bluuebunny/crossref_metadata_2025 huggingface dataset:
Seasonal (circannual) periodicity of spontaneous intracerebral hemorrhage in minnesota
Authors: Ramirez‐Lassepas Manuel , Haus Erhard , Lakatua David J. , Sackett Linda , Swoyer Jackie
Published: January 2005

Seasonal periodicity in the onset of spontaneous intracerebral hemorrhage was studied in 118 consecutive cases occurring during a six‐year span. The patients were urban residents of eastern Minnesota, a region characterized by wide seasonal fluctuations in daylight and temperature. The greatest number of cases consistently occurred each year during January and February. Circsianual (about one year) periodicity was demonstrated by statistical analysis using rhythmometric techniques. This periodicity coincided with that reported for mortality from cerebrovascular and cardiovascular diseases in the United States and elsewhere. Available information suggests that in populations at high risk for vascular diseases, climatic conditions might act as synchronizers to endogenous rhythms influencing the periodic occurrence of pathological vascular events.
Spasms of the iris sphincter
Authors: Stanley Thompson H. , Corbett James J.
Published: January 2005

A patient is described who suffered from intermittent spasms of the pupillary sphincter and ciliary muscle. Both intraocular muscles contracted several times a minute, producing sporadic darkening of vision in the affected eye. The spasms continued for eleven days, so it was possible to document them pupillographically. This curious phenomenon seems to have been the result of a transient “irritability” of the ciliary ganglion.
Nonaminergic striatal neurons convert exogenous l‐dopa to dopamine in parkinsonism
Authors: Melamed Eldad , Hefti Franz , Wurtman Richard J.
Published: January 2005

In intact striatum, the enzyme dopa decarboxylase is localized predominantly in dopaminergic nerve terminals. In Parkinson disease, loss of dopaminergic neurons is associated with massive depletion of striatal decarboxylase activity. Nevertheless, efficacy of exogenous L‐dopa in parkinsonism is generally believed to result from its enzymatic decarboxylation to dopamine in the corpus striatum. It has previously been suggested that, after degeneration of nigrostriatal pathways, decarboxylation of administered L‐dopa may occur mainly at such striatal sites as surviving dopaminergic terminals, serotonergic neurons, or capillaries; but currently available data do not favor these hypotheses.

Recent experimental studies indicate that a substantial amount of decarboxylase activity is localized in striatal interneurons or efferent neurons that may not normally synthesize monoamines. We propose that after depletion of dopaminergic terminals, these nonaminergic striatal neurons may contain a large fraction of residual dopa decarboxylase activity and may represent an important locus for conversion of administered dopa to functional dopamine in the parkinsonian corpus striatum.
Amyotrophic lateral sclerosis among chamorro migrants from guam
Authors: Garruto Ralph M. , Gajdusek D. Carleton , Chen Kwang‐Ming
Published: January 2005

Amyotrophic lateral sclerosis (ALS), which occurs in unusually high incidence among the Chamorro people on the island of Guam, has developed in 28 Chamorro migrants‐24 of whom had onset in the United States, Japan, Germany, or Korea‐‐after periods of absence from Guam of 1 to 34 years. Thus, the latency period for the disease, if caused by environmental factors on Guam, may be over three decades. Four further patients developed ALS within 1 to 14 years of their return to Guam after long‐term residence in the continental United States. The minimum exposure time to environmental variables on Guam, based on age at migration, was 18 years, and all patients had spent their childhood and adolescence on Guam. Estimates of crude mortality rates for ALS from these data are considerably higher than for the United States population, and lower than the ALS mortality rates for nonmigrant Chamorros on Guam.
Mollaret meningitis: Report of a case with recovery after colchicine
Authors: Mora Jesus S. , Gimeno Alberto
Published: January 2005

A 13‐year‐old boy developed closely spaced episodes of recurrent febrile meningitis that persisted for 22 months despite antibiotic therapy. During exacerbations the cerebrospinal fluid contained up to 2,500 leukocytes per cubic millimeter, including up to 20% activated monocytoid cells, with high IgM and IgG levels. No cause for the disorder was found, but steroids improved the condition and colchicine therapy apparently cured it.
Normal pyruvate dehydrogenase complex activity in patients with Friedreich's ataxia
Authors: Constantopoulos George , Chang Charles S. C. , Barranger John A.
Published: January 2005

Normal activity of the pyruvate dehydrogenase complex was found in platelet‐enriched preparations from two siblings with Friedreich's ataxia who had been reported previously as being deficient in this enzyme.
Brainstem auditory evoked responses in spinocerebellar engeneration and wilson disease
Authors: Fujita Masashi , Hosoki Masako , Miyazaki Motoji
Published: January 2005

Brainstem auditory evoked responses (BAERs) were studied in 30 healthy subjects, 20 patients with spinocerebellar ENgeneration (SCD), and 6 patients with Wilson disease. In addition to the standard 8 click per second stimulation rate, increased stimulation frequency was applied which identified wave V for its stability under different stimulation rates. No prolongation of wave latencies was noted in SCD patients, irrespective of clinical features, except in 1 patient who had a latency slightly above the normal range. Wilson disease with neurological symptoms produced prolongation of wave latencies in every case, whereas the patients without neurological involvement showed a normal BAER pattern.
Lisuride in parkinsonism
Authors: Parkes J. D. , Schachter M. , Marsden C. D. , Smith B. , Wilson A.
Published: January 2005

