bluuebunny/crossref_metadata_2025 huggingface dataset:
Cerebellar cortex ultrastructure in ataxia‐telangiectasia
Authors: Paula‐Barbosa M. M. , Ruela C. , Tavares M. A. , Pontes C. , Saraiva A. , Cruz C.
Published: January 2005

An ultrastructural study of a biopsy fragment from the cerebellar cortex of a patient with ataxia‐telangiectasia was performed. There was a sharp reduction in the number of Purkinje cells and an increase in the number of Golgi epithelial cells. Despite the degeneration of Purkinje cell dendrites, the volumetric density of parallel fiber varicosities and the number of synapses in the molecular layer were only slightly reduced. No qualitative nor quantitative abnormalities were noted in the granular layer.

An analysis is made of the severe changes in cerebellar circuitry of this patient and their probable functional implications.
Dissociation of early SEP components in unilateral traumatic section of the lower medulla
Authors: Mauguière F. , Courjon J. , Schott B.
Published: January 2005

Spinal and scalp early SEPs were recorded, using a noncephalic reference electrode, in a patient with a traumatic cervicomedullary lesion causing unilateral loss of position sense. Cervical N11 and N13 and scalp‐recorded far‐field P14 SEPs were clearly dissociated following stimulation of the affected side. The findings suggest that the P14 component is generated above the foramen magnum, whereas the cervical N13 has a spinal generator.
Neurotoxicity of radiological contrast agents
Authors: Junck Larry , Marshall William H.
Published: January 2005

The most important complications of intravascular administration of contrast agents include idiosyncratic (anaphylactoid) reactions, shock, congestive heart failure, cardiac arrhythmias, acute renal failure, and neurotoxic effects. The incidence of serious neurotoxic effects is low. Entry of contrast agents into the central nervous system normally is limited but may be increased by osmotic opening of the blood‐brain barrier with cerebral arteriography or arch aortography. Most neurotoxic effects are thought to represent direct effects of the contrast agent on brain or spinal cord. Adverse effects with arteriography include seizures, transient cortical blindness, brain edema, injury. Most cases of focal brain deficit (other than cortical blindness) are attributed to embolism secondary to the catheter. Seizures may occur with intravenous administration, especially in patients with brain tumors or other processes disrupting the blood‐brain barrier. The most important adverse effects observed with myelographic agents include acute and chronic meningeal reactions with iophendylate, and seizures and transient encephalopathy with metrizamide.
Regional metabolic changes in the spinal cord related to spinal shock and later hyperreflexia in monkeys
Authors: Schwartzman Robert J. , Eidelberg Eduardo , Alexander Guillermo M. , Yu Jen
Published: January 2005

The regional metabolic rate for glucose (rMRglu) was studied in macaque monkeys after spinal cord transection at T10, both during spinal shock and after reflexes had returned. The rMRglu was measured in all Rexed layers in cord segments both caudal and rostral to the level of the transection utilizing the quantitative [14C]deoxyglucose technique. The monkeys in spinal shock exhibited a significant increase in rMRglu in Rexed layer I and a significant reduction in layers VI to IX of the lumbosacral cord. In contrast, after reflexes had returned the rMRglu was increased in all Rexed layers of the lumbosacral cord; this increase was significant in layers I, V, and VI.
“Primary” spinal intramedullary lymphomas: A review
Authors: Hautzer N. W. , Robitaille Y. , Aiyesimoju A.
Published: January 2005

Primary spinal lymphoma carries a low incidence and poor prognosis. The most reliable diagnostic signs have been segmental swelling of the cord on myelography and increased cellularity of cerebrospinal fluid. Biopsies of spinal tumors or their cerebral extensions showed lymphoma of the non‐Hodgkin type only. Peroxidase‐antiperoxidase staining for light chains helps to distinguish well differentiated lymphomas from infectious processes in some instances. A lymphoma in an immunosuppressed patient fulfilling all criteria for primary growth in the spinal cord is described, and the case compared with others in the literature. Combined radiotherapy and chemotherapy provided a 48‐month survival in one patient.
Neurological complications of vasculitis
Authors: Moore Patricia M. , Cupps Thomas R.
Published: January 2005

The vasculitides are a group of disorders that include the polyarteritis nodosa group of systemic necrotizing vasculitides, hypersensitivity vasculitis, Wegeners's granulomatosis, lymphomatoid granulomatosis, giant cell arteritis, Behçet's disease, and isolated angiitis of the central nervous system. Classification is based on clinical, angiographic, and histological features. The frequency and distribution of neurological involvement vary with the underlying disorder and may provide the initial symptoms. Polyarteritis nodosa and Wegener's granulomatosis may affect the central and peripheral nervous systems, whereas isolated angiitis of the central nervous system and Behçet's disease affect the central nervous system alone. Neurological dysfunction occurs in 80% of patients with polyarteritis nodosa and fewer than 10% of patients with hypersensitivity vasculitis.

