronal loss accompanied the gliosis, but no myelin loss was evident. The claustra and substantia nigra also showed severe astrocytosis and degenerative changes. Amyloid deposits and neuronal cytoskeletal inclusions were absent.

We measured the levels of the monoamine neurotransmitters and metabolites in striatum of 14 patients with end-stage dominantly inherited olivopontocerebellar atrophy (OPCA). On average, dopamine levels were reduced in putamen (-53%), caudate (-35%), and nucleus accumbens (-31%). However, individual patient values showed a wide variation, indicating that mild to moderate striatal dopamine loss is a common but not constant feature of OPCA. Seven patients had marked putamen dopamine loss (-62% to -81%) but without evidence of correspondingly severe substantia nigra cell damage; this suggests the possibility of a "dying-back" phenomenon in which nerve terminal loss precedes cell body degeneration. Severe substantia nigra cell loss with almost total (-95% to -99%) putamen and caudate dopamine depletion was present in two patients; however, none of the 14 patients had had a clinical diagnosis of parkinsonism or was receiving antiparkinsonian medication. Mean striatal serotonin levels were normal, whereas concentrations of the serotonin metabolite 5-hydroxyindoleacetic acid were elevated by 47% to 63%; this suggests increased activity of raphe dorsalis serotonin neurons innervating the striatum, which might aggravate the functional consequences of the dopamine deficit.

We present a correlation of the morphometric cell density analysis with previous biochemical findings for the inferior olivary nucleus and Purkinje cell layer of the cerebellum from 10 patients (three kindreds) with dominant olivopontocerebellar atrophy. We have analyzed brain amino compounds of these patients and found a decrease of aspartic acid and glutamic acid in the cerebellar cortex and of aspartic acid in the inferior olives. The cell density analysis revealed decreased cell counts, with a mean of 34% of olivary cells and 42% of Purkinje cells surviving when compared with 10 control brains. The cell counts were then correlated with the amino acid analyses. The correlation coefficient for aspartic acid content and surviving neurons in the inferior olive was .87 and that for aspartic acid content and Purkinje cell density was .86. Comparison of glutamic acid content and Purkinje cell density yielded a correlation coefficient of .75. The correlations appear to indicate a relationship between these particular cells or the area they occupy and the decreased content of the two amino acids.

We measured the activity of choline acetyltransferase, the cholinergic marker enzyme, in the brains of 17 patients from five established pedigrees with dominantly inherited olivopontocerebellar atrophy (OPCA). OPCA is a group of cerebellar ataxia disorders in which serious intellectual impairment is not typically considered to be an accompanying feature. Patients from all five pedigrees demonstrated markedly reduced choline acetyltransferase activity in the cerebral cortex, with less severe changes in the hippocampus. Although the magnitude of the cortical choline acetyltransferase deficit is comparable to that seen in the brains of patients with Alzheimer's disease, none of our OPCA patients appeared, on last examination, to have severe global dementia of the Alzheimer type. Determination of the clinical significance of our biochemical data must await the results of studies in which the cognitive status of OPCA individuals has been accurately assessed.

To determine the possibility of genetic linkage of spinocerebellar ataxia with the histocompatibility loci, we performed HLA typing and linkage analysis on 19 members of a kindred in which spinocerebellar ataxia was segregating in an autosomal dominant inheritance pattern. The ataxia locus was located on chromosome 6 at 12-cM distance from the HLA complex with lod score of 3.15 (odds is greater than 1400:1 favoring linkage over chance findings). Thus, the presence of the ataxia gene in members of this kindred at risk can be predicted with about 90 per cent accuracy by means of HLA typing in informative matings.