Lisuride is a soluble ergolene derivative with endocrine effects similar to but more potent than those of bromocriptine. In nine subjects with idiopathic, postencephalitic, or drug‐induced parkinsonism, lisuride at a dosage of 0.05 to 0.15 mg intravenously caused an immediate improvement in tremor, rigidity, akinesia, and postural deformity, but also caused chorea and orofacial dyskinesia. Improvement lasted 2 to 3 hours. Lisuride had little or no effect in a single patient with progressive supranuclear palsy. Oral lisuride therapy, 0.8 to 4.8 mg daily, had similar effects but occasionally caused reduced awareness and hallucinations.
Nerve fiber conduction velocity distributions: Studies of normal and diabetic human nerves
Authors: Cummins Kenneth L. , Dorfman Leslie J.
Published: January 2005

We have assessed the clinical applicability of a previously ENscribed new method for analyzing the distribution of conduction velocities (DCV) in peripheral nerve. Surface‐recorded median sensorimotor compound action potentials (CAPs) were found to contain contributions from classes of fibers conducting between 25 and 80 m/sec in the elbow‐to‐axilla segment. Test‐retest correlation coefficients ranged from 0.91 to 0.98. Increasing stimulus intensity recruited additional fiber activity into the CAP with relative preferential activation of faster‐conducting fibers at lower stimulus levels. Changes in limb temperatur altered the conduction velocity of all fiber classes 4% per ENgree Celsius. A comparison of fiber diameter distribution and DCVs in a normal sural nerve gave a correlation of 0.70 over the range of 7 to 12 μ. Diabetic patients with minimal or no clinical polyneuropathy showed varying ENgrees of DCV shift toward the slower conduction velocities, sometimes even in the face of normal conventional nerve conduction studies. These findings indicate that the DCV may be consiENred the electrophysiological counterpart—not equivalent—of the fiber diameter distribution, with the capacity to distinguish subtle differences in conduction properties of normal and diseased human nerves.
Postictal pleocytosis
Authors: Schmidley James W. , Simon Roger P.
Published: January 2005

Six patients showed a transient and otherwise unexplained cerebrospinal fluid (CSF) pleocytosis following a flurry of generalized convulsions. Each had an obvious cause for repeated seizures. No evidence was found for an infectious, inflammatory, neoplastic, or other cause for the pleocytosis. All CSF specimens were clear and colorless, under normal pressure, and bacteriologically sterile. The maximal leukocyte count ranged from 9 to 80 per cubic millimeter and reached a maximum on the day after cessation of convulsions. No specimen contained more than 650 erythrocytes. Two patients initially had a mildly increased CSF protein; glucose values were unremarkable.

We propose that the pleocytosis in these patients was a result of frequently repeated generalized convulsions. The mechanism of postictal pleocytosis is uncertain. It may result from transient breakdown of the blood‐brain barrier, which has been demonstrated after seizures in experimental animals. Although infectious causes must first be considered and rigorously searched for, it appears that seizures alone may cause a transient CSF pleocytosis.
Vestibular testing in basilar artery migraine
Authors: Eviatar Lydia
Published: January 2005

Vestibular function studies were performed in 20 children with symptoms suggesting basilar artery migraine. Sixteen children had abnormalities on bithermal caloric testing. Labyrinthine preponderance was found in 1 child with labyrinthine concussion and in 6 children with symptoms suggesting ischemia of the territory supplied by the basilar artery and the occipital branches of the posterior cerebral arteries. Directional preponderance was found in 4 children with symptoms suggesting ischemia of the posterior temporal branches of the posterior cerebral arteries. Five children had both labyrinthine and directional preponderance. Normal electronystagmographic findings were present in 4 children and correlated clinically with a milder illness. Head trauma was the triggering factor for the migraine in 5 children. Five children developed complex partial seizures and had evidence of directional preponderance (2 children) and combined labyrinthine and directional preponderance (2 children).

The presence of abnormalities on electronystagmography in children with basilar artery migraine correlates with the severity of the disease. Directional preponderance or labyrinthine and directional preponderance are more frequently correlated with ischemia in the posterior temporal branches of the posterior cerebral arteries and may suggest a higher risk of developing vertiginous seizures when ischemia is prolonged.
Vascular adrenergic receptor responses in skeletal muscle in myotonic dystrophy
Authors: Mechler F. , Mastaglia F. L.
Published: January 2005

The pharmacological responses of vascular adrenergic receptors to intravenously administered epinephrine, phentolamine, and propranolol were assessed by measuring muscle blood flow (MBF) changes in the tibialis anterior muscle using the xenon 133 clearance technique and were compared in 8 normal subjects and 11 patients with myotonic dystrophy. In cases with advanced involvement of the muscle, the resting MBF was reduced and was not significantly altered by epinephrine before or after α‐ or β‐receptor blockade. In patients in whom the tibialis anterior muscle was normal or only minimally affected clinically, a paradoxical reduction in the epinephrine‐induced increase in MBF was found after a blockade by phentolamine, and the epinephrine‐induced MBF increase was not completely blocked by propranolol as in the normal subjects. These findings point to a functional alteration in the properties of vascular adrenergic receptors in muscle in myotonic dystrophy. While this may be another manifestation of a widespread cell membrane defect in the disease, the possibility that the changes are secondary to the myotonic state cannot be excluded.
Progressive sensory neuropathy in patients without carcinoma: A disorder with distinctive clinical and electrophysiological findings
Authors: Kaufman Michael D. , Hopkins Linton C. , Hurwitz Barrie J.
Published: January 2005