The mechanism of neurological dysfunction in the vasculitides is tissue ischemia. The clinical effects of ischemia vary, and symptoms may be transient or prolonged. Mononeuritis multiplex, polyneuropathy, and stroke are frequent complications, but encephalopathies, cranial neuropathies, and brachial plexopathies are seen as well. The occurrence of symptoms late in the course of a disease suggests ischemia resulting from healed, scarred vessels as well as from those that are acutely inflamed; this is the case in Takayasu's arteritis and possibly also in polyarteritis nodosa. Treatment is based on identifying and removing the sensitizing agent when possible. Wegener's granulomatosis requires therapy with cyclophosphamide; temporal arteritis, with corticosteroids. In other vasculitides a balance must be reached between the progression of the disease and the side effects of immunosuppression.
Cerebellar blood flow and metabolism in cerebral hemisphere infarction
Authors: Wayne Martin W. R. , Raichle Marcus E.
Published: January 2005

Positron emission tomography was used to study the effect of supratentorial infarction on cerebellar metabolic rate for oxygen and cerebellar blood flow. In a control group of patients, the mean cerebellar metabolic rate for oxygen was 2.97 ± 0.11 (standard error of the mean [SEM]) ml−1 · min−1 · hg−1 and mean cerebellar blood flow was 41.1 ± 1.5 ml · min−1 · hg−1. No significant right‐left asymmetry in either cerebellar metabolic rate for oxygen or cerebellar blood flow was noted. Patients with frontal lobe infarction showed 16.8 ± 1.8% (cerebellar metabolic rate for oxygen) and 19.6 ± 2.1% (cerebellar blood flow) differences between cerebellar hemispheres, with the hemisphere contralateral to the cerebral infarction having the lower values. These differences were highly significant (p < 0.001). In addition, cerebellar blood flow and cerebellar metabolic rate for oxygen were significantly decreased in the ipsilateral cerebellar hemisphere (metabolism: 2.13 ± 0.19 ml · min−1 · hg−1; p < 0.002; blood flow: 35.2 ± 2.4 ml · min−1 · hg−1; p < 0.05). Patients with parietooccipital infarction also showed a significant bilateral decrease in cerebellar metabolic rate for oxygen (2.43 ± 0.11 ml · min−1 · hg−1) and cerebellar blood flow (34.6 ± 2.5 ml · min−1 · hg−1) relative to control subjects, but no significant cerebellar asymmetry. Our findings demonstrate a general depression of cerebellar blood flow and metabolism from cerebral hemisphere infarction unrelated to the site of infarction as well as a specific depression occurring contralateral to infarction involving the frontal lobe. These are among the first quantitative data concerning regional cerebellar metabolic rates for oxygen and cerebellar blood flow in humans.
Neuropathological features of a lupus‐like disorder in autoimmune mice
Authors: Rudick Richard A. , Eskin Thomas A.
Published: January 2005

Neurological syndromes are prominent in systemic lupus erythematosus, but the neuropathological mechanisms resulting in neurological dysfunction are unknown. We report a neuropathological study of the central nervous system in female NZB/W F1 mice, an animal model of systemic lupus erythematosus. NZB/W mice were studied at 3, 5, 8, 12, and 14 months of age, and 36‐month‐old female C57B16N/NIA mice were studied as aged controls. A lymphoproliferative process was identified in the central nervous system of 39% of 8‐ to 12‐month‐old and all 14‐month‐old NZB/W mice. Infiltrates of lymphocytes and plasma cells were seen in subarachnoid, choroid plexus interstitial, and Virchow‐Robin spaces. Lymphoid cells occasionally infiltrated brain parenchyma or accumulated as nodular masses. Concomitant visceral lymphoid infiltration was noted in 14‐month‐old mice. Dense deposits were seen ultrastructurally in the basal lamina of brain parenchymal capillaries of 14‐month‐old NZB/W mice. These dense deposits were similar in appearance to immune complexes described in glomerular basal lamina, and appeared concomitantly with an advanced lupus‐like glomerulopathy. Similar deposits were not observed in choroid plexus. The possible relevance of these neuropathological changes to human central nervous system lupus is discussed.
Natural killer cells activity in patients with multiple sclerosis given α interferon
Authors: Rice George P. A. , Casali Paolo , Merigan Thomas C. , Oldstone Michael B. A.
Published: January 2005

The purpose of this study was to examine the function and regulation of natural killer cells in vitro and in vivo in patients with multiple sclerosis. Of 12 patients who received 5 × 106 international units of human & interferon, 9 demonstrated an increase in natural killer cell activity within 48 hours as defined by the lysis of 51Cr‐labeled K‐562 cells. The activity was normal before treatment, unlike that of tumor‐bearing patients, and reached baseline levels within one week despite continuous interferon administration over the next six months. The same patients given a placebo preparation failed to show this enhanced natural killer cell activity.

We also studied K‐562 killing in 36 other patients and age‐ and sex‐matched control subjects and were unable to demonstrate any differences between the two groups or any correlation of natural killer cell function with disease activity. The in vitro augmentation of natural killer cell activity by purified measles virions, which is associated with the release of interferon, and by the isolated glycoproteins of measles virus, which activates natural killer cells without the extracellular release of interferon, was similar in both patients and control subjects. Further, the proportion of cells defined by the monoclonal antibody HNK‐1, which defines both natural killer cells and a small subset of cytotoxic T lymphocytes, was normal. In patients with multiple sclerosis, the normality of natural killer cell function, as defined by these several interrelated assays, speaks against a defect in this nonspecific antiviral defense mechanism in the pathogenesis of the disease.
Schwann cell remyelination of demyelinated axons in spinal cord multiple sclerosis lesions
Authors: Itoyama Yasuto , Webster Henry DeF. , Richardson Edward P. , Trapp Bruce D.
Published: January 2005

