bluuebunny/crossref_metadata_2025 huggingface dataset:
Serum demyelinating factors in multiple sclerosis
Authors: Seil Fredrick J. , Westall Fred C. , Romine John S. , Salk Jonas
Published: January 2005

Sera from 21 patients with multiple sclerosis were applied to myelinated spinal cord cultures and evaluated for demyelinating activity. Samples were collected at various times when patients were on or off therapy with myelin basic protein or prednisone or both, and at various stages of disease. Five of 31 serum samples from 17 patients exhibited demyelinating activity when tested in a 40% concentration in tissue culture nutrient medium. Four of the 10 serum samples taken from patients with active or remitting multiple sclerosis (2 samples from the same patient) were demyelinative; 1 of 21 sera collected during stationary periods of disease was positive. There was no correlation between serum demyelinating activity and the presence or absence of treatment. Sera positive for demyelinating activity failed to inhibit myelin formation in initially unmyelinated cerebellar cultures.
Kearns‐sayre syndrome with reduced plasma and cerebrospinal fluid folate
Authors: Allen Richard J. , DiMauro Salvatore , Coulter David L. , Papadimitriou Alexander , Rothenberg Sheldon P.
Published: January 2005

A young woman with Kearns‐Sayre syndrome and progressive central nervous system deterioration over 15 years had decreased plasma and cerebrospinal fluid folate levels while receiving phenytoin for a seizure disorder. A muscle biopsy showed a “ragged red fiber” myopathy with reduced muscle carnitine and mitochondrial enzymes. Computed tomographic brain scans showed cerebral white matter hypodensities and bilateral calcification of the basal ganglia. The mechanism for the folate deficiency and altered ratio of plasma to cerebrospinal fluid folate is unknown, but the deficiency may be responsive to replacement therapy.
The crossed upgoing toe sign: A clinical study
Authors: Willoughby Ernest W. , Eason Robert
Published: January 2005

We compared the crossed upgoing toe sign with the plantar response as an indicator of pyramidal tract dysfunction in 125 normal subjects and 192 patients with neurological disorders. A positive crossed upgoing toe sign was associated significantly with a partial pyramidal tract lesion in the contralateral cerebral hemisphere with a frequency similar to that of Babinski's sign. Unlike Babinski's sign, however, the positive crossed upgoing toe sign was lost when pyramidal weakness was severe enough to produce paralysis of voluntary dorsiflexion of the great toe, and it was found only rarely with pyramidal tract lesions of the spinal cord. The crossed upgoing toe sign has little value as a sensitive indicator of a pyramidal tract lesion. It is potentially of limited value as an aid in determining the level of a pyramidal tract lesion (cerebral hemisphere versus spinal cord), but its usefulness is seriously impaired by the high frequency of false positive signs in normal subjects and patients with other neurological disorders.
Quantitative sudomotor axon reflex test in normal and neuropathic subjects
Authors: Low P. A. , Caskey P. E. , Tuck R. R. , Fealey R. D. , Dyck P. J.
Published: January 2005

We have quantified postganglionic sweat output in human subjects resulting from axon reflex stimulation using acetylcholine electrophoresis. Dehumidified nitrogen of controlled temperature and flow rate was passed through an acrylic plastic chamber placed over a defined area of skin. Sweat droplets were evaporated; humidity change was sensed by a narrow‐range humidity sensor housed in a temperature‐controlled compartment and was plotted on a chart recorder. The time integral (area under the curve) was continuously integrated and converted to absolute units using a derived equation. Because stimulation and recording were simultaneous, an accurate determination of the latency of the sweat response was also possible.

