bluuebunny/crossref_metadata_2025 huggingface dataset:
Review of 38 cases of subacute sclerosing panencephalitis: Effect of amantadine on the natural course of the disease
Authors: Robertson William C. , Clark David B. , Markesbery William R.
Published: January 2005

Thirty‐eight cases of subacute sclerosing panencephalitis (SSPE) were reviewed. Deterioration in school performance, personality changes, and seizures were common early symptoms. Initial examination frequently showed myoclonus, spasticity, and extrapyramidal dysfunction, and in two‐thirds of the patients these findings were asymmetrical or focal. Retinitis or papilledema was present on initial examination in 50% of the patients. At last follow‐up 24 children had died, with a mean survival of 42 months. Most patients reached a state of severe neurological impairment within 13 months. Subsequent evidence of improvement was noted in 10 children and was sustained in 4. Fifteen patients received antiviral treatment. Ten treated patients died from 5 to 133 months (mean, 58) from onset of their illness, while 15 untreated patients survived a mean of 33 months.

Duration of survival appeared to be affected most by treatment with amantadine. Three patients treated with the drug were alive 97 to 139 months after onset of SSPE, and 5 died with a mean survival of 78 months. Five of 6 individuals treated with rifampin died after a mean survival of 27 months. Prolonged remissions occurred only in patients treated with amantadine. Although the number of treated individuals was small, our data suggest that amantadine may affect the natural course of SSPE.
Range of variations in motor unit potentials during reinnervation after traumatic nerve lesions in humans
Authors: Borenstein S. , Desmedt J. E.
Published: January 2005

Successive stages in the reinnervation of denervated muscle fibers following a complete or partial motor nerve lesion in humans were analyzed with the concentric needle electrode during voluntary contraction. Motor unit potential (MUP) patterns were displayed by coherent EMG, in which the potential triggers an oscilloscope sweep and a digital delay line enables the early part of the MUP to be displayed. After a complete nerve lesion, MUPs showing the response of as few as one to three muscle fibers can be observed. MUPs become highly polyphasic as more muscle fibers are incorporated. Marked latency jitter and intermittent blocking of components as well as the occurrence of linked potentials result in complex patterns that may be confused with a “myopathic” pattern of brief small spikes if conventional observation of free‐running sweeps is used alone. After a partial nerve lesion, the healthy motor axons achieve extensive collateral reinnervation of the denervated muscle fibers whereby linked potentials of various latencies are added after the original MUP.

Adequate understanding of these characteristic features can help clarify some current issues in electromyography. The mechanisms by which long‐latency linked potentials and desynchronized components are produced during remodeling of motor units in the course of regeneration involve a number of factors such as slow conduction along regenerated axon branches, ectopic motor end‐plates, insecure neuromuscular transmission, and muscle fiber atrophy.
Restoration of dopaminergic function by grafting of fetal rat substantia nigra to the caudate nucleus: Long‐term behavioral, biochemical, and histochemical studies
Authors: Freed William J. , Perlow Mark J. , Karoum Farouk , Seiger Ake , Olson Lars , Hoffer Barry J. , Wyatt Richard Jed
Published: January 2005

Motor deficits produced in rats by unilateral substantia nigra lesions have been found to be reduced by grafts of fetal rat substantia nigra to the dopamine‐denervated caudate nucleus. In the present study these grafts were examined behaviorally, histochemically, and biochemically over six‐ to 10‐month periods. The grafts were found to survive in a healthy condition and contain catecholaminergic cells and fibers after eight to ten months. Concentrations of dopamine in adjoining parts of the caudate nucleus were increased when examined six months after grafting. Apomorphine‐induced rotation was reduced by the grafts, and these reductions persisted for at least six months. Although signs of aging were observed in the brains of the host animals when sacrificed eight to ten months after grafting, the grafts remained healthy and showed no signs of aging or deterioration. It is concluded that substantia nigra grafts can become permanent, functional constituents of the brains of host animals with prior substantia nigra lesions.
Lymph nodes—A possible site for sympathetic neuronal regulation of immune responses
Authors: Giron Louis T. , Crutcher Keith A. , Davis James N.
Published: January 2005

Noradrenergic fibers were identified in rat cervical lymph nodes by fluorescence histochemistry and confirmed by radioenzymatic determination of norepinephrine. After superior cervical ganglionectomy, lymph node norepinephrine and noradrenergic fluorescence were strikingly decreased. The α‐adrenergic radioligand [3H] dihydroergocryptine bound to saturable sites on lymph node membranes (0.20 pmol/mg protein; affinity constant, 8 nM) with characteristics expected for α‐adrenergic receptor binding. The α‐adrenergic binding sites were present in high concentration in membranes from lymph node stroma but were negligible in membranes of lymphocytes teased from nodes. In lymph nodes the ratio of α‐adrenergic binding sites to norepinephrine content was higher than ratios of many other sympathetically innervated tissues such as iris or vas deferens. The presence of sympathetic innervation and the high concentration of α‐adrenergic binding sites relative to that innervation suggest that the sympathetic nervous system modulates an intrinsic lymph node function through α‐adrenergic mechanisms.
Treatment of periodic alternating nystagmus
Authors: Michael Halmagyi G. , Rudge Peter , Gresty Michael A. , John Leigh R. , Zee David S.
Published: January 2005