To investigate remyelination in multiple sclerosis lesions, we immunostained spinal cord sections from patients with multiple sclerosis and neurologically normal (control) patients with antisera to P0 protein, a major constituent of peripheral nervous system myelin, and myelin basic protein, which is found in both central and peripheral nervous system myelin. In sections from five of the eight patients with no clinical or pathological evidence of neurological disease, P0 immunostaining was confined to peripheral myelin sheaths in dorsal and ventral roots. They were intensely stained, and peripheral‐central nervous system transition zones were clearly demarcated. Sections from the other three control patients contained a few P0‐stained sheaths in the central nervous system near root entry zones or among marginal glia near the dorsal sulcus. Spinal cord sections from six of the ten patients with multiple sclerosis contained clusters of myelin sheaths immunostained by P0 antiserum. These regenerating sheaths of peripheral nervous system origin were most numerous in large lesions and were commonly located in central areas or peripherally near root entry zones. The sheaths were observed frequently in areas of active demyelination and appeared morphologically normal even when surrounded by debris‐filled macrophages. Near margins of small inactive plaques were a few basic protein‐stained oligodendroglia extending processes to thin basic protein‐stained sheaths. These regenerating sheaths of central origin were relatively uncommon and were not observed in areas of active myelin breakdown. Our observations suggest that Schwann cell remyelination is more extensive in spinal multiple sclerosis lesions than previously thought. In very large spinal cord plaques, Schwann cells also apparently have a more important role in myelin sheath regeneration than do oligodendroglia.
Neurological complications of acquired immune deficiency syndrome: Analysis of 50 patients
Authors: Snider William D. , Simpson David M. , Nielsen Surl , Gold W. M. Jonathan , Metroka Craig E. , Posner Jerome B.
Published: January 2005

Fifty patients with acquired immune deficiency syndrome had complications affecting the central or peripheral nervous systems or both. The patients were either male homosexuals, intravenous drug abusers, or recently arrived Haitian refugees. They ranged in age from 25 to 56. Central nervous system complications were of four kinds: (1) Infections included Toxoplasma gondii abscesses in 5 patients, progressive multifocal leukoencephalopathy in 2, cryptococcal meningitis in 2, Candida albicans in 1, and possible Mycobacterium avium intracellulare in 3. Eighteen patients suffered a subacute encephalitis possibly attributable to cytomegalovirus infection. (2) Tumors consisted of primary lymphoma of the brain in 3 patients and meningeal invasion by systemic lymphoma in 4. (3) Vascular complications included nonbacterial thrombotic endocarditis in 2 patients and cerebral hemorrhages in the setting of thrombocytopenia in 3. (4) Undiagnosed central nervous system problems were evidenced as focal brain lesions in 3 patients and self‐limiting aseptic meningitis in 4. Peripheral neuropathy occurred in 8 patients.
Polymorphous meningitis with atypical mononuclear cells in Sjögren's syndrome
Authors: de la Monte Suzanne M. , Hutchins Grover M. , Gupta Prabodh K.
Published: January 2005

Central nervous system complications in Sjögren's syndrome have been reported sporadically for year. We examined the nature and frequency of central nervous system abnormalities in 11 patients with clinically documented Sjögren's syndrome on whom postmortem examination was performed. In 9, characteristic mixed (polymorphous) inflammatory infiltrates containing large atypical mononuclear cells were observed in the leptomeninges, choroid plexus, or both; only 5 of the 9 had neurological symptoms, however. Among patients with central nervous system lesions, 3 had definite vasculocentric inflammation and 1 had a necrotizing vasculitis with extensive subarachnoid hemorrhage. Four patients had evidence of chronic subarachnoid microhemorrhage associated with polymorphous meningitis. Atypical mononuclear cells in a polymorphous inflammatory exudate were observed in antemortem cerebrospinal fluid cytological specimens from 2 of the patients. The findings suggest that central nervous system involvement in Sjögren's syndrome is common and that neurological symptoms are related to polymorphous meningitis and vasculitis. Detection of atypical mononuclear cells in cerebrospinal fluid specimens may be of diagnostic value.
Postmortem observations on β‐glucuronidase deficiency presenting as hydrops fetalis
Authors: Irani Dina , Kim Han‐Seob , El‐Hibri Huda , Dutton Robert V. , Beaudet Arthur , Armstrong Dawna
Published: January 2005

This study reports a case of type VII mucopolysaccharidosis (β‐glucuronidase deficiency) presenting as lethal hydrops fetalis. Skin fibroblast cultures established postmortem revealed deficient β‐glucuronidase activity. Mucopolysaccharides were stored in various cells of the brain, heart, kidney, liver, and spleen. The stages of maturation of the bones, kidneys, and brain were discrepant, the brain being the least mature organ. A delay in central nervous system maturation may account for psychomotor retardation in some patients with this enzyme deficiency.
Alzheimer's disease: Genetic aspects and associated clinical disorders
Authors: Heyman Albert , E William E. , Hurwitz Barrie J. , Schmechel Donald , Sigmon Alverta H. , Weinberg Tina , Helms Michael J. , Swift Michael
Published: January 2005