Quantitative sudomotor axon reflex tests were performed on the left forearm and foot of 33 female and 29 male normal subjects aged 11 to 69 years. Acetylcholine, 10%, was electrophoresed for 5 mA‐minutes in the forearm and 10 mA‐minutes in the foot, and recording was continued for an additional 5 minutes. The mean sweat output in males was 2.7 and 3.0 times that in females in forearm and foot, respectively (p < 0.0001). Studies in selected autonomic neuropathies confirm that quantitative sudomotor axon reflex tests will detect postganglionic sudomotor abnormalities sensitively and reproducibly.
Hereditary form of sustained muscle activity of peripheral nerve origin causing generalized myokymia and muscle stiffness
Authors: Auger Raymond G. , Daube Jasper R. , Gomez Manuel R. , Lambert Edward H.
Published: January 2005

Six patients in two unrelated families had a hereditary form of sustained muscle activity of peripheral nerve origin. Although varying in severity among affected family members, the disease was manifested clinically as generalized myokymia and muscle stiffness. Motor and sensory nerve conduction velocities and results of nerve biopsy were normal. Repetitive after‐discharges followed each motor nerve stimulus. Needle electrode examination demonstrated spontaneous recurrent bursts of motor unit activity. The burst activity of a motor unit occurred rhythmically (0.5 to 4 bursts per second) and independently of the activity of other units. Within a burst, the firing rate was high (150 to 300 Hz). The clinical and electromyographic abnormalities improved considerably after treatment with carbamazepine or phenytoin.
Calcium and vitamin D metabolism in guamanian chamorros with amyotrophic lateral sclerosis and parkinsonism–dementia
Authors: Yanagihara Richard , Garruto Ralph M. , Gajdusek D. Carleton , Tomita Akio , Uchikawa Takashi , Konagaya Yoko , Chen Kwang‐Ming , Sobue Itsuro , Plato Chris C. , Gibbs Clarence J.
Published: January 2005

We evaluated 16 Guamanian Chamorros with amyotrophic lateral sclerosis and 33 patients with parkinsonism‐dementia for disturbances of calcium and vitamin D metabolism. The serum immunoreactive parathyroid hormone level was mildly elevated in 6 patients with amyotrophic lateral sclerosis and in 5 patients with parkinsonism‐dementia. There were significant positive correlations between serum immunoreactive parathyroid levels and duration of illness in male patients with motor neuron disease, but not in female patients or in patients with parkinsonism‐dementia. Intestinal absorption of calcium, as assessed by serum and urinary activity of calcium 47 following oral administration, was decreased in 2 patients with amyotrophic lateral sclerosis and in 4 patients with parkinsonism‐dementia, all of whom had low levels of serum 1,25‐dihydroxyvitamin D. Reduction in cortical bone mass were striking in patients with motor neuron disease. A significant negative correlation was found between the percentage of cortical area of the second metacarpal bone and muscle atrophy and weakness, and significant positive correlations were found between degree of immobility and ratio of urinary hydroxproline to creatinine in patients with amyotrophic lateral sclerosis and parkinsonism‐dementia. In general abnormalities in calcium metabolism were subtle. Thus, if the demonstrated deposition of metals, particularly calcium and aluminum, in central nervous system tissues of Guamanians with these two conditions is a cause of the diseases and of the early appearance of neurofibrillary tangles in neurons, the accumulation has apparently occurred long before onset of symptoms, and detectable abnormalities of calcium and vitamin D metabolism may already have been corrected.
The lymphoproliferative response to measles virus in twins with multiple sclerosis
Authors: Greebstein Jeffrey I. , McFarland Henery F. , Mingioli Elizabeth S. , McFarlin Dale E.
Published: January 2005

The cellular immune response to measles virus, as measured by lymphocyte proliferation in normal individuals, is considerably lower than that to mumps or vaccinia viruses, and stable multiple sclerosis patients do not differ significantly from the norm. The response to these viruses was studied in 28 twin sets both concordant and discordant for multiple sclerosis. Normal responses to mumps and vaccinia viruses occurred throughout. Seven affected twins manifested a persistently elevated response to measles virus, whereas the unaffected twins had a (normal) low response. The differences were unrelated to differences in T cell subsets, unusual kinetics of the response, or differential susceptibility of lymphocytes to the effects of measles virus infection in vitro. The specificity of the response resides in an E+ subpopulation, and the addition of low‐responder E+ cells to high‐responder E+ cells failed to identify an active low‐responder suppressor population. These findings suggest the presence of clonally expanded measles‐specific T cell populations in the high responders with multiple sclerosis.
Levels of adenylate deaminase, adenylate kinase, and creatine kinase in frozen human muscle biopsy specimens relative to type 1/type 2 fiber distribution: Evidence for a carrier state of myoadenylate deaminase deficiency
Authors: Fishbein William N. , Davis John I. , Armbrustmacher Vernon W. , Griffin Joe L. , Foster Walter D.
Published: January 2005