Seven patients with severe progressive impairment of kinesthetic sense, mild dysfunction of cutaneous sense, and sparing of motor function were examined during a 3‐year period. The clinical and electrophysiological findings are described in detail. None of these seven has had evidence of cancer despite a thorough investigation and a 3‐ to 16‐year (average, 7 years) period of symptoms. These patients' symptoms were indistinguishable from those of patients with sensory neuropathy and coexistent carcinoma, suggesting that progressive sensory neuropathy is not invariably associated with carcinoma.
Multifocal varicella‐zoster virus leukoencephalitis temporally remote from herpes zoster
Authors: Horten Bruce , Price Richard W. , Jimenez Daisy
Published: January 2005

Two patients with cancer, one with Hodkin's disease and the other with a granulosa cell tumor of the ovary, developed a progressive, eventually fatal infection of the central nervous system exhibiting multifocal symptoms and signs. Pathologically, gross abnormalities of the brain resembled those in progressive multifocal leukoencephalopathy (PML), with discrete and confluent plaque‐like lesions concentrated in the white matter, particularly along the gray‐white junction. Microscopically, pathological changes differed distinctly from those associated with PML; in addition to confluent foci of white matter injury characterized by early demyelination and subsequent necrosis, prominent Cowdry type A eosinophilic intranuclear inclusions were noted in oligodendrocytes, astrocytes, and neurons. By electron microscopy, intranuclear spherical particles consistent in size and appearance with herpesvirus nucleocapsids were found within the lesions. Immunoperoxidase studies detected varicella‐zoster virus (VZV) antigens in infected cells, implicating this virus as the responsible agent despite a lapse of many months between the cutaneous herpes zoster and onset of cerebral symptoms in both patients.
Galvanic vestibular tests in the assessment of coma and brain death
Authors: Toglia Joseph U. , Adam Rasheed U. , Stewart George
Published: January 2005

Galvanic stimulation over the mastoid process elicits oculovestibular responses that provide a measure of brainstem function. The equipment is simple and is available in most neurodiagnostic laboratories. This type of test provides an advantage over caloric testing in the presence of external and middle ear disease and basal skull fracture and can be a useful adjunct in the evaluation of coma and brain death.
Usefulness of electrophysiological studies in the diagnosis of lumbosacral root disease
Authors: Tonzola Richard F. , Ackil Albert A. , Shahani Bhagwan T. , Young Robert R.
Published: January 2005

Clinical, electrophysiological, and myelographic findings were correlated in 57 patients with the clinical diagnosis of lumbosacral root disease. Conventional motor and sensory (including sural nerve) conduction studies were normal in all patients. Electromyography, late response studies in different muscles of the lower extremity, the myelogram, or combinations of these tests were abnormal in 44 patients (77%). Of 36 patients (63%) with abnormal myelograms, 14 had normal electrophysiological studies. Twenty‐nine (51%) had an abnormal electrophysiological finding; although 8 patients in this group had a normal myelogram, 2 had an abnormal discogram and 1 an abnormal epidurogram. Electrophysiological or myelographic findings, in some cases both, correlated well with clinical signs and symptoms in 41 patients (72%). H‐reflex and F response studies, when abnormal, helped in localizing a lesion in the appropriate root distribution.
Focal hyperemia followed by spreading oligemia and impaired activation of rcbf in classic migraine
Authors: Olesen Jes , Larsen Bo , Lauritzen Martin
Published: January 2005

Regional cerebral blood flow (rCBF) was measured in 254 areas of a hemisphere with the xenon 133 intraarterial injection method. Six cases of classic migraine were followed from the normal state into the prodromal phase, and in 3 cases further into the headache phase. One patient with common migraine was similarly followed during his only classic attack. The attacks were initiated by focal hyperemia in 3 patients. During prodromes all patients displayed occipitoparietal rCBF reduction (oligemia), but in only 1 case did the reduction approach critical values. Oligemia gradually spread anteriorly in the course of 15 to 45 minutes. In 4 patients a global oligemia was observed. In 4 patients severe headache was present concomitantly with oligemia and with no sign of hyperemia or nonhomogeneous brain perfusion. The normal rCBF increase during cortical activity (hand movement, speech, and similar activities) was impaired in 6 patients. The results indicate that the vasospastic model of the migraine attack is too simplistic.
Sustained upgaze in coma
Authors: Keane James R.
Published: January 2005

Coma with sustained upward gaze deviation followed cardiac arrest in 15 patients and prolonged systemic hypotension in 2 patients. Neuropathological examinations confirmed the expected diffuse cerebral and cerebellar damage with relative sparing of the brainstem. No focal lesions were found in the upper midbrain or pretectum. Six patients developed slow, downward‐beating nystagmus as the forced upward gaze resolved. Four of 6 patients with posthypoxic facial myoclonic jerks demonstrated synchronous upward gaze movements. Forced upgaze in coma usually is the result of severe hypoxic encephalopathy. While anatomical localization is obscure, this sign does not indicate a structural lesion in the pretectum or midbrain and may result from damage to cerebellar pathways.
Autologous lymphocyte proliferation in multiple sclerosis and the effect of intravenous ACTH
Authors: Birnbaum Gray , Kotilinek Linda
Published: January 2005