We examined the behaviour of [3H]inositol-1,4,5-trisphosphate (IP3) binding in autopsied cerebellar and cerebral cortex of 10 neurologically normal controls and 8 patients with end-stage olivopontocerebellar atrophy (OPCA), a cerebellar ataxia disorder characterized histologically by severe degeneration of Purkinje cells. [3H]IP3 binding to normal human cerebellar cortex was 6-15 times higher than in cerebral cortex. As compared with the controls, mean [3H]IP3 binding to cerebellar cortex was markedly reduced by 61% in the OPCA patients whereas levels were normal in frontal and occipital cortices. Since the Purkinje cell dendrite receives neuronal input from granule cells and climbing fibers utilizing glutamate and aspartate, respectively, as neurotransmitters, the reduced IP3 binding in OPCA cerebellar cortex may reflect a loss of Purkinje cells containing these excitatory amino acid receptors linked to the phosphatidylinositide second messenger system. Our data suggest that in humans, IP3 receptors may be highly localized to the Purkinje cell dendrite.

Dominantly inherited spinocerebellar ataxia (OPCA 1) poses a particular problem for genetic counseling due to late age of onset. Liability for disease using observed age of onset can be combined with HLA linkage data to provide revised estimates of risk for affection. Where data is informative, certain combinations may yield estimates which improve so rapidly with age that postponement of childbearing may become a strong consideration as an option.

T HIS report concerns 7 cases of Wallenberg's lateral medullary syndrome studied by vertebral angiography. Krayenbiihl 4 and Krayenbtthl and Ya~argil s in two reports mentioned that they have studied 12 cases but actually 5 cases were reported, 3 in their first publication and ~ in the second (several were not clinically lateral medullary syndromes). They found partial or complete occlusion of the vertebral ar tery at the arch of the atlas as the characteristic angiographic picture for this clinical syndrome. There are other scattered reports of angiographic studies done in cases of the lateral medullary syndrome usually as part of a large series of vertebral angiograms but, as far as we know, KrayenbUhl and Ya~argil's is the largest series reported previously. An excellent demonstration of the pathologic findings in this syndrome has been given by Fisher et al. 3 Those authors found occlusion of the vertebral ar tery in 7 of 16 pathologically proven cases, occlusion of the vertebral and the posterior inferior cerebellar arteries in 5, occlusion of the posterior inferior cerebellar ar tery alone in r and in ~ cases no vascular occlusion was found. In those cases in which just occlusion of the vertebral ar tery was present, small branches of tha t ar tery had supplied the infarcted lateral medullary area and the posterior inferior cerebellar ar tery was absent or not involved in the

Fifty-one twin pairs, one or both members of each with multiple sclerosis (MS), were analyzed for extraneous events occurring prior to onset. Six patient groupings allowed comparison of events with and without the genetic factor. Comparison of monozygotic, discordant (one involved), and dizygotic discordant twins showed a difference. The affected members of the monozygotic pairs encountered prior to onset more birth anoxia, unusual infantile and childhood infections, major operations, and childbirth than did their unaffected twins, a difference not found when comparing dizygotic twins. The difference was most evident in the monozygotic discordant twins interviewed 30 years after onset of MS. If concordant (both involved) twins are analyzed by early vs late age of onset, these events occur at an older age in the late- and at younger age in the early-onset patients, suggesting that they may determine the age of onset of symptoms; they are suspected to be important in the causation of MS in genetically susceptible individuals, although the mechanism of their action is unknown.

A critical review is presented of 12 clinical trials with Hydergine (a hydrogenated ergot alkaloid preparation) in the treatment of dementia. Qualitative and quantitative comparisons of improvement in symptoms showed that Hydergine consistently produced statistically significant (p less than or equal to 0.05) improvement in 13 symptoms associated with dementia. However, because of the small magnitude of the improvement and the absence of indications of long-term benefit, Hydergine would seem to be of minor value in dementia therapy. Further research with better methodology and design might lead to a different conclusion.

We studied a family with essential tremor of the arms. Some members also had tremor of the trunk and legs on standing, but not on walking, sitting, or reclining. Tremor was evoked, regardless of body or limb position, by strong tonic contraction of the appropriate muscles; it was a type of static postural tremor. Beta-adrenergic blockers had no effect on the tremor of the trunk or legs, but clonazepam was beneficial.