Myoadenylate deaminase deficiency is believed to refelct a genettic deficiency of skeletal muscle, but it pattern of inheritance has not been established. We examined, histochemically and by quantitative biochemical assay, muscle biopsy specimens from 3 putative carriers of this disorder. Adenylate kinease and creatine kinease were assayed in parallel with adenylate deaminease in order to establish enzyme activity ratios and the variation of each enzyme with fiber‐type distribution. Control tissue consisted of 34 biopsy specimens without notable abnormalities from 30 patients, and included 4 specimen pairs with disaparate fiber‐type contributions. By linear regression analysis, adenylate deam inase level averaged 2.8‐fold higher, and adenylate kinase 4.5‐fold higher, in type 2 than in type 1 fibers, whereas creatine kinase level did not differ. The slopes of the regression lines resulting from analysis of the four specimen pairs from infividual patients agreed well with the overll regression line in each plot. The 3 putative carriers had adenylate deaminease levels 2.5 to 5.7 times lower than the mean control value for thier fiber‐type distribution, but at least 20 times higher than thier enzyme‐deficient kinfolk. This finding indicates that a carrier state does exist, and that the deficencny state reflects an autosomal recessive inheritance pattern. Three additional biopsy specimens were excluded from evaluation when preliminary analysis showed elevated adenylate kinease/adenylate deaminase ratios that were outliers at the 1% level. This result suggests a carrier incidence of 10% in the muscle biopsy specimen population, which would markedly bias population estimates if undetected.
Hypometric saccades and low‐gain pursuit resulting from a thalamic hemorrhage
Authors: Brigell Mitchell , Babikian Viken , Goodwin James A.
Published: January 2005

Saccadic and pursuit eye movements were measured in a patient with a right thalamic hemorrhage using an infrared reflection technique. Saccades were hypometric when directed away from the side of the lesion, and pursuit was low in gain, with many interposed saccades, when directed toward the side of the lesion. This eye movement pattern is similar to that observed in hemidecorticate patients. We postulate that the ocular abnormalities observed in our patients were secondary to an interruption of the efferent pathways from the right cerebral areas mediating saccadic and smoothpursuit eye movements.
Microangiopathy of vasa nervorum in dysglobulinemic neuropathy
Authors: Powell Henry C. , Rodriguez Moses , Hughes Richard A. C.
Published: January 2005

Thickening of vessel walls resulting from endothelial proliferation was observed in the vasa nervorum of eleven patients with peripheral neuropathy associated with dysglobulinemia. Electron microscopy showed endothelial proliferation accompanied by abnormal accumulation of masses of intracytoplasmic filaments in each case. Of the eleven patients with dysglobulinemia, nine had monoclonal gammopathy and two were found to have polyclonal elevation of gamma globulin levels. Symptoms of neuropathy characteristically preceded detection of serum protein abnormalities by months to years. Nerve fiber lesions involved primarily the axon in four cases; segmental demyelination was the principal abnormality in the other seven. Both abnormalities were present to some degree in all eleven patients. Biphasic myelinopathy with uniform separation of myelin lamellae attributable to globulin deposition was observed in four cases.