Two patients with longstanding acquired periodic alternating nystagmus (PAN) were treated with baclofen, 30 mg/day. Baclofen abolished the PAN and relieved oscillopsia in both patients but was ineffective in another patient with congenital PAN.
Epilepsy in adults
Authors: So Elson L. , Penry J. Kiffin
Published: January 2005

The past decade has seen advances in the management of patients with epilepsy. The development of practical long‐term electroencephalographic techniques, with or without simultaneous video recording, has increased the accuracy of diagnosis of seizure types. The technique also provides clinicians and investigators with a method for establishing the clinical efficacy of antiepileptic drugs and determining their therapeutic serum concentrations. Computerized tomography has enhanced the identification of structural brain lesions. Most of the reported CT abnormalities consist of diffuse and focal atrophies, mild ventricular dilatations, and porencephalies. CT has detected tumors in 8 to 10% of the patients regardless of age or type of seizure involved. New concepts of antiepileptic drug therapy have developed from the recognition of pharmacological properties peculiar to each agent. Determination of serum antiepileptic drug levels has to be utilized to reduce the problem of pharmacokinetic variability from one patient to another and in the same patient at different times, so that dosage can be individualized to achieve maximum therapeutic effects with least toxicity. Review of the literature on pregnancy in epileptic women shows that a third to half experienced more seizures during gestation. Reduced serum levels of most antiepileptic drugs have recently been observed during gestation. Infants of epileptic women taking antiepileptic drugs have a two to three times greater risk for congenital anomalies than infants of nonepileptic women. However, with the exception of oxazolidinediones, evidennce to date has not proved the teratogenicity of antiepileptic drugs. The role of genetic factors and the effect of seizures during pregnancy have not been determined.

Modest progress has been made in epilepsy rehabilitation, but serious problems still remain. The unemployment rate of persons with epilepsy is twice the national average. Half of those who are successfully employed did not disclose their disorder at the time of employment. Several prognostic indicators have been reported, but the validity of many of these indicators is questionable. For example, does shorter life expectancy apply to all subgroups, or does it vary according to seizure type and cause? The life expectancy, treatment response, and proability of remission in epileptic persons must be reevaluated after consistent applications of current methods of epilepsy management.
Diabetic thoracoabdominal neuropathy: Clinical and electrodiagnostic features
Authors: Sun Sallie F. , Streib Erich W.
Published: January 2005

Five diabetic patients developed upper back or abdominal pain associated with substantial weight loss in three. Electrophysiological evidence of associated thoracoabdominal somatic neuropathy was found in all cases. Signs of a generalized neuropathy were present in two patients, and four had asymmetrical proximal leg weakness. At least two have improved spontaneously. The syndrome is closely related to diabetic amyotrophy; it is probably more common than is recognized.
Clinical and electroencephalographic characteristics of midline parasagittal foci
Authors: Pedley Timothy A. , Tharp Barry R. , Herman Kathleen
Published: January 2005

We report the clinical and electroencephalographic (EEG) characteristics of 14 patients with midline parasagittal epileptogenic foci. Montages not including the vertex electrodes demonstrated the abnormal activity poorly or not at all. The clinical manifestations were diverse and included tonic‐clonic, atypical absence, and partial seizures. In the majority, no cause could be established. The interictal EEG abnormality did not predict the pattern of clinical attack. Half the patients had evidence of structural brain disease. Four patients had no history of seizures.
An ultramicroscopic study of skin and conjunctival biopsies in chronic neurological disorders of childhood
Authors: Arsenio‐Nunes M. L. , Goutières F. , Aicardi J.
Published: January 2005

One hundred twenty‐seven skin and 59 conjunctival biopsies were performed in 136 pediatric patients with chronic neurological disorders. Ultrastructural abnormalities were found in 48 of 72 patients with unequivocally progressive encephalopathies (67 of which could not be diagnosed by biochemical means) and in 4 patients with miscellaneous diseases (fetal rebulla syndrome and hereditary motor‐sensory neuropathy), but in none of 55 patients with static or dubiously progressive encephalopathies. Of the 48 positive biopsies in the group with progressive encephalopathies, 39 showed abnormalities characteristic enough to permit a specific diagnosis (lysosomal storage disorders, neuroaxonal dystrophy, ophthalmoplegia‐plus) in conjunction with a compatible clinical picture. In 9 cases no diagnosis could be made despite the finding of lamellar or vacuolar inclusions. In no case were discrepancies observed between the results of skin and conjunctival biopsies. Although biochemical analysis is the simplest and most effective technique for the diagnosis of most progressive encephalopathies, skin or conjunctival biopsy is a valuable procedure in patients without detectable enzyme deficiency.
Blepharospasm and oromandibular dystonia (Meige's syndrome) in sisters
Authors: Nutt John G. , Hammerstad John P.
Published: January 2005

Isolated idiopathic oromandibular dystonia and blepharospasm (Meige's syndrome) has generally been described as a nonfamilial disorder. This report describes Meige's syndrome in 2 sisters free of other neurological and psychiatric disease.
Inverse ocular bobbing
Authors: Knobler Robert L. , Somasundaram Mahendra , Schutta Henry S.
Published: January 2005

