bluuebunny/crossref_metadata_2025_split huggingface dataset:
Peripheral nerve conduction in fabry disease
Authors: Sheth Kumudchandra J. , Swick Herbert M. 
Published: January 2005

AbstractPeripheral nerve conduction was studied in 34 members, aged 3 to 54 years, from three separate kindreds with Fabry disease. Nerve conduction velocity was prolonged in one‐third (11 of 34) of the study group. Of the 9 control family members with normal plasma α‐galactosidase levels, 8 had normal velocity. A 6‐year‐old girl with normal plasma and leukocyte α‐galactosidase levels and absent corneal deposits had slightly lower than normal nerve conduction velocity. One of the 3 obligate female carriers and 1 of 10 suspected carriers of Fabry disease had prolonged conduction velocity but normal distal latency. On the other hand, 8 of 12 males affected with Fabry disease demonstrated slow nerve conduction velocity while 4 of the 8 had prolonged distal latency. Frequently, only one of the two nerves studied showed abnormalities. These abnormalities were not related to patient age. Interfamilial variations in the abnormalities were present.
Prevention of experimental allergic encephalomyelitis in lewis rats with peptide 68‐88 of guinea pig myelin basic protein
Authors: Chou Frank C.‐H. , Chou C.‐H. Jen , Fritz Robert B. , Kibler Robert F. 
Published: January 2005

AbstractThe highly encephalitogenic guinea pig peptide 68‐88 has been used to develop an effective and reproducible model of protection in the Lewis rat. Doses as low as 0.1 nmol of peptide protected 70% of rats when injected intraperitoneally six and four weeks prior to challenge with 0.05 nmol of the peptide in complete Freund's adjuvant. Fragments derived from guinea pig peptide 68‐88 by selective enzyme cleavage were then tested for their capacity to provide protection in this model system. These fragments had previously been well characterized both biochemically and immunologically.The protection provided by each fragment closely paralleled its capacity to induce disease. This suggests that the region of peptide 68‐88 required for protection is similar to that needed for induction of experimental allergic encephalomyelitis and the other T‐cell functions of the peptide. B‐cells did not appear to participate; peptide 68‐85, which has no capacity to produce antibody against peptide 68‐88, gave full protection, while peptide 79‐88, which contains the major B‐cell determinant of the peptide, afforded no protection. Rat peptide 68‐88 did not protect against challenge with the guinea pig peptide, demonstrating a critical role for serine 79. These studies support the concept that nonencephlaitogenic agents do not protect against experimental allergic encephalomyelitis at doses comparable to those of encephalitogenic agents.
Surgically correctable lesions solely detected by CT scan in adult‐onset chronic epilepsy
Authors: Jabbari Bahman , Huott Archer D. , Chiro Giovani Di , Martins Albert N. , Youngblood Lloyd A. , Harper Michael G. 
Published: January 2005

AbstractA prospective study of 162 patients with adult‐onset chronic epilepsy revealed 4 in whom computed tomographic scanning was the only diagnostic test that detected the presence of a surgically correctable cerebral lesion. These patients had suffered from seizures for 1 to 14 years and at the time of operation demonstrated no clinical neurological deficits except organic mental changes.
Fetal methylmercury poisoning: Clinical and toxicological data on 29 cases
Authors: Marsh David O. , Myers Gary J. , Clarkson Thomas W. , Amin‐Zaki Laman , Tikriti Sadoun , Majeed Muhammed A. 
Published: January 2005

AbstractThis report describes psychomotor retardation in infants caused by prenatal exposure to methylmercury. A study of 29 mother‐infant pairs established a relationship between maximum maternal hair mercury concentration during pregnancy and the frequency of neurological effects in the infants. The latter included delayed achievement of developmental milestones with or without neurological signs. The infants were 4 1/2 to 5 years of age on last examination. Ten infants of mothers who had maximum hair concentrations in the range of 99 to 384 parts per million (ppm) had a significantly higher frequency of abnormal findings than those in two groups having lower maternal hair mercury concentrations (12 to 85 and 0 to 11 ppm). The group sizes were too small to allow identification of a specific ‘threshold’ maternal hair concentration above which such effects can be expected. Transient paresthesias during pregnancy occurred in 80% of the mothers in the higher concentration group (99 to 384 ppm) as compared with 30% and 22% in the lower groups. Neurological abnormalities were found in some children whose mothers had been asymptomatic during pregnancy.
Diagnosis of infantile neuroaxonal dystrophy by skin biopsy
Authors: Wisniewski Krystyna , Wisniewski Henry M. 
Published: January 2005