The microvascular changes included endothelial cytoplasmic enlargement, virtually obliterating the vessel lumen in many instances, with thickening of pericytes, in which intracytoplasmic filaments were prominent and pinocytotic vesicles numerous. No extracellular filaments were noted, and amyloid stains were negative. Possible effects of these microvascular changes include ischemia resulting from severe vascular luminal narrowing, and altered vascular permeability. Severe loss of axons in this group of neuropathies may be the result of ischemia, whereas altered vascular permeability may admit globulin into the endoneurium, where it may directly affect the myelin sheath and precipitate demyelination.
Mechanisms of recovery from aphasia: Evidence from serial xenon 133 cerebral blood flow studies
Authors: Knopman David S. , Rubens Alan B. , Selnes Ola A. , Klassen Arthur C. , Meyer Maurice W.
Published: January 2005

In 21 patients who suffered aphasia resulting from left hemisphere ischemic infarction, the xenon 133 inhalation cerebral blood flow techique was used to measure cerebral blood flow within 3 months and 5 to 12 months after stroke. In addition to baseline measurements, cerebral blood flow measurements were also carried out while the patients were performing purposeful listening. In patients with incomplete recovery of comprehension and left posterior temporal–inferior parietal lesions, greater cerebral blood flow occurred with listening in the right inferior frontal region in the late studies than in the early studies. In patients with nearly complete recovery of comprehension and without left posterior temporal–inferior parietal lesions, early listening studies showed diffuse right hemisphere increases in cerebral blood flow. Later listening studies in this latter patient group showed greater cerebral blood flow in the left posterior temporal–inferior parietal region. The study provides evidence for participation of the right hemisphere is language comprehension in recovering aphasics, and for later return of function in left hemisphere regions that may have been functionally impaired early during recovery.
Value of computed tomographic scan in the evaluation of adult patients after their first seizure
Authors: Ramirez‐Lassepas Manuel , Morillo Luis R. , Gumnit Robert J. , Cipolle Robert J.
Published: January 2005

The role of the computed tomographic (CT) scan in evaluating adults after their first seizure(s) was determined by reviewing the hospital records of 148 patients studied within 30 days of their ictus. Patients with alcohol or drug intoxication or withdrawal or with known brain tumor, craniotomy, or open skull fracture were excluded. Type of seizure; neurological, CT, and electroencephalographic findings; final diagnosis; and hospital outcome were cross correlated.

The cause of seizure was established in 71 patients (48%); a structural lesion was identified by CT in 55 (37%), and 16 (11%) had metabolic seizures. CT findings agreed with the results of neurological examination 82% of the time. Structural lesions (including three tumors) were found by CT in 14 patients (15%) with nonfocal findings and in 12 (22%) of those with generalized electroencephalographic abnormalities.

Our findings are similar to those reported in adult epileptics and lead us to conclude that the CT scan is a valuable aid in establishing the cause of seizures and in disclosing early lesions.
Tissue culture demyelination by normal human serum
Authors: Silberberg Donald H. , Manning Margaret C. , Schreiber Alan D.
Published: January 2005

Serum from all of 20 normal individuals induced some degree of demyelination when applied to well‐myelinated mouse cerebellum cultures. An intact complement sequence through C5 is required. Demyelinating activity was heat labile at 56°C for 30 minutes but was not destroyed at 50°C for 30 minutes (which inhibits properdin factor B and alternate complement pathway activation, but not the classic complement pathway). Sera from patients with agammaglobulinemia, C4 deficiency, or C6 deficiency all induced demyelination. Our results suggest that tissue culture demyelination results from nonimmunoglobulin activation of the alternate complement pathway and is not limited to sera from patients with neurological disease.
Azathioprine in the treatment of myasthenia gravis
Authors: Witte Arnold S. , Cornblath David R. , Parry Gareth J. , Lisak Robert P. , Schatz Norman J.
Published: January 2005

Twenty‐four patients with myasthenia gravis were treated with azathioprine. Eighteen of the patients tolerated the drug. Six discontinued azathioprine therapy because of toxicity. Of the 18 patients, 15 (83%) improved while receiving azathioprine; in 8 (44%) improvement was felt to result solely from azathioprine. Initial response was seen after 4 to 10 months of treatment, with a mean of 6.4 months. Patients continued to improve for up to 24 months, with the mean time of peak improvement being 14 months. Relapse occurred within one year in all 6 patients in whom azathioprine administration was discontinued. Azathioprine is a reasonable alternative to corticosteroids in selected myasthenic patients requiring immunosuppression.
Augmented anti—acetylcholine receptor response following long‐term penicillamine administration
Authors: Bever Christopher T. , Dretchen Kenneth L. , Blake George J. , Chang Hai W. , Penn Audrey S. , Asofsky Richard
Published: January 2005