A new form of abnormal spontaneous eye movement termed inverse ocular bobbing is described. It consists of a slow downward eye movement followed by delayed quick return upward to midposition. It is contrasted with other types of abnormal vertical eye movements.
GM1 Gangliosidosis: Phenotypic variation in a single family
Authors: Farrell Donald F. , Ochs Ute
Published: January 2005

Infantile, juvenile, and adult forms of GM1 gangliosidosis have been well characterized. Certain genetic and biochemical studies have suggested that the phenotypic variation found in GM1 gangliosidosis results from different allelic mutations affecting the GM1 ganglioside β‐galactosidase locus and that different combinations of these mutations account for the clinical heterogeneity of this illness. A family in which both the infantile and juvenile forms of GM1 gangliosidosis occurred, the children sharing a common mutation of their acid β‐galactosidase activity, supports the allelic nature of these different clinical forms of the disease. From the observations made in this unique family, additional phenotypes of GM1 gangliosidosis might be anticipated.
GM1 Gangliosidosis: Enzymatic variation in a single family
Authors: Farrell Donald F. , Macmartin Marian P.
Published: January 2005

Acid β‐galactosidase activity can be separated into multiple molecular forms by isoelectric focusing on cellulose acetate membranes. The residual acid β‐galactosidase in the juvenile form of GM1 gangliosidosis has three bands of enzyme activity with an apparent isoelectric pH (pI) range from 4.9 to 5.2, whereas that in the infantile form has a single band with an apparent isoelectric pI of 5.2. Separation of residual acid β‐galactosidase into multiple molecular forms by analytical isoelectric focusing demonstrates enzymatic differences that can be correlated with the allelic mutations that affect the GM1 ganglioside β‐galactosidase locus.
The effect of thiamine deficiency on local cerebral glucose utilization
Authors: Hakim A. M. , Pappius H. M.
Published: January 2005

Rats maintained on a thiamine‐free diet for two to seven weeks and control animals were studied by the [14C]deoxyglucose technique prior to the development of histological lesions. This technique permits measurement of local cerebral glucose utilization (LCGU) in discrete nuclei and tracts. Levels of thiamine in brain and blood were also determined. In the 41 central nervous system (CNS) structures in which it was measured, cerebral glucose utilization decreased with diminishing concentration of cerebral thiamine. Thus, the primary metabolic consequence of thiamine deficiency is a widespread reduction in cerebral glucose utilization. Furthermore, with decreasing cerebral thiamine concentrations, glucose utilization declined more rapidly in many of the structures which in humans develop histological lesions with prolonged thiamine deficiency than in structures less susceptible to the development of lesions. One determinant of the specific distribution of histological lesions occurring in human thiamine deficiency may be the variable rate at which the CNS structures lose their metabolic activity with continuing thiamine deficiency.
Neurogenic pulmonary edema in unexpected, unexplained death of epileptic patients
Authors: Terrence Christopher F. , Rao Gutti R. , Perper Joshua A.
Published: January 2005

Eight cases of unexpected, unexplained death in young ambulatory epileptics were examined postmortem with special attention to the heart and lungs. Lung weights uniformly exceeded the expected value, with gross evidence of hemorrhagic pulmonary edema. Microscopic examination revealed moderate to severe pulmonary edema with protein‐rich fluid as well as alveolar hemorrhage. There was no evidence of recent or old myocardial disease.

Although death due to a seizure is usually thought to be almost instantaneous, the neurogenic pulmonary edema exemplified by these cases takes time to develop and may be remediable. The high frequency of absent or nontherapeutic anticonvulsant levels at the time of death in these patients may play in a possible centrally mediated adrenergic cause of neurogenic pulmonary edema and ventricular arrhythmia.
Chronic GM1 gangliosidosis presenting as dystonia: I. Clinical and pathological features
Authors: Goldman James E. , Katz David , Rapin Isabelle , Purpura Dominick P. , Suzuki Kinuko
Published: January 2005

Clinical and pathological studies are reported from investigation of a 27‐year‐old man with GM1 gangliosidosis who experienced a slowly progressive dystonia that began about age 4, primarily affected the face and limbs, and eventually became almost totally incapacitating. There was only mild intellectual deterioration; myoclonus, seizures, and macular cherry‐red spots were never observed. Postmortem examination revealed intraneuronal storage, localized predominantly to the basal ganglia, in which neurons contained round, multilamellated inclusions. Golgi studies revealed meganeurites arising from medium spiny neurons. Other areas of the central nervous system appeared relatively unaffected, although small basilar dilatations were observed in scattered cortical pyramidal neurons and Purkinje cell dendrites showed focal swellings. Vacuolated cells of the reticuloendothelial system were observed, including Kupffer cells and histiocytes in the spleen, marrow, and intestinal tract. Biochemical analysis revealed a generalized β‐galactosidase deficiency with specific accumulation of GM1 ganglioside in the basal ganglia.
Local cerebral glucose utilization in thermally traumatized rat brain
Authors: Pappius H. M.
Published: January 2005