Genetic aspects and associated clinical disorders were studied in a consecutive series of 68 men and women in whom Alzheimer's disease appeared at or before age 70. Secondary cases of dementia were found in 17 (25%) of the families, affecting 22 of the probands' siblings and parents. The cumulative incidence of Alzheimer's disease in these relatives was approximately 14% at age 75. An increased frequency of Down's syndrome was observed among relatives of the probands: a rate of 3.6 per 1, 000, as compared with an expected rate of 1.3 per 1, 000. A history of thyroid disease was established in 9 (19.6%) of the 46 female probands, a frequency greater than that reported in the general population. There was no excess of hematological malignancies among the blood relatives, and parental age at the time of birth of the probands did not differ from the norm. The results of this study indicate that early‐onset Alzheimer's disease is associated with a genetic factor manifested in a substantial familial aggregation of dementia, a probable excess of Down's syndrome in the probands' relatives, and a possible association with thyroid dysfunction in women with this form of dementia.
Long‐term effects of treatment on endocrine function in children with brain tumors
Authors: Duffner Patricia K. , Cohen Michael E. , Anderson Stephen W. , Voorhess Mary L. , MacGillivray Margaret H. , Panahon Alvin , Brecher Martin L.
Published: January 2005

Fourteen children with brain tumors received endocrine evaluations at least one year following completion of cranial irradiation. Treatment consisted of operation (13 patients), craniospinal irradiation (6), whole brain irradiation (5), posterior fossa irradiation (3), and chemotherapy (10). Endocrine evaluation included bone age roentgenography and measurement of growth hormone (using sequential arginine and insulin stimulation), thyroxine, thyroid‐stimulating hormone, plasma cortisol, testosterone, prolactin, and urinary follicle‐stimulating hormone and luteinzing hormone. Ten of 12 children (83%) had abnormal responses to both tests of growth hormone stimulation. All growth hormonedeficient patients treated prior to puberty and tested at least 2 years following completion of cranial irradiation had decelerated linear growth. Results of thyroid function tests were abnormal in 4 patients: 2 patients had evidence of primary hypothyroidism, and 2 showed secondary or tertiary hypothyroidism. Two patients had inadequate cortisol responses to insulin hypoglycemia. Urinary follicle‐stimulating hormone and luteinizing hormone, serum prolactin, and serum testosterone levels were appropriate for age in all patients.
Ross river virus–induced demyelination: II. Ultrastructural studies
Authors: Seay Alan R. , Wolinsky Jerry S.
Published: January 2005

Focal central nervous system demyelination is a prominent feature of Ross River virus encephalitis in mice. The present ultrastructural study shows that oligodendrocytes are a primary site of viral replication. The earliest myelin disruption occurs in association with an inflammatory infiltrate composed primarily of polymorphonuclear leukocytes, which are later replaced by macrophages. Viral particles are found in oligodendrocytes, selected neuronal populations, macrophages, and polymorphonuclear leukocytes through the end of the first week of infection as macrophages remove myelin from normal‐appearing axons. Between the second and third weeks of infection, axons within foci of demyelination partially remyelinate with central myelin. Schwann cells are not found within regions of central remyelination. Cyclophosphamide treatment does not prevent or delay demyelination or remyelination. Results of this and previous studies strongly suggest that Ross River virus‐induced demyelination is not immune mediated but rather the direct result of viral infection of oligodendrocytes.
Regulation of regional cerebral blood flow during and between migraine attacks
Authors: Lauritzen Martin , Olsen Tom Skyhoj , Lassen Niels A. , Paulson Olaf B.
Published: January 2005

Cerebrovascular reactivity to voluntary hyperventilation, moderate hypertension, and physiological activation was studied in nine patients during induced migraine attacks and in four patients between their attacks. Regional cerebral blood flow was measured by the xenon 133 injection technique in 254 areas of one hemisphere. The partly hypoperfused hemisphere allowed for comparison of adjacent hypoperfused and normally perfused brain areas. During attacks the carbon dioxide reactivity was decreased to 2.8 ± 0.8% per mm Hg in the oligemic regions compared with 5.8 ± 0.8% per mm Hg in the normally perfused brain. Blood pressure autoregulation was normal in all brain regions. Regional blood flow increase in response to physiological activation was severely impaired in the hypoperfused brain areas, whereas neighboring normally perfused regions reacted normally. Confinement of the regulation abnormalities to the area of the oligemia supports our suggestion that the blood flow changes are caused by a change in local metabolism. Between attacks of migraine, the patients had normal regulation of brain circulation.
Resumption of behavior following intracarotid sodium amobarbital injection
Authors: Rausch Rebecca , Fedio Paul , Ary Catherine M. , Engel Jerome , Crandall Paul H.
Published: January 2005

Recovery rates of selected behaviour following intracartid sodium amobarbital injection were examined in 17 patients with unilateral focal temporal lobe epilepsy. Left hemisphere injections resulted in more prolonged disruption of naming (p < 0.00001), reading (p < 0.0001), matching‐to‐sample behaviours (p < 0.05), and short‐term recognition memory for pictures (p < 0.0001) and words (p < 0.0003) than did right hemisphere injections. In addition, recovery of behaviours was prolonged if the injection was administered to the hemisphere contralateral to that with the primary seizure focus (p < 0.005). A trend toward a similar pattern was seen in the postinjection electroencephalogram with the maximum slowing found in the injected hemisphere prolonged if the epileptic focus was in the contralateral hemisphere (p = 0.06). These findings suggest a negative effect of an epileptic lesion on the contralateral hemisphere.
Hyperekplexia: A syndrome of pathological startle responeses
Authors: Sáenz‐Lope E. , Herranz‐Tanarro F. J. , Masdeu J. C. , Peña J. R. Chacón
Published: January 2005