AbstractSkin biopsy with ultrastructural examination of cutaneous nerves showed dystrophic axons (spheroids), confirming a diagnosis of infantile neuroaxonal dystrophy. Independent of axonal changes, a few endoneurial and Schwann cells showed cytoplasmic inclusion bodies composed of structures similar to those seen in the spheroids.
Peripheral neuropathy in the cherry‐red spot—myoclonus syndrome (Sialidosis type I)
Authors: Steinman L. , Tharp B. R. , Dorfman L. J. , Forno L. S. , Sogg R. L. , Kelts K. A. , O'Brien J. S. 
Published: January 2005

AbstractA patient with the cherry‐red spot‐myoclonus syndrome associated with autosomal recessive deficiency of lysosomal neuraminidase was found to have clinical, electrophysiological, and morphological evidence of a sensorimotor peripheral polyneuropathy with distinctive inclusions in the cytoplasm of Schwann cells.
Pathological verification of ischemic score in differentiation of dementias
Authors: Rosen Wilma G. , Terry Robert D. , Fuld Paula A. , Katzman Robert , Peck Arthur 
Published: January 2005

AbstractFourteen case histories of persons who had a histological diagnosis of either senile dementia of the Alzheimer type, multiinfarct dementia, or a mixed dementia composed of these two types and who showed evidence of a moderate to severe dementia on psychological testing were rated for the presence of thirteen clinical features comprising Hachinski's Ischemic Score. These features are frequently considered primarily characteristic of vascular dementia. Persons with senile dementia of the Alzheimer type were clearly differentiable from persons with multiinfarct dementia and mixed dementia, while the latter two groups were indistinguishable from one another. In our sample, eight features were found to characterize those persons with vascular dementia. These data verify the usefulness of the Ischemic Score in differentiating between senile dementia of the Alzheimer type and vascular dementia.
Slowed nerve conduction with wrist flexion in carpal tunnel syndrome
Authors: Schwartz M. S. , Gordon J. A. , Swash M. 
Published: January 2005

AbstractPhalen's wrist flexion test may reproduce the sensory symptoms of carpal tunnel syndrome (CTS). We have used this test in conjunction with motor and sensory latency studies in 20 patients with symptomatic CTS in one or both hands. The test caused an increase in the motor or sensory latency in half the patients. In 2 patients the latencies were abnormal only after wrist flexion. The test may thus be useful in diagnosis of borderline cases.
Lobar cerebral hemorrhages: Acute clinical syndromes in 26 cases
Authors: Ropper Allan H. , Davis Kenneth R. 
Published: January 2005

AbstractThe acute syndromes and CT findings are described in 26 cases of spontaneous cerebral hemorrhage. Occipital hemorrhage (11 cases) caused severe pain around the ipsilateral eye and dense hemianopia. Left temporal hemorrhage (7 cases) began with mild pain in or just anterior to the ear, fluent dysphasia with poor auditory comprehension but relatively good repetition, and a visual deficit subtending less than a hemianopia. Frontal hemorrhage (4 cases) caused a distinctive syndrome beginning with severe contralateral arm weakness, minimal leg and face weakness, and frontal headache. Parietal hemorrhage (3 cases) began with anterior temporal (“temple”) headache and hemisensory deficit, sometimes involving the trunk to the midline. One patient had a right temporal hemorrhage.Spontaneous lobar hemorrhage and branch artery embolism in the same region produce similar clinical syndromes. Headache is a first and prominent symptom. A rapid but not instantaneous onset over several minutes, when combined with one of the typical syndromes, suggests lobar hemorrhage rather than other types of stroke.Ancillary investigations (including CT scanning, angiography in 11 patients, and autopsy in 4) disclosed 2 patients with bleeding diatheses due to warfarin, 2 with arteriovenous malformations, and 1 with metastatic tumor. Only 8 of the 26 patients had chronic hypertension (blood pressure greater than 130/85 mm Hg), suggesting that hypertension is not an etiological factor in most lobar hemorrhages.
Oligoclonal IgG and free light chains in multiple sclerosis demonstrated by thin‐layer polyacrylamide gel isoelectric focusing and immunofixation
Authors: Laurenzi Maria Assunta , Mavra Milica , Kam‐Hansen Slavenka , Link Hans 
Published: January 2005