Local cerebal glucose utilization (LCGU) Was studied using the 14C‐deoxyglucose method in rats with focal thermally induced lesions in the left parietal cortex. A depression of LCGU developed with time after production of the lesion, being most prominent throughout the cortical areas of the lesioned hemisphere: 42 ± 2% (SE) of normal 3 days after the lesion, was induced. Corresponding results in other regions were: contralateral cortical areas, 86 ± 2%; ipsilateral and contralateral subcortical structures, 74 ± 2% and 84 ± 1%, respectively. Brainstem structures were not affected. In white matter, bilateral depression LCGU reached its peak 24 hours after production of the lesion—the reduction ipsilateral to the lesion was 61 ± 4% of normal and the contralateral reduction was 64 ± 4%. LCGU returned to normal within 5 days in all affected areas. No corresponding change in local cerebral blood flow were observed. These results suggest a widespread depression in the functional state of the traumatized brain.
Canine inherited ataxia
Authors: Cork Linda C. , Troncoso Juan C. , Price Donald L.
Published: January 2005

A previously unrecognized canine disorder consists of familial cortical cerebellar degeneration with many characteristics that are similar to inherited ataxias in humans. The disease occurs in Gordon setters and appears to be inherited as an autosomal recessive trait. The dogs appear normal during the first 6 months of life, but betwen 6 and 12 months of age they develop a broad‐based gait and dysmetria, followed by truncal ataxia. Nystagmus occurs late. The disorder is slowly and insidiously progressive. Neuropathological examination shows atrophy of the cerebellar cortex, particularly the pars in termedia. There is loss of Purkinje and granule cells, more severe in older dogs. Other parts of the neuraxis appear normal. These pathological changes resemble those of the familial human cerebellar cortical atrophies.
Baclofen in the treatment of staff‐man syndrome
Authors: Miller Frank , Korsvik Holly
Published: January 2005

Baclofen, 90 mg daily, was given to a patient with stiff‐man syndrome diagnosed by electromyography and clinical criteria. The patient, bedridden for three years, experienced a decrease in rigidity, cramping, stiffness, and spasm and was able to walk on the thirtieth day of treatment.
A comparison of stapedial reflex fatigue with repetitive stimulation and single‐fiber emg in myasthenia gravis
Authors: Kramer Lynn D. , Ruth Roger A. , Johns Michael E. , Sanders Donald B.
Published: January 2005

The pattern of stapedial reflex fatigue in response to pulsed acoustic stimulation was measured and compared to results of repetitive nerve stimulation and single‐fiber electromyography (EMG) in 89 patients with myasthenia gravis. Studies were also made on 22 patients with other neuromuscular disorders and 40 control subjects with no evidence of neuromuscular impairment. Stapedial reflex fatigue exceeded normal control values in 84% of the patients with myasthenia gravis. Repetitive stimulation and single‐fiber EMG measurements were abnormal in 56% and 91% of this same population, respectively. Stapedial reflex abnormalities were most prevalent in patients with mild forms of myasthenia (predominantly ocular or oropharyngeal weakness). Of 22 nonmyasthenic patients with neuromuscular disease tested, 6 had abnormal stapedial reflex fatigue according to our normal values, indicating that this form of testing also detects other diseases of the motor unit. The measurement of stapedial reflex fatigue is painless, is easy to perform, and requires minimal patient cooperation. Due to the relatively high occurrence of abnormal stapedial reflex fatigue in patients with myasthenia gravis, this procedure appears to have considerable potential value in screening and monitoring patients for the presence of defects in neuromuscular transmission.
Prognosis of optic neuritis with special reference to cerebrospinal fluid immunoglobuhs and measles virus antibodes
Authors: Nikoskelainen Eeva , Frey Harry , Salmi Aimo
Published: January 2005

Forty‐eight patients with optic neuritis (ON), first seen in 1970 to 1973, were neurologically and neuroophthal mologically reexamined after 7 to 10 years. Twenty‐seven patients (56%) had probable MS, and 9 (19%) had possible MS. During the attack of ON, the cerebrospinal fluid (CSF) samples and serum/CSF measles antibody ratios were studied. Twenty patients had increased relative immunoglobulin G (IgG % of total protein) in their CSF; 19 of these had probable or possible MS. However, 17 of 28 patients with a normal relative IgG value had also developed MS. CSF electrophoresis was abnormal in 20 patients with ON; reexamination showed that 19 had probable or possible MS. Sixteen of 27 patients with normal electrophoresis had also developed MS. Serum/CSF measles antibody ratio had decreased in 19 patients; 13 of these had probable MS and 3 had possible MS. Of 29 patients with a normal measles antibody ratio, 14 had probable MS and 6 had possible MS. The conclusion is that examination of the CSF in ON gives valuable prognostic information because increased relative IgG, abnormal electrophoresis, or a decreased measles antibody ratio implies a high risk of developing MS. A normal CSF does not, however, rule out the possibility of dissemination.
Viral antibody activity of oligodonal and polyclonal immunoglobulins synthesized within the central nervous system in multiple sclerosis
Authors: Rostrom Björn , Link Hans , Laurenzi Maria A. , Kam‐Hansen Slavenka , Norrby Erling , Wahren Britta
Published: January 2005