We describe a family and three sporadic cases of startle disease, or hyperekplexia. Sudden unexpected noises caused the patients to fall rigidly, often injuring themselves but retaining consciousness. This unusual entity differs from startle epilepsy and cataplexy. Clonazepam proved ineffective in three patients. Valproic acid, 5‐hydroxytryptophan, or piracetam markedly reduced the abnormal startle in three patients.
Neurological disorders associated with deficiency of glutamate dehydrogenase
Authors: Plaitakis Andreas , Berl Soll , Yahr Melvin D.
Published: January 2005

Glutamate dehydrogenase (GDH) activity was measured in leukocytes from 88 patients with various types of degenerative neurological disorders affecting primarily the cerebellum and/or the basal ganglia, and 26 healthy control subjects. Twelve patients with slowly progressive multiple‐system atrophic disorders were found to have a partial deficiency of this enzyme (52% of control level). The majority of these patients evidenced a constellation of neurological findings consistent with the diagnosis of olivopontocerebellar atrophy, although others were atypical in their neurological manifestations. Thus, GDH‐deficient patients were encountered with predominantly extrapyramidal manifestations (atypical Parkinson's disease), cerebellar dysfunction with peripheral neuropathy, or anterior horn cell signs, suggesting that a pleomorphic phenotypic expression of the enzymatic deficiency may occur. Seven cases of GDH deficiency were familial and 5 were sporadic. The former patient group consisted of siblings of either sex, but no parents or offspring were affected. The genetic pattern of the disorder is compatible with autosomal recessive inheritance. Patients with dominantly inherited olivopontocerebellar atrophy or other types of cerebellar or basal ganglia degenerative neurological disorders showed normal GDH activity. Leukocyte GDH was fractionated into “particulate‐heat labile” and “soluble‐heat stable” components. In the patients the decrease in activity was limited to the “particulate‐heat labile” component. A genetic mutation of a GDH “isoenzyme” may occur in some patients with multiple‐system degeneration.
Whole body insulin resistance in myotonic dystrophy
Authors: Moxley Richard T. , Corbett Alastair J. , Minaker Kenneth L. , Rowe John W.
Published: January 2005

To quantitate the degree of whole body insulin resistance in patients with myotonic dystrophy, three separate euglycemic insulin infusions were given to ambulatory patients and the results compared with findings in normal control subjects. Basal glucose and insulin values were similar for the two groups. There was no significant difference in insulin clearance rates between normal subjects and patients at the three insulin infusion rates used. A highly significant decrease in the whole body glucose disposal rate was seen during the 120‐minute insulin infusion in the patients with myotonic dystrophy compared with normal subjects at all three insulin dosages (20 mU/m2/min: 2.18 ± 0.29 [standard error] versus 5.49 ± 1.72 mg/kg/min, p < 0.0001; 80 mU/m2/min: 4.16 ± 0.34 versus 8.49 ± 0.45 mg/kg/min, p < 0.0001; 200 mU/m2/min: 5.22 ± 0.53 versus 10.06 ± 0.50 mg/kg/min, p < 0.0001). These marked decreases in glucose disposal rates for the patients were adjusted in accordance with their 24‐hour urinary creatinine excretion rate to correct for the difference in muscle mass between patients and controls. This adjusted glucose disposal rate was 15 to 25% lower (p < 0.02) in the patients with myotonic dystrophy at insulin infusion rates of 20 and 80 mU. During the 200 mU insulin infusions, the adjusted glucose disposal rate remained lower than that in normal subjects but was of borderline statistical significance. These studies suggest that moderately severe whole body insulin resistance is responsible for the postprandial hyperinsulinemia typically seen in patients with myotonic dystrophy.
Amyloid plaques in the brains of mice with Creutzfeldt–Jakob disease
Authors: Tateishi Jun , Nagara Hitoshi , Hikita Kohtaro , Sato Yuji
Published: January 2005

By intracerebral inoculation with the brain homogenates from 9 patients with Creutzfeldt‐Jakob disease, amyloid plaques were induced in mouse brains after incubation periods of 403 to 835 days. The plaques existed mainly in the cerebral white matter beneath the lateral ventricle walls amd were more numerous in the hemisphere where injection was made. Morpholoogical findings of the plaques were almost identical to those seen in patients wtih kuru and Creutzfeldt‐Jakob disease, and in animals with scrapie. They were also similiar to the cores of senile plaques seen in patients with alheimer's disease.
IgM Demyelinative neuropathy with amyloidosis and biclonal gammopathy
Authors: Julien J. , Vital C. , Vallat J. M. , Lagueny A. , Ferrer X. , Deminiere C. , Leboutet M. J. , Effroy C.
Published: January 2005

A 59‐year‐old man developed a sensorimotor neuropathy of the upper and lower limbs, associated with a biclonal gammopathy, within the space of a few months. Each of two paraproteins was coupled with a distinct IgM kappa IgG lambda light chain. Examination of a nerve biopsy specimen by electron microscopy revealed a demyelinative process with a widening of the interlaminar space in the myelin sheath, as well as deposits of amyloid substance between nerve fibers. Direct immunofluorescence revealed the presence of IgM and of the kappa light chain in certain Schwann cells, while the lambda IgG was fixed to the amyloid deposits. Immunoperosidase histochemistry showed a positive reaction in normal human nerve tissue to the immune serum IgM and kappa light chain. The findings suggest that the widening of the interlaminar space of the diseased myelin corresponds to an active fixation of immunoglobulin on the sheath of the Schwann cell. The presence of two light chains in this patient's gammopathy caused a dual pathology: the kappa chain, a demyelinative neuropathy, and the lambda chain, a primary amyloidosis, with deposits in the peripheral nerve and in the kidney.
Contralateral cerebellar hypometabolism following cerebral insult: A positron emission tomographic study
Authors: Kushner Michael , Alavi Abass , Reivich Martin , Dann Robert , Burke Allan , Robinson Gerald
Published: January 2005