EXTRAPYRAMIDAL DISORDERS In general, the neurologic diseases that are classified as extrapyramidal disorders are characterized by the presence of involuntary movements. The structures involved in producing extrapyramidal diseases are, for the most part, the basal ganglia.1.2 Parkinsonism Parkinsonism is the most common of the extrapyramidal disorders; prevalence is estimated at 1% of the population over age 50 years, and approximately 36,000 new cases are identified each year. The disease is characterized by rigidity of trunk, limbs, and head; by resting or occasionally intention tremor of the head or limbs, usually the hands; and by bradykinesia, or slowness in initiating voluntary movements. Presence of one component of the triad of rigidity, tremor, and bradykinesia suggests the disease and constitutes the minimal clinical criterion for diagnosis. But the clinical array of signs in parkinsonism is often much broader than the triad cited, and various other features that affect prognosis can be

A 24-year-old man from rural Mississippi had a case of California encephalitis (CE) that evolved as a subacute encephalomyelitis. The incidence of CE in adults is low, especially in the southeastern United States, and to our knowledge the clinical profile of symptomatic disease in adults has not been established. The characteristics of CE and St Louis encephalitis, the usual cause of arboviral illness affecting adults in the Southeast, differ.

Analysis of 60 Irish multiple sclerosis patients and matched controls for events prior to onset showed that parents of the patients died at a younger age and that there was a significant excess of infectious, traumatic, and operative events before age 20 in the patient group. A slight increase of possible perinatal anoxia, of possibly infectious infantile illnesses and burns in early childhood, and of operative events in the second decade also was found. Multiple sclerosis may be the unique response of the human nervous system in the susceptible individual to nonspecific injury and infection occurring first in early life and then in the second decade.

We found that the rate of progression of two adult hereditary neurologic disorders (dominant ataxia and Huntington disease) correlated inversely with the age at onset. The earlier the onset, the more rapid the course; the later the onset, the slower the course. Alzheimer disease/senile dementia followed a similar pattern. The rate of progression of a nonhereditary progressive neurologic disorder, ALS, showed the opposite trend.

We had been struck over the past few years in this area by the startling lack of aphasia in patients with large dominant hemisphere infarcts who were often illiterate. The question arose as to whether this was due to the illiteracy or possibly to the patient's race since our state contains a large number of elderly uneducated black citizens. Therefore this study is to determine the presence or absence of aphasia in stroke patients in relation to sex, race, age, and the degree of literacy.

ALTHOUGH the diagnosis of nonorganic hemiplegia and hemianesthesia associated with conversion reactions is not difficult for the experienced neuropsychiatrist, neurologist or neurosurgeon it does p...

The reported incidence of positive scans in the presence of cerebral infarction varies between 25 and 81 per cent (1–8). In a previous report (9) of 129 cases, the present authors found 41 per cent positive, with the greatest number of positive scans in the third week postinfarction. The purposes of the present study are several: (a) to better elucidate the type of cerebrovascular lesion which will produce an abnormal brain scan, (b) to more clearly define the post-infarct periods when the scans are negative and positive, (c) to correlate infarct size as judged clinically with the brain scan results, (d) to demonstrate infarcts by scan in the anterior, middle, and posterior cerebral and the basilar-vertebral artery distributions, and (e) to evaluate the brain scan for its value in diagnosis and prognosis. Materials and Method This study analyses 179 brain scans in 152 patients with the diagnosis of cerebral infarction. Scans were performed three to five hours after intravenous injection of Hg203- or Hg197...

From the many confusing terms currently employed to describe occlusive aortic arch disease, two are selected: aortic arch syndrome to describe the general clinical entity regardless of etiology; and Takayasu's arteritis to describe the idiopathic aortitis usually affecting young women. All others would best be discarded, particularly that of pulseless disease. It is suggested that Takayasu's arteritis is a connective tissue disease which like lupus erythematosus is sex limited; that two factors may be important in its localization: an autoimmunopathy affecting vascular elastin, and hemodynamic stress. The clinical diagnosis of Takayasu's arteritis can be made with certainty only by exclusion or pathologic study. Factors of helpful diagnostic value include: abnormal serum electrophoretic pattern, persistent elevation of the erythrocyte sedimentation rate and angiocardiographic changes.