Thin‐layer polyacrylamide gel isoelectric focusing (PAG IEF), a very high capacity method for separating immunoglobulins (Ig), was performed on cerebrospinal fluid (CSF) and serum. It was followed by antigen immunofixation with measles, mumps, herpes simplex (HSV), and rubella virus antigens and anti‐human Ig autoradiography in order to demonstrate viral antibodies in separated Ig zones. Two of 11 control patients and 21 of 25 patients with multiple sclerosis (MS) displayed one or more zones of viral antibodies in the CSF without any counterpart, or with distinctly fainter zones, in the serum. Such reaction patterns were taken to indicate the possibility of intrathecal antibody synthesis. Antibody synthesis to measles was found in one to five zones in 76% of the patients with MS; antibody zones were found to HSV in 36% of the patients, to mumps in 12%, and to rubella in 12%. In 36% of the patients, two or three different antibody specificities (of which one was always measles) were found simultaneously in individual autoradiogram zones. For all viral antibodies detected in the CSF autoradiograms, their counterparts in oligoclonal or polyclonal IgG zones (or both) were demonstrable by PAG IEF of the corresponding CSF. The majority of patients with MS also had one or more oligoclonal CSF IgG zones without known antibody specificity. Antigen immunofixation and autoradiography are mainly qualitative. It is not known whether the viral antibodies present in oligoclonal or polyclonal IgG zones in MS CSF reflect a polyclonal B cell activation, a disease‐specific immune reaction, or both.
Adverse effects of phenobarbital on morphological and biochemical development of fetal mouse spinal cord neurons in culture
Authors: Bergey G. K. , Swaiman K. F. , Schrier B. K. , Fitzgerald S. , Nelson P. G.
Published: January 2005

The effects of chronic phenobarbital treatment on neuronal development were assessed in cell cultures of fetal rodent spinal cord. The activity of the principal enzyme involved in acetylcholine synthesis, choline acetyltransferase (CAT), and counts of large spinal cord neurons were used as indicators of neuronal development. Phenobarbital (30 to 120 m̈g/ml) added to cultures for two‐ or six‐week periods produced dose‐dependent decreases in both CAT activity and neuron counts. Barbituric acid at doses equimolar to those at which phenobarbital produced these decreases had no effect.
Prolonged intracerebral infection with poliovirus in asymptomatic mice
Authors: Miller James R.
Published: January 2005

A heretofore unrecognized prolonged asymptomatic infection has been identified in mice intracerebrally inoculated with Lansing strain type II poliovirus. Virus was detected by infectivity assay and nucleic acid hybridization and was found in brains as long as 77 days after injection. Viral RNA replication occurred, indicating that persisting virus was not an inactive residuum of the infecting dose. Virus was sometimes found by infectivity even when viral RNA could not be demonstrated by hybridization, but detection by nucleic acid hybridization alone never occurred. Among 56 animals–including mice that died of infection, mice with prolonged infection, and survivors from which virus was not recovered–no animal had a serum neutralization titer above 1:2 in a plaque reduction assay Moreover, brain homogenates of 30 survivors without virus did not have neutralization titers above 1:4 in the plaque reduction assay. These findings were the same as in mock‐infected mice.
Temporospatial effects on orthodromic sensory potential propagation during ischemia
Authors: Nielsen Viggo Kamp , Kardel Troels
Published: January 2005

Sensory potentials recorded at two to four sites along the median nerve during ischemia in normal subjects were analyzed with respect to the effects of conduction distance and duration of ischemia. Latency increased rectilinearly with the squared duration of ischemia in minutes, and the rate of the latency increase along the nerve was a power of the preischemic latency, a measure of conduction distance. This finding indicates a temporospatial slowing of sensory conduction velocity of ischemic nerves. Calculated latencies fitted with measured values within ±0.2 msec in 95% of 385 recordings. The compound potential amplitude decreased exponentially with the concomitant increase in temporal dispersion, which was the major cause for the decrease in amplitude during the first 20 minutes of ischemia. The nerve conduction pattern during ischemia may serve as a model for biochemical axon membrane dysfunction, a possible factor in the pathogenesis of toxic‐metabolic neuropathies.
Altered muscle polyamine levels in human neuromuscular diseases
Authors: Kaminska Anna M. , Stern Lawrence Z. , Russell Diane Haddock
Published: January 2005

Intracellular polyamine levels were measured in 17 patients with neuromuscular diseases and 13 normal controls and were correlated with morphological changes in muscle biopsies. Elevated polyamine levels were found in muscles with marked morphological changes, regardless of the nature of the disease process.
Alpha‐pattern coma in a 2‐month‐old child
Authors: Homan Richard W. , Jones Mary Gene
Published: January 2005

A 2‐month‐old infant is described in whom the electroencephalographic finding of alpha‐frequency activity was correlated with a clinical state of unresponsiveness. This condition occurred following a period of hypoxia and was similar to alpha‐pattern coma in adults. The finding of alpha‐pattern coma in a young infant suggests that alpha‐frequency activity in comatose states is abnormally generated since alpha‐frequency activity normally is not found in this age group.
Headache
Authors: Lance James W.
Published: January 2005

Present views on the cause and treatment of temporal arteritis, trigeminal neuralgia, pain arising from the neck, benign intracranial hypertension, and other headaches of intracranial origin are summarized. The clinical components of migraine are correlated with recent studies of cerebral blood flow, monoamine changes, and the platelet release reaction. Psychological, physiological, and pharmacological management is based on the holistic concept of migraine as an uninhibited protective reaction. Cluster headache is subdivided into three varieties which respond preferentially to different medication. Tension headache may depend more on vascular mechanisms than excessive muscle contraction, but treatment is still directed at behavioral management and relaxation training with the aid of antidepressant therapy.
Some morphometric aspects of the brain in senile dementia of the alzheimer type
Authors: Terry Robert D. , Peck Arthur , DeTeresa Richard , Schechter Ruben , Horoupian Dikran S.
Published: January 2005