Positron emission tomographic studies of 16 patients with cerebral ischemia and brain tumor showed asymmetries in cerebellar metabolism not encountered in 14 normal control subjects. An asymmetry was present in 62.5% of cases. The lower metabolic rate occurred in the cerebellar hemisphere contralateral to the cerebral lesion (p <0.001; sign test). In all cases computed tomography showed the supratentorial lesion to be unilateral and the posterior fossa contents to be unaffected. The presence of depressed cerebellar metabolism was highly associated with involvement of the contralateral parietal lobe (p <0.02; phi coefficient). The presence of a cerebellar abnormality was not related to the presence of any particular sign. Serial studies showed normalization of cerebellar metabolism over time. It is likely that this effect is a result of interruption of the functional interconnections between the cerebrum and the cerebellum.
Eye movements induced by head rotation in unresponsive patients
Authors: Leigh R. John , Hanley Daniel F. , Munschauer Frederick E. , Lasker Adrian G.
Published: January 2005

Eye movements induced by head rotation were studied in 6 patients in acute coma, 4 patients in a persistent vegetative state, and 6 healthy, alert control subjects. Results from control subjects suggest that the oculocephalic response in the supine position is principally a vestibulo‐ocular reflex. A position‐step rotation of the head produced an initial oppositely directed eye movement, followed by a drift of the eyes back toward midline with a negative exponential time course. The time constant of this drift was greater than or equal to 10 seconds in control subjects but less than or equal to 1.5 seconds in unconscious patients and less than or equal to 0.5 seconds in vegetative patients. The rapid drift back of the eyes in unresponsive patients implies dysfunction of reticular and, possibly, cerebellar connections; the rate of this drift may indicate the severity and extent of brain injury. Sinusoidal head rotation produced slow and quick phases of nystagmus in normal subjects. Quick phases were absent in patients in acute coma; although present in vegetative patients, the quick phases did not keep the eyes close to primary position, as was the case in control subjects.
Quantitation of the sweating deficiency in diabetes mellitus
Authors: Kennedy William R. , Sakuta Manabu , Sutherland David , Goetz Frederick C.
Published: January 2005

This report introduces quantitative tests for the evaluation of sweating and the results obtained in 81 diabetic and 30 control subjects. The tests rely on the ability of pilocarpine, introduced into the skin by iontophoresis, to stimulate sweating from fully or partially innervated sweat glands but not from denervated glands. Many diabetic patients had a reduced number of excitable sweat glands and a low volume of sweat per square centimeter of skin. The results of the sweat tests correlated best with the clinically determined perception of pain from pinprick. The similar degree of involvement of sudomotor axons and pain‐conveying axons may be related to the known similarity in size and reinnervation patterns. There was poor correlation of the sweating deficiency with alpha motor conduction velocity and with denervation of foot muscles as determined by the evoked muscle action potential. The number of excitable sweat glands was usually normal if the muscle action potential was above 0.5 mv, and often normal even when a muscle action potential was unobtainable. Every diabetic patient with abnormal sweating and several with normal sweating had reduced heart rate fluctuation during a standard Valsalva maneuver or during slow respiration.
Molecular genetics, recombinant DNA techniques, and genetic neurological disease
Authors: Rosenberg Roger N.
Published: January 2005

The molecular defects responsible for Huntington's disease, the spinocerebellar degenerations, myotonic muscular dystrophy, neurofibromatosis, and tuberous sclerosis, among other major dominant inherited diseases of the nervous system, will be identified using the new techniques of molecular genetics. With synthesized nucleic acid segments complementary to portions of the patient's DNA, known as complementary DNA probes, it will be possible to identify and isolate the mutant gene responsible for a particular disease. These events are referred to as gene cloning. In addition, complex genetic regulatory mechanisms involved in cell differentiation during neuroembryogenesis will be elucidated with the application of these strategies. It is important for the clinician to become familiar with the precision and potential of these new methodologies, because they will soon influence significantly the practice of neurology.
Diagnostic imaging of herpes simplex virus encephalitis using a radiolabeled antiviral drug: Autoradiographic assessment in an animal model
Authors: Saito Yutaka , Rubenstein Richard , Price Richard W. , Fox Jack J. , Watanabe Kyoichi A.
Published: January 2005

To develop a new approach to the diagnosis of herpes simplex encephalitis, we used a radiolabeled antiviral drug, 2′‐fluoro‐5‐methyl‐1‐β‐D‐arabinosyluracil labeled with carbon 14 ([14C]FMAU), as a probe for selectively imaging brain infection in a rat model by quantitative autoradiography. A high correlation was found between focal infection, as defined by immunoperoxidase viral antigen staining, and increased regional [14C]FMAU uptake in brain sections. Two potential sources of false‐positive imaging were defined: high concentrations of drug in the choroid plexus because of its higher permeability compared with brain, and drug sequestration by proliferating uninfected cell populations. Our results support the soundness of the proposed strategy of using a labeled antiviral drug that is selectively phosphorylated by herpes simplex virus type 1 thymidine kinase in conjunction with scanning methods for human diagnosis, and also define some of the factors that must be taken into account when planning clinical application.
Paraproteins in the spinal fluid of patients with paraproteinemic polyneuropathies
Authors: Dalakas Marinos C. , Papadopoulos Nicholas M.
Published: January 2005