The proliferative reponse of peripheral blood T cells to autologous non–T cells, a reaction called the autologous mixed lympbocyte reaction (MLR), was significantly increased in 17 patients with active multiple sclerosis (MS) compared to age‐ and sex‐matched individuals with other neurological diseases (OND). Following a 10‐day course of intravenous adrenocorticotropic hormone (ACTH) therapy the value were reduced to control levels. No differences were noted between MS patients and controls in their response to alloantigens. The increased autologous MLR in patients with active MS appeared to result from an increased stimulatory capacity of non–T cells rather than from an intrinsically greater T cell proliferative potential. ACTH appeared to induce a change in the populations of circulating non–T cells such that these cells had a decreased stimulatory capacity in both autologous and allogeneic MLR. The disease in stimulatory MLR was, however, significantly greater than the decrease in allogeneic stimulatory capacity, suggesting a functional decrease of specific non–T cellenriched subpopulations. No significant changes in the numbers of myeloperoxidase‐positive (MP+) cells were noted in the blood of MS patients before and after ACTH therapy. Since the autologous MLR results in generation of cells that regulate immune responsiveness, the changes noted provide additional evidence for abnormal immune regulation in MS.
Chronic GM1 gangliosidosis presenting as dystonia: II. Biochemical studies
Authors: Kobayashi Takuro , Suzuki Kunihiko
Published: January 2005

A patient with chronic GM1 gangliosidosis was studies enzymatically and biochemically. Leukocyte acid β‐galactosidase activity was severely deficient. In brain and liver, the 4‐methylumbelliferyl β‐galactosidase with acidic pH optimum and lactosylceramidase II were deficient while other hydrolases were present in normal amounts, including sialidase determined with N‐acetylneuramin‐lactose and fetuin as substrates. Nautral β‐galatosidase in liver was incerased up to fourfold over the control. Corresponding to the pathological findings, GM1 ganglioside sialic acid was increased in the basal ganglia to 57% of the total (normal, 12 to 16%), accounting for the rise in total ganglioside to 180% of normal in this region. Only slight to moderate elevations in the proportion of GM1 ganglioside were noted in the cerebral cortex and white matter, without major increase in total ganglioside. Elevated asislo GM1 ganglioside was also confined to the basal ganglia. There was no increase in hepatic glycoproteins or in keratan sulfate‐like materials. This is the only known patient with chronic GM1 gangliosidosis in whom abnormal accumulation of GM1 ganglioside has been demonstrated in affected tissue and sialidase deficiency has been excluded as the primary genetic defect.
Progressive neurological disease associated with chronic cytomegalovirus infection
Authors: Bray Patrick F. , Bale James F. , Anderson Robert E. , Kern Earl R.
Published: January 2005

Cytomegalovirus (CMV), the most common infectious cause of nervous system damage in the fetus and newborn infant, generally has been thought to exert its teratogenic effect during early pregnancy, and it has been assumed that clinical or postmortem abnormalities reflect static insults incurred earlier. Over the past two years we have identified two infants who presented with various clinical signs suggesting CMV infection. Serial CT head scans showed mild hydrocephalus progressing to extensive loss of brain substance and hydrocephalus in one and a normal scan going on to polycystic encephalomalacia in the other. Both infants had urine or leukocyte cultures that were repeatedly positive for CMV. These data suggest that CMV can cause progressive central nervous system damage after birth. The findings may account for some unexplained brain malformations and for a portion of the neurodegenerative disorders occurring in babies who appear normal at birth.
Myasthenia induced by monoclonal anti‐acetylcholine receptor antibodies: Clinical and electrophysiological aspects
Authors: Burres Steven A. , Crayton John W. , Gomez Christopher M. , Richman David P.
Published: January 2005

To investigate individual components of the humoral immune response directed against the acetylcholine receptor in experimental myasthenia, we injected monoclonal antireceptor antibody into normal rats. These antibodies were produced by cloned hybridoma lines formed by fusion of immune spleen cells with the myeloma cell line P3‐X63‐Ag8. Antibodies from two of seven clones induced acute experimental myasthenia, manifested by clinical weakness, decremental electromyographic response to repetitive nerve stimulation, and diminished mean amplitudes of miniature end‐plate potentials (mepps): 0.55 ± 0.02 mv (SEM) versus 0.71 ± 0.02 mv for controls (p < 0.001). In clinically affected animals, mepp amplitudes from the diaphragm correlated with decremental responses in the gastrocnemius muscle (r = −0.91, p < 0.01). The results suggest that binding of a single antibody species to a single determinant on the acetylcholine receptor molecule is sufficient to induce acute experimental myasthenia gravis.
Isoniazid‐induced elevation of CSF GABA levels and effects on chorea in huntington's disease
Authors: Manyam Bala V. , Katz Leonard , Hare Theodore A. , Kaniefski Kathryn , Tremblay Robert D.
Published: January 2005

A randomized, double‐blind, crossover, placebo‐controlled clinical trial of oral isoniazid was undertaken in eight men with known Huntington's disease. Six completed the trial. Overall chorea scores indicated some amelioration, but clinical improvement was noticed in only two patients and was mild. Side effects included anorexia and elevation of liver enzyme levels. Cerebrospinal fluid (CSF) and plasma γ‐aminobutyric acid (GABA) concentrations were measured simultaneously. Mean CSF GABA increased threefold following treatment with isoniaid (414 ± 52 SEM pmol/ml) compared to placebo (120 ± 11 pmol/ml). No significant changes occurred in plasma GABA levels between the placebo and drug treatment phases. Reversal of central GABA deficiency appears not to correct extrapyramidal symptoms in Huntington's disease.
Iris pigmentation (melanin) in idiopathic dystonic syndromes including torticollis
Authors: Korein Julius
Published: January 2005