Because of the association of D‐penicillamine (DP) therapy with myasthenia gravis, we have studied long‐term DP treatment in five inbred strains of mice with doses comparable to those used in patients with rheumatoid arthritis. No clinical weakness or anti—acetylcholine receptor (AChR) antibody developed with up to 6 months of treatment, but augmented responses did occur to challenge with purified AChR in adjuvant. Anti‐AChR antibody titers in C57BL/6 and C3H/He mice were significantly higher after challenge with AChR in DP‐treated than in control mice. Augmented anti‐AChR titers were not seen in strain A mice, but after 6 months of DP treatment increased susceptibility developed to the induction of experimental autoimmune myasthnia gravis. Nine weeks after challenge with purified AChR, 10 of 11 mice developed clinical weakness, leading to death in 6. Results of edrophonium testing were positive in 5 of 6 mice, and electrophysiological abnormalities were demonstrated in 3 of the surviving mice.

Long‐term DP treatment is associated with augmented anti‐AChR antibody responses in C3H/He and C57BL/6 mice, and increased susceptibility to experimental autoimmune myasthenia gravis in strain A mice.
Coronary artery disease in patients with cerebrovascular disease: A prospective study
Authors: Rokey Roxann , Rolak Loren A. , Harati Yadollah , Kutka N. , Verani Mario S.
Published: January 2005

Coronary artery disease is the cause of death in most patients who have transient ischemic attacks or stroke. Evaluation for this condition is not routinely performed in such patients, and no prospective studies have been reported. We prospectively examined 50 consecutive patients with transient ischemic attacks or mild stroke to determine the prevalence and importance of coronary artery disease. All patients were examined by a cardiologist and underwent both exercise thallium‐201 scintigraphy and exercise radionuclide ventriculography. Sixteen patients were suspected to have coronary artery disease on the basis of clinical evaluation. In 15 of these the disease was confirmed by the nuclear scans. The remaining 34 patients had no clinical evidence of heart disease, yet 14 had abnormal cardiac scans. Twenty of 22 patients with abnormal scans who underwent cardiac catheterization had significant coronary artery disease or a cardiomyopathy. The discovery of heart disease altered clinical management in 13 patients. Overall, 29 of 50 patients had significant coronary artery disease, compared with a 7% prevelence of the condition in other patients of similar age at the same institution.
Clinical features of carbamyl phosphate synthetase‐I deficiency in an adult
Authors: Call Gregory , Seay Alan R. , Sherry Richard , Qureshi Ijaz A.
Published: January 2005

Carbamyl phosphate synthetase‐I (CPS‐I) catalyzes the first reaction required for the conversion of ammonia to urea through the urea cycle. Severe CPS‐I deficiency causes marked hyperammonemia with encephalopathy in infancy and usually results in death within the first few months of life. We describe a 33‐year‐old woman whose CPS‐I activity is less than 5% of normal. She has had mild, intermittent symptoms throughout life but has never experienced severe encephalopathy. Although mildly retarded, she has no major neurological deficits. Therapy with a low‐protein diet, lactulose, and sodium benzoate has prevented recurrence of hyperammonemia and symptoms. Cranial computed tomographic scans demonstrate prominent lucency of cerebral white matter, and cerebral evoked potential recordings indicate slowed central conduction. These findings suggest that the metabolic disturbances in this patient may have adversely affected central myelin formation or maintenance. This woman represents, to our knowledge, the oldest reported patient with CPS‐I deficiency, and the case illustrates the need to consider urea cycle disorders in the differential diagnosis of intermittent neurological symptoms regardless of the patient's age.
Cerebral amyloid angiopathy causing intracranial hemorrhage
Authors: Kalyan‐Raman Uma P. , Kalyan‐Raman Krishna
Published: January 2005