High‐resolution agarose gel electrophoresis, combined with immunofixation electrophoresis, was used to detect and identify immunoglobulins in the cerebrospinal fluid of six patients with paraproteinemic polyneuropathy. In four patients with serum IgMk monoclonal protein, we found a discrete band in the cerebrospinal fluid identified also as IgMk; one patient with serum IgGk had an IgGk cerebrospinal fluid band, and one patient with serum IgAk had an IgAk monoclonal band in the cerebrospinal fluid. The permeability of the blood–cerebrospinal fluid barrier was increased 3 to 10 times in all these patients. The findings indicate that in patients with paraproteinemic polyneuropathy, the increased permeability of the blood–cerebrospinal fluid barrier results in influx of serum proteins into the cerebrospinal fluid, including high‐molecular‐weight IgM. Because monoclonal IgM, unlike monoclonal IgG and IgA, is not found in the cerebrospinal fluid of neurologically intact patients, its presence in the cerebrospinal fluid of patients with paraproteinemias should lead the physician to suspect neurological involvement and can be of diagnostic value.
Computed tomography in Alexander's disease
Authors: Farrell Kevin , Chuang Sylvester , Becker Laurence E.
Published: January 2005

Computed tomography demonstrated contrast‐enhancing lesions in the periventricular frontal regions, caudate nuclei, and thalami in an infant with Alexander's disease. The distribution of the enhancing lesions corresponded to the areas in which Rosenthal fibers were most prominent. These radiological findings have not been described in other white matter diseases; thus, they may help to distinguish Alexander's disease from Canavan's disease and decrease the necessity for diagnostic brain biopsy.
The biological behavior of primary cerebral neuroblastoma: A reappraisal of the clincal course in a series of 70 cases
Authors: Bennett James P. , Rubinstein Lucien J.
Published: January 2005

The outcome in 70 patients with primary cerebral neuroblastoma was evaluated in light of their age incidence, histological subtypes, and modes of treatment. In more than 80% of the patients, primary cerebral neuroblastoma occurred in the first decade, most often before the age 2 years (26%). Over half of the tumors were histologically of the classic variant, the remainder showing varying degrees of mesenchymal stromal response (desmoplastic or transitional variants). Patients with a tumor of the desmoplastic or transitional variant without ganglionic differentiation had a lower mean age at the time of diagnosis than those harboring a tumor of the classic variant. Age could not be correlated with the presence of ganglionic differentiation.

The overall 3‐year survival rate was 60%. At least 30% of patients have a 5‐year postoperative survival. Elight patients survivied 7 years after diagnosis, and 3 for more than 10 years. The period of risk for tumor recurrence or death was a function of the initial age at diagnsis (Collin's law). Most deaths or fatal recurrences occurred within the first 3 years after operation. No corelation could be made between survival times and histological variant of the tumor or the presence of ganglionic differentiation.

Our data do not indicate that, overall, postoperative irradiation with or without chemotherapy improved length of survival, but an occasional favorable clinical response to chemotherapy did occur. Because of the high incidence of local recurrence and the relative frequency of cerebrospinal metastasis, prophylactic whole brain and spinal irradiation is probably justified.
Activity of multiple sclerosis during pregnancy and puerperium
Authors: Korn‐Lubetzki Isabelle , Kahana Esther , Cooper Gerald , Abramsky Oded
Published: January 2005

The influence of pregnancy on multiple sclerosis was studied in 338 women by determining in each trimester of pregnancy and post partum the number of relapses and the corresponding relapse rate. Eighty‐five relapses occurred in association with 199 pregnancies, most (65) in the postpartum period, and a low number of relapses (2) were recorded in the last trimester of pregnancy. Comparing the average exacerbation rate of the study group with that of patients with multiple sclerosis in Israel (0.28 relapses per person per year), we found a statistically significant decrease in the third trimester (0.04) and a high increase in the first three months post partum (0.82). This pattern of remissions at the end of pregnancy and exacerbations post partum is similar to that observed in other putative autoimmune diseases.
Effect of vasodilating drugs on intracortical and extracortical vascular resistance following middle cerebral artery occlusion in cats
Authors: Date H. , Hossmann K.‐A.
Published: January 2005

In 24 lightly anesthetized cats acute cerebral infarction was produced by transorbital occlusion of the left middle cerebral artery. Pial artery pressure and blood flow were measured in the center of the middle cerebral artery territory, and changes of segmental resistance were determined upstream and downstream of the pial arterial recording site. After middle cerebral artery occlusion, pial arterial pressure fell from 59 to 13 mm Hg, and blood flow from 67.4 to 19.6 ml/100 gm/min. Downstream (intracortical) vascular resistance slightly decreased from 0.93 to 0.72 mm Hg · ml−1. 100 gm · minute, and upstream (extracortical) resistance rose form 0.89 to 6.18 mm Hg · ml−1. 100 gm · min. Before middle cerebral artery occlusion, intracarotid infusion of papaverine (625 μg/kg/min) and prostacyclin (500 ng/kg/min) caused a reduction of downstream resistance, and intracarotid infusion of nimodipine (670 ng/kg/min) reduced both downstream and upstream resistance. After vascular occlusion all three drugs reduced mainly upstream resistance but cerebral blood flow did not significantly improve because blood pressure also fell. Intracerebral steal phenomena were never observed.
Value of ultrasound for identification of acute hemorrhagic necrosis of thalamus and basal ganglia in an asphyxiated term infant
Authors: Kreusser Katherine L. , Schmidt Robert E. , Shackelford Gray D. , Volpe Joseph J.
Published: January 2005