Iris pigmentation was evaluated in 153 Caucasian patients with torticollis and other focal, segmental, or generalized dystonias of unknown cause. Since these disorders are rare in non‐Caucasians, it was hypothesized that a relationship might exist between decreased melanin metabolism, reflected by iris pigmentation, and a genetic predisposition to these disorders of voluntary movement. Patients were separated into two groups on the basis of iris pigmentation. Compared to control groups, there was a statistically significant reversal in the ratio of patients with light eyes versus dark eyes. Further comparisons were made in two groups of patients with drug‐induced nonparkinsonian dyskinesias; no significant correlation was found between their dyskinetic syndrome and iris pigmentation. Data concerning race, ethnic origin, and other disorders of the basal ganglia support the hypothesis that there may be genetic predilection in individuals whose metabolic pathways produce less systemic melanin.
CSF changes during acute meningoencephalitis in mice caused by encephalomyocarditis virus
Authors: Griffin Diane E.
Published: January 2005

Encephalomyelitis was induced in mice by intravenous inoculation with encephalomyocarditis virus. Examination of the cerebrospinal fluid revealed marked pleocytosis and increased amounts of protein, including immunoglobulins. These proteins entered the cerebrospinal fluid by transfer from serum during the period of central nervous system infection.
Multiple sclerosis: Capping of surface immunoglobulin G on macrophages engaged in myelin breakdown
Authors: Prineas J. W. , Graham J. S.
Published: January 2005

Macrophages were examined for immunoglobulin G (IgG) and albumin in actively demyelinating lesions in two patients with multiple sclerosis (MS) using the peroxidase‐antiperoxidase immunocytochemical technique. In both cases, macrophages were present that stained for cytoplasmic or surface IgG or both. In one case, in which the tissue was rapidly fixed in chilled fixative, macrophages located among myelinated nerve fibers at plaque margins, but not elsewhere in the plaque, revealed surface IgG in the form of caps restricted to one or both poles of the cell. These caps were absent in sections stained for albumin. Because capping implies the presence of a multivalent ligand close to the cell surface and because cap formation was observed only in macrophages contacting myelin sheaths, we suggest that antimyelin antibody cytophilic for macrophages may be present in the central nervous system in MS, and that immune ligand‐mediated phagocytosis may play a role in myelin breakdown in the disease. This study provides the first direct evidence that IgG participates locally in myelin breakdown in MS.
Congenital hemihypertrophy: Oncogenic potential of the hypertrophic side
Authors: Furukawa Tetsuo , Shinohara Tamiko
Published: January 2005

Congenital hemihypertrophy is a rare condition, occasionally associated with neoplasms on the affected side of the body. Investigation of three cases disclosed increased growth rate of cultured skin keratinocytes and fibroblasts and high skin temperature on the hypertrophic side. These observations suggest the oncogenic potential of the affected side in the condition.
Vitamin E deficiency in Werdnig‐Hoffmann disease
Authors: Shapira Yehuda , Amit Rami , Rachmilewitz Eliezer
Published: January 2005

Vitamin E levels were measured in the plasma of infants and children with various neuromuscular disorders. Seven of 8 infants with Werdnig‐Hoffmann disease (WHD) had a singificantly lower plasma vitmin E level (p < 0.01) than age‐matched normal controls, children with congenital myopathies, or children with muscular dystrophy. Vitamin E deficiency in WHD is not caused by malabsorption. A therapeutic trial of vtamin course of the disease. Vitamin E deficiency may play a role in the pathologenesis of WHD.
The incidence and prevalence of reported multiple sclerosis
Authors: Baum Herbert M. , Rothschild Beth B.
Published: January 2005

A national survey, sponsored by the National Institute of Neurological and Communicative Disorders and Stroke, to determine the incidence, prevalence, and economic impact of multiple sclerosis has just been completed. These data are the first report of the results. Based on the data gathered, it is estimated that on Januaryuary 1, 1976, there were a reported 123,000 multiple sclerosis patients in the conterminous United States (a rate of 58 per 100,000). The annual incidence for the period 1970–1975 was estimated to be 8,800 (a rate of 4.2 per 100,000). The pattern of the disease being more common among females, whites, persons aged 30 to 50 years, and individuals living above the 37th parallel was also demonstrated. In addition to demographic characteristics, selected disease characteristics of the incidence and prevalence populations were also examined.
Peripheral nerve unmyelinated axons following lumbar sympathectomy
Authors: Chad D. , Shoukimas G. M. , Bradley W. G. , Bosch E. P. , Shahani B. T. , Young R. R.
Published: January 2005

One year after bilateral lumbar sympathectomy for Raynaud's syndrome, a young woman underwent fascicular biopsy of the sural nerve to help elucidate the cause of lower limb pain. The morphological features, density, and diameter spectrum of unmyelinated fibers were normal. The sympathetic nervous system appears to contribute few, if any, axons to the total population of unmyelinated axons in this cutaneous nerve.
Resetting of tremor by mechanical perturbations: A comparison of essential tremor and parkinsonian tremor
Authors: Lee Robert G. , Stein Richard B.
Published: January 2005