Ten cases of sporadic cerebral amyloid angiopathy (CAA) were seen as intracranial hemorrhage. CAA was seen as a stroke or catastrophic intracranial hemorrhage in all cases. CAA complicated by intracranial hemorrhage carried a high mortality; 9 of 10 patients died in spite of aggressive medical or surgical treatment. Senile dementia of Alzheimer's type was an associated finding in only 1 patient. Computed tomographic scanning in 4 patients initially was not diagnostic for hemorrhage. Lambda chains and amyloid P protein were demonstrated in the areas of amyloid angiopathy immunocytochemically. The pathogenesis of hemorrhage in CAA could be either increased fragility of amyloid‐laden blood vessels or rupture of microaneurysms. Surgical treatment of intracranial hemorrhage in CAA should therefore be undertaken with caution.
Hexacarbon neuropathy: Cell body changes are early, dynamic, and specific
Authors: Sterman Arnold B.
Published: January 2005

To study the evolution of cell body alterations during toxic neuropathy we exposed rats to the prototype neurotoxin 2,5‐hexanedione and examined perikarya of lumbar dorsal root ganglia with electron microscopy and stereology at three stages of neuropathy. Compared to unintoxicated controls, neurons from rats with incipient (four weeks) and intermediate (six to seven weeks) neuropathy showed dispersion of Nissl substance and significant decreases (p < 0.001) in the volume fractions of Nissl bodies, but not of mitochondria or Golgi apparatus. However, at advanced (twelve to fourteen weeks) stages the volume fraction of Nissl bodies had increased and no longer differed from that of control; distinct chromatolysis‐like changes also became prominent. To evaluate the specificity of this remodeling we compared current morphometric results to data from rats exposed to acrylamide monomer and found significant differences (p < 0.001) in the volume fractions of Nissl bodies and mitochondria. We conclude: (1) in axonopathy, cell body remodeling occurs early and advances as a dynamic, evolving process, and (2) distinct differences in the patterns of cell body changes can distinguish the neuropathies studied, implying distinct cell body functions.
Principles and pitfalls of nerve conduction studies
Authors: Kimura Jun
Published: January 2005

This report reviews the fundamental principles and the changing concepts of nerve stimulation techniques, and discusses the proper application of these techniques in the differential diagnosis of peripheral nerve disorders. Nerve conduction studies help delineate the extent and distribution of the neural lesion and distinguish two major categories of peripheral nerve disease: demyelination and axonal degeneration. Although the method is based on simple principles, pitfalls abound in practice.

Variability in nerve conduction measurement may result from temperature change, variations among nerve segments, and the effects of age. Other sources of error include excessive spread of stimulation current, anomalous innervation, temporal dispersion, and inaccuracy of surface measurement. Unlike a bipolar dervation, which selectively records near‐field potentials, a referential recording may give rise to stationary far‐field peaks from a moving source. Overlooking this possibility can lead to an incorrect interpretation of findings.

Conventional nerve conduction studies deal primarily with measurements of the distal nerve segments in an extremity. More recent techniques are applicable to less accessible anatomical regions, as illustrated by elicitation of the blink reflex, F wave, and H reflex, and the use of the inching technique. Other methods used to assess special aspects of nerve conduction include the ischemic test and studies of slow‐conducting fibers.
Transient visual obscurations with elevated optic discs
Authors: Sadun Alfredo A. , Currie John N. , Lessell Simmons
Published: January 2005

The pathogenesis of the transient obscurations of vision that occur with papilledema is a subject of speculation and debate. We present four examples of transient obscurations of vision that were clinically indistinguishable from those of papilledema; they occurred in patients with elevated optic discs from causes not related to increased intracranial pressure. The underlying mechanism for visual obscurations in all of these patients appears to be transient ischemia of the optic nerve head consequent to increased tissue pressure. Axonal swelling, intraneural masses, and increased influx of interstitial fluid may all contribute to increases in tissue pressure in the optic nerve head. The consequent reduction in perfusion pressure renders the small, low‐pressure vessels that supply the optic nerve head vulnerable to compromise. Brief fluctuations in intracranial or systemic blood pressure may then result in transient loss of function in the eyes. We postulate that such mechanisms may apply in all cases of optic disc elevation, including papilledema.
More intense focal seizure types after callosal section: The role of inhibition
Authors: Spencer Susan S. , Spencer Dennis D. , Glaser Gilbert H. , Williamson Peter D. , Mattson Richard H.
Published: January 2005