AbstractA modified technique of isoelectric focusing on thin‐layer polyacrylamide gel followed by immunofixation with monospecific antisera was used to identify individual cerebrospinal fluid (CSF) and serum proteins and to define the oligoclonal reaction observed in multiple sclerosis (MS). “Normal” IgG gave about 20 to 30 bands at pH 3.5 to 9.5, IgA about 10 bands at pH 3.5 to 6.4, β‐trace protein a smear at pH 3.5 to 8.5, and γ‐trace protein 1 or 2 bands at pH 8.0, 9.5, or both.Up to 11 oligoclonal IgG bands migrating between pH 6.5 and 9.5 were found in CSF from 26 of 27 consecutive patients with MS and also in 20 of the corresponding sera, although at lower numbers and concentrations. In 26 patients, 1 or more of the bands corresponding to normal polyclonal IgG were stronger in CSF than in serum. These data support the hypothesis that two colonies of lymphocytes are activated intrathecally, one of them synthesizing oligoclonal and the other polyclonal IgG. Up to 11 mostly faint bands of free light chains, predominantly of λ type and migrating between pH 3.5 and 9.5, were found in 8 of 9 CSF specimens from patients with MS.
Skin and conjunctival nerve biopsies in adrenoleukodystrophy and its variants
Authors: Martin J. J. , Ceuterick C. , Libert J. 
Published: January 2005

AbstractThis study demonstrates that skin or conjunctival biopsies are capable of diagnosing adrenoleukodystrophy and its variants. Electron microscopy of cutaneous nerve twigs in eleven patients showed characteristic curved clefts and leaflets in Schwann cells surrounding myelinated axons. In addition, three conjunctival biopsies were done, two of which were positive. The two peripheral nerve trunk biopsies performed also showed characteristic Schwann cell changes.
Infantile neuronal degeneration masquerading as werdnig‐hoffmann disease
Authors: Steiman Gerald S. , Rorke Lucy Balian , Brown Mark J. 
Published: January 2005

AbstractWe investigated two infants whose clinical illness resembled Werdnig‐Hoffmann disease (WHD). Motor nerve conduction velocities in one infant were markedly decreased, suggesting a demyelinative neuropathy. Pathological examination of the central nervous system in both patients revealed the expected loss of cell bodies in the anterior horn and motor cranial nerves. In addition, there was widespread neuron loss and gliosis in both gray and white matter of the thalamus, cerebellum, pons, and spinal cord. Motor, sensory, and mixed peripheral nerves of one infant showed evidence of segmental demyelination with only mild fiber loss. These and similar patients previously described have a disorder that should be separated from WHD and can be termed infantile neuronal degeneration.
Locked‐in syndrome with recovery
Authors: Khurana Ramesh K. , Genut A. A. , Yannakakis George D. 
Published: January 2005

AbstractWe report three patients who recovered from “locked‐in” syndrome of presumed vascular origin. One patient narrated her experience of the “locked‐in” phase and its termination. Another demonstrated palatal myoclonus and mirror movements upon recovery. These patients deserve considerate handling and vigorous medical support.
Treatment of intractable temporal lobe‐limbic epilepsy (complex partial seizures) by temporal lobectomy
Authors: Glaser Gilbert H. 
Published: January 2005

AbstractThe results of lobectomy in carefully selected patients with intractable epilepsy now are realized as worth the risk, for complete seizure relief can be achieved in up to 50% of such cases, and moderate to good seizure control in at least an additional 25%, with reduction in the drug requirement. Along with seizure relief often comes improvement in behavior and in social and personality development and life adjustments. Temporal lobectomy for treatment of temporal lobe‐limbic epilepsy therefore also has preventive value, especially if performed as early as the criteria indicate in appropriately selected patients. The resected tissue allows careful morphological studies that can enhance our knowledge of the pathogenesis of this disorder.
Sustained limbic seizures induced by intraamygdaloid kainic acid in the baboon: Symptomatology and neuropathological consequences
Authors: Menini Christian , Meldrum Brian S. , Riche Danielle , Silva‐Comte Carmen , Stutzmann Jean Marie 
Published: January 2005

AbstractIn Papio papio baboons chronically prepared for cortical and deep electroencephalographic recording, injection of kainic acid into the amygdala (7 animals) or temporal pole (2 animals) gave rise to focal epileptic discharges lasting 15 to 150 hours. Electrographically, the seizure activity spread ipsilaterally and contralaterally within the limbic system but did not become generalized. The principal associated motor signs were arrest of movement and oral automatisms. Histological examination after two to ten days demonstrated lesions (neuron loss and gliosis) at the injection site that varied according to the amount of kainic acid injected (2 to 68 μg). “Remote” lesions occurred in the ipsilateral hippocampus (end‐folium and Sommer sector) and neocortex (occipital and frontal regions). The hippocampal lesions were comparable to those previously described as consequent to status epilepticus.
Tolosa‐hunt syndrome: A case with associated facial nerve palsy
Authors: Swerdlow Barry 
Published: January 2005