With the use of a computer‐controlled torque motor, experiments were carried out on 11 patients with essential tremor and 13 with parkinsonian tremor to determine the effect of mechanical displacements at the wrist joint on the established pattern of tremor. Analysis of the timing of tremor bursts in electromyographic recordings before and following the stimuli revealed that the phase of essential tremor could be readily reset by external perturbations. In the majority of the parkinsonian patients the same type of stimuli had very little effect on the pattern of tremor. Differences between predicted and actual times of occurrence of tremor bursts following the stimuli were used to calculate a normalized resetting index, with 0 representing no resetting and 1, complete resetting. For the patients with essential tremor the mean resetting index was 0.64 ± 0.14 (SD); for the parkinsonian patients the values were 0.16 ± 0.19. The results suggest that oscillations within spinal reflex loops play a major role in the generation of essential tremor. Reflex mechanisms are less important in parkinsonian tremor, which is more likely dependent on oscillations originating in the central nervous system.
Dependence of the achilles tendon reflex on the excitability of spinal reflex pathways
Authors: Burke David , McKeon Brian , Skuse Nevell F.
Published: January 2005

Muscle afferent activity from the triceps surae was recorded during experimentally induced alterations in amplitude of the Achilles tendon jerk. No changes in the neural afferent response to tendon percussion or in the background level of neural activity occurred when the reflex response was altered by discomfort, distraction, changes in attention, or changes in the rate of tendon percussion. Reinforcement of the Achilles tendon jerk by forceful contraction of the forearm muscles did not alter the relationship between intensity of the tendon tap and amplitude of the evoked neural afferent volley. Nevertheless, such maneuvers lowered the reflex threshold and raised reflex sensitivity so that a smaller afferent volley was required to produce a tendon jerk, and an increase in the afferent volley produced a disproportionately greater increase in reflex electromyographic activity than would have occurred at rest. Reinforcement maneuvers potentiated the H‐reflex but did not alter the electrically induced afferent volley or the background level of neural activity. It is concluded that these changes in reflex responsiveness occurred through intrinsic spinal mechanisms independent of the fusimotor system.
Myoclonus and ocular oscillations induced by L‐tryptophan
Authors: Baloh Robert W. , Dietz John , Spooner Joseph W.
Published: January 2005

A patient with chronic manic‐depressive illness developed generalized myoclonus and spontaneous ocular oscillations after a single 2 gm dose of L‐tryptophan. She had been pretreated with both a tricyclic antidepressant and a monoamine oxidase inhibitor. The involuntary movements gradually disappeared within 24 hours after the drugs were discontinued. Electrooculographic recording 7 hours after onset of the abnormal eye movements revealed square‐wave jerks and hypometric voluntary saccades. Pursuit as well as optokinetic and vestibular slow phases were normal except for superimposition of the square‐wave jerks. Repeat recording 24 hours later was entirely normal.
Local cerebral glucose metabolism in newborn dogs: Effects of hypoxia and halothane anesthesia
Authors: Duffy T. E. , Cavazzuti M. , Cruz N. F. , Sokoloff L.
Published: January 2005

Local cerebral glucose utilization (LCGU) was measured in 36 neuroanatomical structures of normal awake, halothane‐anesthetized, and hypoxic newborn puppies by the autoradiographic 2‐[14C]deoxyglucose method. In normal animals, LCGU was highest in the vestibular nucleus and in other gray matter nuclei of the brainstem and declined in a caudal‐to‐rostral progression through the neuraxis (i.e., LCGU of cerebellum > thalamus ≅ caudateputamen > cerebral cortex). Lowest rates of glucose metabolism were detected in white matter structures. Halothane anesthesia (1.5% inspired) caused few changes in local glucose metabolism, the most notable being decreased LCGU among structures of the auditory system (cochlear nucleus, lateral lemniscus, inferior colliculus) and increased LCGU in the interpeduncular nucleus. Acute systemic hypoxia (arterial oxygen tension of approximately 12 mm Hg) produced markedly heterogeneous effects on local glucose metabolism: LCGU was increased in some gray matter structures, decreased in the thalamus, and substantially increased in the subcortical white matter and corpus callosum. In puppies whose brains were frozen in situ after 55 minutes of hypoxia, the concentration of lactate was increased ten‐ to elevenfold in cortical gray and subcortical white matter, but the concentrations of glucose, adenosine triphosphate, and phosphocreatine declined to a greater extent in the white matter. The results suggest that during hypoxia the high rate of glycolysis in white matter exceeded substrate supply so that glucose availability became the limiting factor for local energy production. Such a mechanism may contribute to the white matter injury that often develops following hypoxic‐ischemic insults in the perinatal period.
Nevoid basal cell carcinoma syndrome and epilepsy
Authors: Murphy Martin J. , Tenser Richard B.
Published: January 2005

Four patients affected with the nevoid basal cell carcinoma syndrome and recurrent seizures are described and the pertinent literature is reviewed. Three of the patients had multiple basal cell carcinomas and cutaneous nevi, dentigerous cysts, and skeletal anomalies characteristic of the syndrome. The seizures had their onset in childhood and were generalized tonic‐clonic in three patients and both tonic‐clonic and complex partial in the fourth. Focal neurological deficits were found in only one patient. Pneumoencephalography in two patients and head CT scan in a third were negative. Electroencephalography showed focal spike discharges in one patient, bilateral anterior paroxysmal slowing in another, and nonspecific shifting temporal theta transients in the other two cases. Psychometric testing yielded full‐scale scores ranging from 39 to 84. In addition to mental retardation, congenital communicating hydrocephalus, and medulloblastoma, seizures should be included among the neurological manifestations of the nevoid basal cell carcinoma syndrome.
A case control study of Creutzfeldt‐Jakob disease: Association with physical injuries
Authors: Kondo Kiyotaro , Kuroiwa Yoshigoro
Published: January 2005