Corpus callosum section reduces the incidence of generalized seizures associated with loss of conciousness. Minimal data are available, however, regarding the types of seizures that may be present following surgery. Five of 17 patients in our clinic who underwent partial or complete corpus callosum section developed more intense and sometimes newly patterned (but not necessarily more frequent) focal seizures postoperatively. These patients were not predictably different from the other 12 in terms of age at onset of seizures or at surgery, cause of seizures, extent of callosum sectioned, radiographically demonstrable structural lesions, or the types of preoperative seizures. All 5 patients, however, had bilateral independent frontal electroencephalographic foci. No patients without these electroencephalographic findings developed more intense focal seizures after surgery. The findings are consistent with the results of experimental animal studies and argue for a suppressive influence of the contralateral hemisphere on some types of seizures.
Ocular palsy preceding chronic relapsing polyneuropathy by several weeks
Authors: Donaghy Michael , Earl C. J.
Published: January 2005

Three patients exhibited an unusual variant of chronic relapsing polyneuritis in which extroacular muscle palsies developed 19 days, 20 days, and 31/2 months, respectively, before the neuropathy affected the limbs. The peripheral Neuropathy evolved over 7 to 12 weeks, lasted 15 months to 13 years, and relapsed in every case. Each patients had dysphagia, areflexia, sensory loss, and weakness of all four limbs, which in two was asymmetrical. Slowed motor nerve conduction and prolonged F wave latencies were present, and the cerebrospinal fluid protien level was elevated without increased cell count. No patient recovered fully despite treatment with immunosuppressant drugs.
Hypophysectomy mitigates skeletal muscle fiber damage in hamster dystrophy
Authors: Karpati G. , Jacob P. , Carpenter S. , Prescott S.
Published: January 2005

Ablation of the pituitary gland by a rediofrequency lesion markedly retarded the musculoskeletal growth of young dystrophic hamsters. The prevalence of centronucleared muscle fibers, which is a reliable cumulative index of the microscopic patholgical expression of dystrophy, was drastically reduced in quadriceps muscles of 35‐ and 45‐day‐old hypophysectomized dystrophic hamsters, campared with sham‐operated controls. Mitigation of skeletal muscle fiber damage by musculoskeletal growth retardation may also occur in human dystrophy.
Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's syndrome
Authors: Wisniewski K. E. , Wisniewski H. M. , Wen G. Y.
Published: January 2005

One hundred brains of patients with Down's syndrome(DS) who died in insstitutions for chronic care were exemined for clinicopathological correlation of Alzheimer's disease. Fifty‐one were below and 49 were above age 30 years at death. Tissues from the right, prefrontal, and hippocampal cortices were processed for microscopy using H & E and Bodian‐periodic acid‐schiff impregnation. M orphometric evluations of plaques and tangles were carried out Plaques or plaques and tangles were found in the brains of 56 patients with DS, 7 below age 30 and 49 above the age of 30. The brains of the patients with DS who also had clinical dementia had more than twenty plaques or plaques and tangles per 1.5 × 106 μm2 of cortex. The numbers of plaques and tangles found in the brains of the patients with DS above the age of 30 greatly increased with age but varied from brain to brain. These observations suggest a coreelation among dementia, the density of plaques and tangles, and age. All 100 brains studied showed early arrest of brain growth and brain atrophy, a condition that may have been due to prenatal arrest of neurogenesis mainly in the granular cell layers, prenatal and postnatal arrest of synaptogenesis, and early aging. Plaques and tangles developed twenty to thirty years earlier and dementia was clinically detected at least three times more frequently (20 to 30%) in DS than it is known to occur in the non‐DS population.
Alterations in regional brain concentrations of neurotensin and bombesin in Parkinson's disease
Authors: Bissette Garth , Nemeroff Charles B. , Decker Michael W. , Kizer John S. , Agid Yves , Javoy‐Agid France
Published: January 2005