AbstractA 54‐year‐old man developed a painful right third nerve palsy with signs of involvement of the first two divisions of the trigeminal nerve and, later, ipsilateral peripheral facial nerve weakness. A cavernous sinus venogram demonstrated narrowing of the third portion of the right ophthalmic vein and poor filling of the adjacent cavernous sinus. Resolution occurred with steroid therapy. The Tolosa‐Hunt syndrome may be a variant of a larger syndrome of recurrent multiple cranial polyneuropathies without identifiable cause.
Kernicterus and central pontine myelinolysis in a 14‐year‐old boy with fulminating viral hepatitis
Authors: Ho Khang‐ChengHo , Hodach Richard , Varma Rajiv , Thorsteinson Vigfus , Hess Timothy , Dale David 
Published: January 2005

AbstractAn adolescent boy with severe viral hepatitis and elevated unconjugated bilirubin was diagnosed postmortem to have kernicterus and central pontine myelinolysis. This association has not been reported previously.
Comparison of agar gel electrophoresis and isoelectric focusing in multiple sclerosis and subacute sclerosing panencephalitis
Authors: Mattson David H. , Roos Raymond P. , Arnason Barry G. W. 
Published: January 2005

AbstractIsoelectric focusing (IEF) and agar gel electrophoresis (AGE) were used to examine cerebrospinal fluid (CSF) and sera from patients with multiple sclerosis (MS) and subacute sclerosing panencephalitis (SSPE). All 15 SSPE samples and 29 of the 33 MS CSF samples showed oligoclonal IgG bands by AGE and IEF. Serum bands were more frequent in SSPE than in MS and were more commonly detected by IEF than by AGE. In MS CSF the number of bands on IEF correlated with: (1) disease duration, (2) CSF IgG, (3) CSF IgG/albumin ratio, and (4) central nervous system IgG synthesis. Serial studies revealed increases in IEF band number in 4 of 10 MS and 3 of 5 SSPE CSF specimens; fluctuations in band intensity were also noted.Densitometric scans of CSF IEF gels showed high, sharply angled IgG band peaks in SSPE; the CSF band peaks in MS were flatter and had higher background IgG. The SSPE pattern could be maEN to resemble the MS pattern through addition of normal polyclonal IgG to SSPE CSF. These findings suggest that in addition to oligoclonal IgG, polyclonal IgG is synthesized locally within the central nervous system in MS.
Intracranial infection in cardiac transplant recipients
Authors: Britt Richard H. , Enzmann Dieter R. , Remington Jack S. 
Published: January 2005

AbstractInfections have produced most of the deaths in the Stanford cardiac transplant program. Of the first 182 transplant recipients, 27 developed nonviral intracranial infections: meningoencephalitis/abscess in 16 patients, meningitis in 9, and rhinocerebral phycomycoses in 2. The responsible organisms included aspergillus, toxoplasma, candida, klebsiella, cryptococcus, coccidioides, listeria, mucor, and rhizopus. Characteristically, the areas of meningoencephalitis and abscesses were multiple and deep seated. Intracranial infections were invariably associated with pulmonary or disseminated infection with the same organism.Computed tomographic (CT) brain scans in patients with meningoencephalitis often showed minimal, nonspecific, low‐density lesions which usually did not exhibit contrast enhancement. At surgery the lesions were found to differ from typical pyogenic abscesses in that capsules were not well developed, and the aspirate consisted of necrotic fragments of edematous white matter and inflammatory cells rather than liquefied pus.Aspergillus infections of the central nervous system usually developed within the first three months after transplantation. Cases of meningitis occurred at variable times after transplantation, but approximately half appeared within 30 days after immunosuppressive therapy for treatment of rejection was increased. The prognosis for brain abscess depended on the causative organism. All patients with aspergillus infection died despite treatment with amphotericin B. The toxoplasma abscess responded to a combination of sulfadiazine and pyrimethamine. Meningitis was successfully suppressed or cured with appropriate treatment except for 1 patient with disseminated Cryptococcosis.
Demyelination of the lateral geniculate nucleus in central pontine myelinolysis
Authors: Goldman James E. , Horoupian Dikran S. 
Published: January 2005