Sixty cases of Creutzfeldt‐Jakob disease reported from 902 neurological clinics throughout Japan between 1975 and 1977 were examined in a retrospective case control study. Histories were obtained from patients, sex‐matched neighbors, and spouses. No association was observed in either sex with five socioeconomic variables; exposures to eight species of animals; ingestion of raw meat or quadruped brains; eighteen diseases, allergies, or immunizations; tooth extraction; blood transfusion; or lumbar puncture. Surgical operations within five years before disease onset were reported for 25.9% and 7.8% of male patients and pooled controls and for 24.2% and 9.6% of female patients and controls. Mechanical injuries were observed for the same period in 33.3% and 9.8% of male patients and controls and in 18.2% and 13.5% of female patients and controls. The operations and mechanical injuries varied in all groups as to body parts affected and nature of the lesions. Physical injuries including operations may be a predisposing factor to the development of Creutzfeldt‐Jakob disease.
Defective mitogenic responses in myasthenia gravis and multiple sclerosis
Authors: Dropcho Edward J. , Richman David P. , Antel Jack P. , Arnason Barry, G. W.
Published: January 2005

Using in vitro lymphocyte proliferation induced by the phytomitogen concanavalin A (ConA), we investigated immune function and regulation in patients with myasthenia gravis (MG) and multiple sclerosis (MS). Unfractionated peripheral blood mononuclear cells of normal individuals responded to a wide range of ConA concentrations; the T cell fraction responded to a lesser degree and only to high concentrations. These findings suggest the presence of two receptors for ConA, one of high affinity present on a non‐T cell accessory cell and the other of low affinity present on T cells. Contrasting defects in the level of response of unfractionated lymphocytes and T cells were found in patients with MG and MS. The peak response of T cells in the MG patients was 22.6 ± 9.6 ± 103 cpm (mean ± SEM) compared with 54.6 ± 6.5 × 10(3) for controls (p < 0.05), while the response of unfractionated lymphocytes did not differ from that in controls. For MS patients, the unfractionated lymphocyte response was diminished: 56.3 ± 2.8 × 103 cpm versus 70.5 ± 4.5 × 103 for controls (p < 0.05), while the T cell response was normal. These results indicate a defect in the direct T cell response in MG; in contrast, in MS the response requiring T cell‐accessory cell interaction is abnormal.
Convulsive syncope in blood donors
Authors: Lin James T.‐Y. , Ziegler Dewey K. , Lai Chi‐Wan , Bayer William
Published: January 2005

Syncope with and without convulsion was studied in unselected blood donors in a community blood center. Convulsive syncope occurred in 0.03% of all blood donors and was more commonly observed when nursing personnel were alerted to its possible occurrence. It was more common in men. Although tonic extensor spasm was the most common convulsive movement, other complex convulsive phenomena occurred, some simulating epileptic seizure. No statistical difference in changes of pulse or blood pressure was found between subjects with convulsive versus nonconvulsive syncope. Similarly, no difference was found between subjects with tonic spasm and those with other convulsive phenomena, nor between those with “early” and those with “delayed” reactions. Marked individual variation may exist in the susceptibility of the central nervous system to ischemia. Some individuals appear to be predisposed to development of seizures in situations of global cerebral ischemia such as occur in hypotension and bradycardia.
Solitary intracranial plasmacytoma: Two patients with extended follow‐up
Authors: Krumholz Allan , Weiss Howard D. , Jiji Violet H. , Bakal Daniel , Kirsh Milton B.
Published: January 2005

Solitary neoplastic proliferation of plasma cells (plasmacytoma) rarely occurs in the central nervous system. The longest follow‐up in nine previously reported patients was 5 years. We have followed two patients with solitary intracranial plasmacytoma for 8 and 13 years, respectively. At the time of biopsy diagnosis, neither showed involvement of the adjacent calvarium or evidence of a systemic plasma cell dyscrasia. The first patient underwent partial excision of a mass in the right occipital lobe that showed no gross or microscopic involvement of the dura. The second patient had partial resection of a mass arising from dura and invading the left temporal lobe. Postoperative irradiation was given to both patients. No clinical or radiological sign of recurrent brain tumor has developed in either patient during the extended follow‐up period. Neither patient has had laboratory or clinical evidence of a systemic plasma cell dyscrasia. Solitary intracranial plasmacytoma may be a “curable” brain tumor.
Neuraminidase deficiency and accumulation of sialic acid in lymphocytes in adult type sialidosis with partial β‐galactosidase deficiency
Authors: Tsuji Shoji , Yamada Takamichi , Tsutsumi Atsuko , Miyatake Tadashi
Published: January 2005

Neuraminidase deficiency has been demonstrated in cultured skin fibroblasts of patients who have adult type sialidosis with partial β‐galactosidase deficiency. A substantial amount of residual enzyme activity has been observed in leukocytes, however. To explain this discrepancy, the nature and distribution of the enzyme were studied. Neuraminidase activity was higher in lymphocytes than in granulocytes of normal controls. In patients' lymphocytes, neuraminidase activity was profoundly decreased and total sialic acid contents were increased 2.3‐fold. Two neuraminidases, one sonication‐labile and the other sonication‐stable, were found in lymphocytes; the former was predominant in cultured skin fibroblasts. The defective enzyme in this disorder was found to be the sonication‐labile neuraminidase in both cultured skin fibroblasts and lymphocytes.
Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjögren syndrome
Authors: Alexander Elaine L. , Craft Carolyn , Dorsch Carole , Moser Robert L. , Provost Thomas T. , Alexander Garrett E.
Published: January 2005