Image analysis apparatus was used to count and measure glial and neuronal perikarya in ten size classes in the midfrontal region and superior temporal gyrus of 18 patients with senile dementia of the Alzheimer type for comparison with 12 age‐matched normal specimens. Brain weight was about 8% less in the dementia group. The major differences had to do with larger neurons, which in dementia were reduced by about 40% in the frontal cortex and 46% in the temporal region. The concentration of neuritic plaques did not correlate significantly with brain weight, cortical thickness, or cell counts.
Newborn brain weight in relation to maturity, sex, and race
Authors: Ho Khang‐Cheng , Roessmann Uros , Hause Lawernce , Monroe Gerald
Published: January 2005

Fresh brain weight, gestational age, body weight, sex, and race were collected from autopsy records of 782 newborns over a 10‐year period. The brain weight of the mature newborn does not differ between males and females of between white and black infants. For the premature, however, brain weight is heavier in white males than in black males and inwhite females than in black females. These differences are similar to those found for adults in our previous studies, and they correspond to differences in gestational age and body weight in the subgroups. The differences in brain weight between races are best explained on the basis of environmental factors.
Olivopontocerebellar atrophy in children: A report of seven cases in two families
Authors: Colan Richard V. , Snead O. Carter , Ceballos Ricardo
Published: January 2005

We present seven cases of progressive ataxia with onset in childhood along with pathological findings in three patients. One patient showed pure cerebellar degeneration and had no visual changes. His brother had classic changes of olivopontocerebellar atrophy with profound amyotropy but no visual changes. A third family member had similar findings with pathological findings intermediate in severity between the first two. The mother and daughter, who are living, are ataxic and have macular degernation. In a second pedigree, all patients affected in three generations were male, but the disease began during adulthood in the first two generations. Myoclonic seizures occureed in the majority of patients.
Neurological manifestations of accidental hypothermia
Authors: Fischbeck Kenneth H. , Simon Roger P.
Published: January 2005

In a series of 97 patients with accidental hypothermia, alcohol abuse and Wernicke's encephalopathy were prominent causes. Pulse, systolic blood pressure, and respiratory rate were all found to decline with decreasing temperature, and there were significant changes (p < 0.01) in level of consciousness, pupillary response, reflexes, and muscle tone. However, even in the lowest temperature range (20° to 27°C, or 68° to 80°F), 6 of 18 patients remained verbally responsive and 10 had intact reflexes. Neither eye movement abnormalities nor extensor plantar responses correlated directly with the degree of hypothermia.
ACTH‐induced cortisol production in multiple sclerosis
Authors: Snyder Bruce D. , Lakatua David J. , Doe Richard P.
Published: January 2005

We measured the total daily urinary cortisol produced by each of 8 patients with multiple sclerosis during intravenous therapy with adrenocorticotropic hormone (ACTH). Results indicated marked daily variation from patient to patient as well as in the same patient, suggesting that ACTH infusions do not result in reliable, consistent, high‐level production of cortisol. We attempted to express the quantity of cortisol produced as an equivalent amount of oral prednisone. This comparison is difficult to make; but, given certain assumptions, the figures suggest that 40 U or 80 U ACTH infusions do not elicit the quantities of steroid generally considered to be indicated for the therapy of major allergic, autoimmune, or neoplastic diseases.
Central somatosensory conduction after head injury
Authors: Hume Ann L. , Cant B. R.
Published: January 2005

In 94 patients with head injuries, conduction between the lower brainstem and the cerebral cortex was studied by recording evoked potentials from the scalp and neck following stimulation of the median nerves. A conduction time 3 SD or more above the normal mean (5.74 ± 0.46 msec) or the absence of an evoked potential over one or both hemispheres was considered abnormal. During successive periods in the first 35 days after injury, the evoked potentials correlated with the outcome (classified as good or not good) in 75 to 84% of patients. Within 3 1/2 days the outcome was correctly predicted in 38 of 49 patients (78%). Six of the 7 surviving patients with persistent asymmetries of the cerebral evoked potentials remained hemiplegic. Serial studies over one year showed that both conduction time and amplitude recovered exponentially over many months, but differences persisted between the patients who made a good recovery and those who remained disabled.
Biophysical and pathological effects of cryogenic nerve lesion
Authors: Myers Robert R. , Powell Henry C. , Heckman Heidi M. , Costello Michael L. , Katz Jordan
Published: January 2005

Changes in endoneurial fluid pressure (EFP) and morphology were studied in rat sciatic nerves frozen for 60 seconds with a cryoprobe designed for human cryoanalgesia. The onset of increased EFP was rapid, and a peak of 23 cm H2O was reached within 90 minutes after injury. EFP levels returned to normal 32 days after freezing. The peak value represents the highest EFP yet recorded in an experimental neuropathy. Microscopic examination revealed severe vascular injury as the probable mechanism of edema, with leakage of horseradish peroxidase tracer at the site of injury and diapedesis of polymorphonuclear cells through vessel walls. Wallerian degeneration was also observed in segments of nerve distal to the site of injury. Analysis of EFP data revealed a biphasic pattern of endoneurial edema: initial marked pressure elevation subsides within hours but is followed by a second peak several days later. We interpret this to suggest superposition of two separate pathological processes following cold injury. At first, extensive vascular damage permits plasma and cellular extravasation, which rapidly increases EFP. Subsequently, nerve fibers undergo wallerian degeneration, a process associated with elevated EFP, which is maximal 6 days after injury.
Myotonic dystrophy: Calcium‐dependent phosphatidic acid synthesis in erythrocytes
Authors: Moore R. B. , Appel S. H. , Plishker G. A.
Published: January 2005