AbstractPostmortem examination of the central nervous system in 6 of 9 patients with central pontine myelinolysis (CPM) revealed demyelination in the lateral geniculate nucleus (LGN). Histologically, the geniculate lesions consisted of myelin swelling and disintegration, largely in the central part of the nucleus, with preservation of axons and neuronal perikarya. The pathological changes resembled those found in the basis pontis in CPM.
Postischemic hypervascularity of infancy: A stage in the evolution of ischemic brain damage with characteristic CT scan
Authors: Shewmon D. Alan , Fine Michael , Masdeu Joseph C. , Palacios Enrique 
Published: January 2005

AbstractOne to 2 weeks after severe brain ischemia, four infants developed an unusual vascular lesion with a characteristic appearance on computed tomography that has not been reported previously. Restricted areas (most frequently the basal ganglia and thalamus) displayed increased attenuation, which enhanced further upon infusion of contrast medium. Autopsy of one infant revealed that the CT abnormality corresponded to an extremely dense neovascular network which had almost completely replaced the parenchyma in that region. We postulate that this pathological change is a stage in the organization of ischemic brain damage reflecting the infant's vascular plasticity. Thus, hypoxia induces marked capillary proliferation in regions of normally high metabolism and capillary density. The CT pattern may prove useful in predicting the location and extent of sequelae to perinatal asphyxia.
Multiple sclerosis serum and cerebrospinal fluid immunoglobulin binding to fc receptors of oligodendrocytes
Authors: Ma Booe I. , Joseph Barry S. , Walsh Michael J. , Potvi Alfred R. , Tourtellotte Wallace W. 
Published: January 2005

AbstractSuspensions of bovine oligodendrocytes were used to study the immunofluorescent staining properties of sera from multiple sclerosis (MS) patients and normal individuals. All sera (14 MS patients, 8 patients with other neurological diseases, and 11 normal individuals) showed positive oligodendrocyte staining by indirect immunofluorescence. Staining persisted after extensive absorption of sera with bovine liver to remove nonspecific binding. Similar findings were obtained for cerebrospinal fluid from all 5 MS patients as well as all 5 patients with other neurological diseases. In additional studies to determine if binding is mediated by the Fc fragment of IgG, results were: (1) ultracentrifuged normal and MS sera failed to react with oligodendrocytes, (2) positive staining with oligodendrocytes was observed after heat aggregation of IgG, (3) ox red blood cells, complexed with antibody, reacted with oligodendrocytes to produce strong rosette formation, and (4) the rosette formation could be blocked by prior treatment of oligodendrocytes with heat‐aggregated IgG. The studies fail to support a previous claim for specific binding of immunoglobulins to oligodendrocytes in MS. However, they confirm and extend previous findings that the binding of IgG to oligodendrocytes may not necessarily be an antigen‐antibody reaction. Therefore, adherence reactions with a putative antibody must exclude Fc‐mediated binding.
Induction of recurrent experimental allergic encephalomyelitis with myelin basic protein
Authors: Panitch Hillel , Ciccone Carlo 
Published: January 2005

AbstractRecurrent experimental allergic encephalomyelitis (EAE) was induced in Lewis rats by inoculation of human myelin basic protein (MBP) and complete Freund's adjuvant (CFA). The animals developed clinical disease characterized by unsteadiness, ataxic gait, and abnormal posturing of the limbs. Spontaneous remissions and relapses were noted for periods up to 120 days. Histologically there were perivascular infiltrates of mononuclear cells, especially prominent in the cerebellar white matter. There was little evidence of demyelination. This study demonstrates that relapsing EAE may be induced with MBP in the rat. Sensitization with other myelin antigens is not required, although immunity to such antigens may be necessary to induce demyelination. It is postulated that relapsing EAE may be associated with a defect in suppressor cell function.
Complex partial status epilepticus in young children
Authors: McBride M. C. , Dooling E. C. , Oppenheimer E. Y. 
Published: January 2005

AbstractComplex partial status epilepticus (CPSE) has rarely been described in children. We have recently studied four girls, aged 1 to 4 years, who presented with CPSE. Their seizures were characterized by decreased level of consciousness, lack of response to familiar persons, diminished response to pain, staring, slow visual tracking, eye deviation, picking at nearby objects, and lip smacking. Three patients developed focal clonic activity during their seizures and one progressed to a generalized motor seizure after 4 hours of CPSE. Two patients had ictal electroencephalograms demonstrating temporpoccipital poly spikes and slow waves.