An infant with profound intrapartum and postpartum asphyxia demonsstrated striking bilateral echodensities in the region of the thalamus and basal ganglia on ultrasound scan. Neuropathological examination confirmed the presence of symmetrical areas of hemorrhagic necrosis involving the thalamus, caudate, putamen, and globus pallidus. The findings demonstrate the value of ultrasonography in the identification of this variety of perinatal hypoxic‐ischemic injury.
Adrenoleukodystrophy: Survey of 303 cases: Biochemistry, diagnosis, and therapy
Authors: Moser Hugo W. , Moser Ann E. , Singh Inderjit , O'Neill P.
Published: January 2005

Adrenoleukodystrophy (ALD) is a genetically determined disorder associated with progressive central demyelination and adrenal cortical insufficiency. All affected persons show increased levels of saturated unbranched very‐long‐chain fatty acids, particularly hexacosanoate (C26:0), because of impaired capacity to degrade these acids. This degradation normally takes place in a subcellular organelle called the peroxisome, and ALD, together with Zellweger's cerebrohepatorenal syndrome, is now considered to belong to the newly formed category of peroxisomal disorders. Biochemical assays permit prenatal diagnosis, as well as identification of most heterozygotes. We have identified 303 patients with ALD in 217 kindreds. These patients show a wide phenotypic variation. Sixty percent of patients had childhood ALD and 17% adrenomyeloneuropathy, both of which are X‐linked, with the gene mapped to Xq28. Neonatal ALD, a distinct entity with autosomal recessive inheritance and points of resemblance to Zellweger's syndrome, accounted for 7% of the cases. Although excess C26:0 in the brain of patients with ALD is partially of dietary origin, dietary C26:0 restriction did not produce clear benefit. Bone marrow transplant lowered the plasma C26:0 level but failed to arrest neurological progression.
The frequency of multiple sclerosis in Italy: A descriptive study in Ferrara
Authors: Granieri Enrico , Tola Rosaria , Paolino Ezio , Rosati Giulio , Carreras Mirko , Monetti V. Cinzia
Published: January 2005

Result of intensive prevalence surveys on multiple sclerosis carried out in different small regions of Italy have suggested that this country falls into the high‐frequency zone for the disease. To verify this hypotiesis by studying a large population, we conducted an intensive incidence and prevalence survey in the province of Ferrara, northern Italy (mean population, 386, 000). Based on 128 patients, the mean incidence per year for the period 1965 through 1979 was 2.2 cases per 100, 000. On October 24, 1981, the prevalence rate was 46.1 cases per 100, 000. The results support the view that northern Italy is a high‐risk area for multiple sclerosis.
Evolution of computed tomographic abnormalities in leptomeningeal metastases
Authors: Jaeckle Kurt A. , Krol George , Posner Jerome B.
Published: January 2005

We obtained serial cranial computed tomography (CT) scans from 25 patients with documented leptomeningeal metastases in orders to correlate the course of clinical symptoms and cerebrospinal fluid abnormalities with the evolution of CT abnormalities. In almost one‐third of patients, relatively specific CT finding such as enhancement of the meninges, obliteration of cisterns or sulci, and hydrocephalus with transependymal edema preceded clinical symptoms. By the time patients became symptomatic, 10 of 25 (40%) had specific abnormalities, and 4 more became specifically abnormally with time. The first scan after cerebrospinal fluid tumor cells were discovered showed specific abnormalities in only 6 patient, but 7 more became specifically abnormal with time. Ventriculomegaly occurred in 18 of 25 patients (72%) and was associated with sulcal and/or cisternal obliteration in one‐half of the patients. In 4 patients sulcal and cisternal obliteration developed prior to ventricular enlargement. Multiple small, enhancing cortical nodules were detected prior to symptom onset and discovery of leptomeningeal tumor in 5 patients.
Dermatomal somatosensory evoked potentials unilateral lumbosacral radiculopathy
Authors: Aminoff Michael J. , Goodin Douglas S. , Barbaro Nicholas M. , Weinstein Philip R. , Rosenblum Mark L.
Published: January 2005

We examined scalp‐recorded somatosensory evoked potentials (SSEPs) to electrical stimulation of the peroneal nerves and to stimulation in the L5 and S1 dermatomes in 19 patients with unilateral radiculopathies involving these segments. For the dermatomal studies at least two trials of 512 responses were recorded from the vertex with reference to both the midfrontal and contralateral parietal electrodes, using an averaging technique. Findings on the symptomatic and asymptomatic sides were compared in each patient. We found that peroneal SSEPs were normal in all patients. Dermatomal SSEPs correctly identified the lesion in 5 patients. In 1 patient dermatomal SSEPs lateralized the lesion correctly but localized it to the adjacent root. In 10 cases dermatomol SSEPs gave misleading information, indicating an abnormality on the asymptomatic side in 1 patient and no abnormality in 9. In the remaining 3 patients, both SSEPs and radiological contrast studies failed to identify any lesion, although the radiculopathy was confirmed by electromyography. These findings raise doubt about the ultimate utility of these evoked potential techniques in the evaluation of patients with suspected radiculopathies.