Recently it was reported that calcium‐dependent phosphatidic acid synthesis in erythrocytes of patients with myotonic muscular dystrophy (MyD) is markedly impaired when compared to that in control subjects. Using 32P‐loaded erythrocytes, we found no significant difference in the levels of 32P‐phosphatidic acid synthesized after exposure to calcium and its ionophore A23187 between patients with MyD and controls. In a batch experiment typical of the experiments with 32P, a twofold increase of phosphatidic acid in both groups was determined by inorganic phosphate measurements. Thus, the specific activity of the 32P‐phosphatidic acid increased four‐ to five‐fold in response to calcium. Analyses of 32P‐polyphosphoinositide breakdown in ghosts and in adenosine triphosphate‐depleted erythrocytes also appeared normal for patients with myotonic muscular dystrophy. Possible discrepancies between the results presented here and those reported previously are discussed.
Human leukocyte antigen in torticollis and other idiopathic dystonic syndromes
Authors: Korein Julius , Willoughby Ernest , Pollack Marchilyn S. , Levidow Lucie , Dupont Bo
Published: January 2005

Two groups totaling 67 patients with idiopathic focal, segmental, and generalized dystonia, including torticollis, were compared with normal controls to determine whether there was a difference in the frequency of A, B, and C locus human leukocyte (HLA) antigens. The results indicated no statistically significant deviations in HLA antigen frequencies between the patients and the normal controls. Thirteen of the patients with idiopathic torsion dystonia were compared with normal controls for DR locus antigens. A trend of increased DR3 antigens observed in the patients may be significant. HLA genotyping of parents and children in nine families was also studied to determine if an HLA‐linked factor could be related to the dystonic syndrome in the children. The results were indeterminate, suggesting that further family studies are required to resolve this issue.
Treatment of hyperkinetic movement disorders with tetrabenazine: A double‐blind crossover study
Authors: Jankovic Joseph
Published: January 2005

Tetrabenazine, a presynaptic monoamine depleting agent, has been reported to have an ameliorating effect in a variety of hyperkinetic movement disorders. In a double‐blind crossover trial of tetrabenazine versus placebo, 19 patients with a variety of hyperkinetic movement disorders were evaluated. During the evaluation period, all but 4 patients were treated for three or more weeks at a maximum dosage of 200 mg per day. The patients were examined and rated using clinical assessment of hyperkinesia, and movies of their activities were randomized and rated by an independent group of neurologists. A good correlation was found between the clinical examination scale and the film analysis score. Improvement was seen in all 4 patients with tardive dyskinesia, 4 of 6 patients with Meige disease, and 5 of 6 patients with other dystonias. One patient with Huntington disease showed marked improvement and 2 patients with congenital choreoathetosis showed only mild improvement. The most frequent side effects included daytime drowsiness, drooling or sialorrhea, insomnia, restlessness and anxiety parkinsonian features, and mild postural hypotension. The adverse effects resolved with continued administration or with reduction in dosage. Tetrabenazine is a useful and safe therapeutic agent in some patients with hyperkinetic movement disorders.
Asymmetrical volumes of the right and left frontal and occipital regions of the human brain
Authors: Weinberger Daniel R. , Luchins Daniel J. , Morihisa John , Wyatt Richard Jed
Published: January 2005

The volume of a portion of the frontal and occipital lobes was measured in 40 brains in the Yakovlev Collection. In 32 cases the volume of the right frontal lobe was larger than that of the left; in the same number of cases the left occipital lobe was larger than the right (p < 0.01). The asymmetries existed in fetal brains as early as 20 weeks of gestation. The results confirm observations made with computed tomography and indicate that this nonrandom asymmetrical pattern is an early manifestation of human brain development.
Abnormal vertical eye movements in the locked‐in syndrome
Authors: Larmande P. , Hénin D. , Jan M. , Elie A. , Gouazé A.
Published: January 2005

As a rule, vertical ocular movements are thought to depend on mesencephalic structures, horizontal gaze on pontine structures. The ocular motility examination of a patient with pontine hemorrhage and no lession of the mesencephalon showed bilateral palsy of horizontal gaze and a dissociated loss of vertical movements: saccades were slowed, while pursuit managements were unchanged. The findings suggest that the pontine reticular formation is necessary in generation of normal vertical ocular saccades.
Experimental models of virus‐induced demyelination of the central nervous system
Authors: Dal Canto Mauro C. , Rabinowitz Stanley G.
Published: January 2005