A 37‐year‐old woman with primary Sjögren syndrome developed mixed cryoglobulinemia and systemic vasculitis. Subarachnoid hemorrhage occurred as a result of necrotizing anterior spinal arteritis. Although rarely seen in mixed cryoglobulinemia, central nervous system complications have recently been documented in Sjögren syndrome. The patient's serum contained antibodies to the Ro(SSA) cytoplasmic antigen, and these antibodies were concentrated in the cryoglobulin fraction. Anti‐Ro(SSA) antibodies are associated with the occurrence of vasculitis in patients with Sjögren syndrome, which suggests that the spinal arteritis and subarachnoid hemorrhage in this patient may have been directly related to the underlying connective tissue disorder.
Hind limb somatosensory evoked potentials in the monkey: The effects of distal axonopathy
Authors: Arezzo J. C. , Schaumburg H. H. , Vaughan H. G. , Spencer P. S. , Barna J.
Published: January 2005

Computer‐averaged short‐latency somatosensory evoked potentials (SLSEP) to unilateral stimulation of the peroneal nerve were recorded from surface electrodes overlying the peripheral nerve, cauda equina, spinal cord, brainstem, and contralateral sensorimotor region. Seven monkeys were studied under normal conditions and at various stages of distal axonopathy induced by systematic acrylamide intoxication. With the use of a noncephalic reference, a series of five small‐amplitude positive components were identified that precede the initial cortical activity. On the basis of timing and topography of the components, the source of the first one, SLSEP1, was localized to the lumbar dorsal root fibers and that of the second, SLSEP2, to the ascending spinal tracts, principally the gracile fasciculus. Bipolar recordings of the SLSEP2 overlying the rostral extreme of the cervical spinal cord provided a sensitive measure of the onset of distal axonopathy. Changes in the timing of this component antedated both abnormalities of spinal or peripheral nerve conduction and behavioral signs of intoxication. The initial alteration was specific to stimulation of the hind limb and was associated with early morphological change limited to the terminal and preterminal portions of the long axons in the gracile fasciculus.
Burn encephalopathy in children
Authors: Mohnot Dhanpat , Snead O. Carter , Benton John W.
Published: January 2005

Among 287 children with burns treated over a recent two‐year period, 13 (5%) showed evidence of encephalopathy. The major clinical symptoms were an altered sensorium and seizures. The majority of symptoms began later than 48 hours after the burn and were accompanied by multiple metabolic aberrations including hypocalcemia. Three children had a relapsing course, and 1 had temporarily enlarged cerebral ventricles. Eleven children improved to normal. In the majority of instances, burn encephalopathy probably reflects central nervous system dysfunction resulting from complex metabolic, hematological, and hemodynamic abnormalities rather than from a single metabolic abnormality.
Stability of monoamine metabolites in human cerebrospinal fluid
Authors: Langlais Philip J. , Bird Edward D. , McEntee William J.
Published: January 2005

Concentrations of 3‐methoxy‐4‐hydroxyphenylglycol (MHPG), homovanillic acid (HVA), and 5‐hydroxyindoleacetic acid (5‐HIAA) were measured in human lumbar crerospinal fluid following storage at room temperature and 4°C for intervals up to 72 hours. These monoamine metabolites were quantitated using a high‐performance liquid chromatographic—electrochemical detector technique. No significant difference could be detected in any of the three metabolites stored at room temperature for up to 48 hours. Additionof ascorbic acid to CSF had no measurable effect on the levels of HVA and 5‐HIAA. Repeated thawing and freezing produced no detectable change in metabolite levels.

The results indicate that these monoamine metabolites are stable at room temperature for up to 48 hours after lumbar puncture and that the addition of an antioxdant, such as ascorbic acid, is not necessary. The data also demonstrate that immediate refrigeration or freezing of samples is not required for maintenance of metabolite levels.
Neuroimmunomodulation: Neural anatomical basis for impairment and facilitation
Authors: Brooks William H. , Cross Richard J. , Roszman Thomas L. , Markesbery William R.
Published: January 2005

Rats with bilateral electrolytic lesions of specific limbic nuclei show alterations in lymphoid cell number and in lymphocyte activation induced in vitro by concanavalin A (Con A). The number of splenocytes decreases after lesioning in the anterior hypothalamus (p < 0.001), ventromedial hypothalamus (p < 0.002), and mamillary bodies (p < 0.001). The number of thymocytes decreases after lesioning of the anterior hypothalamus (p < 0.001) and increases after hippocampal lesioning (p < 0.001). Spleen cell responsivensess to Con A decreases subsequent to lesioning of the anterior hypothalamus, whereas reactivity was enhanced after lesion placement in the mamillary bodies (p < 0.002), hippocampus (p < 0.001), and amygdaloid complex (p < 0.001). Thymocyte mitogen reactivity is increased by lesions of the hipocampus (p < 0.001), and amygdaloid complex (p < 0.001). These effects manifest themselves maximally 4 days after lesioning, with a return to normal by day 14. These preliminary data indicate that quantitative and qualitative lymphocyte functions are altered by ablation of selected brain nuclei, thereby suggesting the presence of neural modulation of immune function.