One of the arguments in favor of a viral pathogenesis for multiple sclerosis is the existence of several experimental and natural animal models of virus‐induced primary demyelination. This review deals comprehensively with such models. Well‐known examples of demyelinating viral infections in their natural host are JHM, Theiler, visna, and canine distemper encephalomyelitides. Recent reports of experimental murine infections with pathogens such as vesicular stomatitis, Chandipura, herpes simplex, Venezuelan equine encephalomyelitis, and Semliki Forest viruses are also discussed. The thrust of the review is to include viral models suspected of producing primary demyelination on an immunopathological basis.
Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment
Authors: Dyck Peter James , O'Brien Peter C. , Oviatt Karen F. , Dinapoli Robert P. , Daube Jasper R. , Bartleson John D. , Mokri Bahram , Swift Thomas , Low Phillip A. , Windebank Anthony J.
Published: January 2005

Of 40 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 28 completed a controlled three‐month trial of prednisone. Prednisone was shown to cause a small but significant improvement over no treatment in scored neurological disability, some measures of computer‐assisted sensory detection threshold, graded muscle strength, and some attributes of nerve conduction. No subset of patients was more likely than another to be responsive to prednisone; those with a progressive course were as likely to be responsive as recurrent cases. This finding provides further justification for classifying progressive with recurrent cases as CIDP and demonstrates that prednisone treatment should not be withheld from patients with progressive disease.
Prolonged migraine aura status
Authors: Hass David C.
Published: January 2005

Two patients with migraine experienced a prolonged migraine aura status consisting of frequent recurrence of auras, hour after hour, day after day, for successive weeks.
Regulation of local cerebral blood flow in normal and hypoxic newborn dogs
Authors: Cavazzuti M. , Duffy T. E.
Published: January 2005

Local cerebral blood flow (LCBF) was measured autoradiographically in newborn puppies by an indicatorfractionation technique using 4‐iodi‐[14C]antipyrine as the diffusible indicator. Measurements were obtained in unanesthetized, normotensive animals, and the sensitivity of blood flow to hypercapnia and acute hypoxia was determined in 32 brain structures. LCBF in normal and hypoxic puppies was correlated with local cerebral glucose utilization (LCGU) obtained under the same experimental conditions (Duffy et al, 1982). In normocapnic (PaCO2 33 mm Hg) control animals, highest rates of blood flow were found in gray matter nuclei of the brainstem, in the medulla oblongata, and in the posterolateral nucleus of the thalamus (50 to 77 ml/100 gm/min); far lower flows were recorded among white matter structures (5 to 11 ml/100 gm/min). The vasodilatory response to both hypercapnia and hypoxia was greatest among brainstem gray matter structures, intermediate among cortical and diencephalic gray matter structures, and least in white matter. When LCBF was plotted as a function of LCGU for control animals, a positive linear correlation was obtained for all structures (p < 0.001), implying that in newborns, as in adults, cerebral blood flow and metabolism are physiologically coupled. In hypoxic puppies, no consistent relationship between LCGU and LCBF could be demonstrated; however, there was a suggestion that the two measurements correlated inversely, presumably reflecting enhanced anaerobic glycolysis in structures (e.g., hemispheric white matter) that were not adequately protected by compensatory hyperemia. White matter damage, a frequent complication of perinatal hypoxia‐asphyxia, may be a consequence in part of the limited capacity of white matter to vasodilate in response to the chemical “signals” of hypercapnia and lactic acidosis.
Sympathetic nerves exert a chronic influence on the intact vasculature that is age related
Authors: Rusterholz David B. , Mueller Shirley M.
Published: January 2005

The hypothesis of this study is that the chronic presence of intact sympathetic innervation influences the structure, and thereby function, of the rabbit ear vascular bed, and that this influence may be age related. Therefore we examined the effect of chronic sympathetic denervation on vascular resistance of the rabbit ear nine to ten weeks after unilateral superior cervical ganglionectomy. Two age groups were studied: growing juvenile rabbits denervated at 4 weeks of age and adult rabbits denervated at 16 weeks. We assessed the flow‐pressure (resistance) curves of the chronically denervated and contralateral innervated isolated rabbit ears during maximum dilation. The flow‐pressure curve was significantly shifted downward in the denervated compared to the contralateral innervated ears of juvenile rabbits operated on at 4 weeks (p < 0.001) but was not different in adult rabbits operated on at 16 weeks. These results support the concept that the sympathetic nerves chronically influence blood vessel properties in growing animals, apart from their acute effect on vasomotor tone.
Clonally restricted B cells in peripheral blood of multiple sclerosis patients: Kappa/lambda staining patterns
Authors: Hauser Stephen L. , Weiner Howard L. , Ault Kenneth A.
Published: January 2005

The presence of clonally restricted B lymphocytes in the peripheral blood of patients with multiple sclerosis (MS) was investigated using a cytofluorometric technique able to detect lymphocytes having homogeneous amounts of surface immunoglobulin of one light chain class. Thirty‐five of 76 analyses (46%) performed on 63 MS patients were positive for increased numbers of clonally restricted B cells as compared with only 7 of 27 (26%) in patients with other neurological diseases (p < 0.05) and 9 of 36 (25%) in control subjects (p < 0.05). This B cell population expressed predominantly kappa light chain determinants in 16 of 18 MS patients (89%) but in only 6 of 11 (55%) controls (p < 0.005). There was no correlation between the presence of abnormal B cell clones in peripheral blood and either disease activity or serum antimeasles antibody titers. The results demonstrate that there are increased numbers of clonally restricted B cells in the peripheral blood of MS patients which may be related to the oligoclonal immunoglobulin found in the serum and cerebrospinal fluid.