Frozen samples of postmortem human brain tissue from patients with Parkinson's disease(n = 25) and control patients who died without neurological disease (n = 25) were assayed for neurotensin and bombesin by specific radioimmunoassay. Twelve brain regions were examined: substantia nigra, ventral tegmental area, periaqueductal gray matter, caudate nucleus, putamen, globus pallidus, amygdala, hippocampus, nucleus accumbens, frontal cortex, cingulate cortex, and entorhinal cortex. In patients with Parkinson's disease, the concentration of neurotensin was significantly reduced in the hippocampus. The concentration of neither peptide was significantly altered in the substantia nigra or ventral tegmental area, two regions known to exhibit reductions in other neurotransmitter substances.
The relationship between levels of cerebrospinal fluid myelin basis protein and IgG measurements in patients with multiple sclerosis
Authors: Warren Kenneth G. , Catz Ingrid
Published: January 2005

Cerebrospinal fluid (CSF) myelin basic protein (MBP) levels, CSF/serum, albumin ratio (CSF/S alb), and 4 CSF IgG measurements‐absolute CSF IgG level (CSF IgG), CSF IgG/albumin ratio, the Tibbling‐Link IgG index, and the daily rate of intrathecal IgG synthesis‐were measured in patients with multiple sclerosis and control subjects. In four clinical subgroups of patients, including 22 with polysymptomatic exacerbations, 22 with monosymptomatic exacerbations, 41 with chronic progressiv disease and 21 in remission, there was no correlation between CSF MBP and either CSF/S alb or the CSF IgG measurements. This finding was also observed in longitudinal studies of patients. CSF MBP lavel, as determined in a cross‐sectional stydy of 325 patients with multiple sclerosis, are an exellent indicator of disease activity.
Atypical multiple sclerosis with concentric of lamellar demyelinated lesions: Two Japanese patients studied post mortem
Authors: Itoyama Yasuto , Tateishi Jun , Kuroiwa Yoshigoro
Published: January 2005

In two Japanese patients with atypical multiple sclerosis studied post mortem, there was a concentric or lamellar pattern of demyelinated lesions. This was detected in the thoracic spinal cord of one patient and in the optic chiasm of the other. In addition, lesions more typical of multiple sclerosis were present. Clinically, both cases ran courses intermediate between Baló's disease and multiple sclerosis. The patients had a relatively rapid progression of disease and had severe visual disability or paraplegia associated with faver, headache, and pleocytosis. The formation of a concentric or lamellar pattern of the lesions in cases of demyelinating diseases may relate to the brief duration or to the acuteness or severity of the disease process.
Induction of reproducible brain infarction by photochemically initiated thrombosis
Authors: Watson Brant D. , Dietrich W. Dalton , Busto Raul , Wachtel Mitchell S. , Ginsberg Myron D.
Published: January 2005

We have used a photochemical reaction in vivo to induce reprodcible thrombosis leading to cerebral infarction in rats. After the intravenous injection of rose bengal, a potent photosensitzing dye, an ischemic lesion was formed by irradiating the left parietal convexity of the exposed skull for 20 minutes with green light(560nm) from a filtered xenon arc lamp. Animals were allowed to surivive form 30 minutes to 15 days after irradiation. Early microscopic alerations within the irradiated zone included the formation of thrombotic plugs and adjacent red blood cell stasis within pial and parenchymal vessels. Scanning electron microscopy revealed frequent platelet aggregates adhering to the vascular endothelium, often resulting in vascular occlusion. Carbon‐black brain perfusion demonstrated that occlusion of vascular channels progressed after irradiation and was complete within 4 hours. Histopathological examination at 1, 5, and 15 days revealed that the associated infarct evolved reproducibly through several characteristic stages, including a phase of massive macrophage infiltration. Although cerbral infarction in this model is initiated by thrombosis of small bolld vessels, the fact that the main pathological features of stroke are consistently reproduced should permit its use in assessing treatment regimens, Further, the capability of producing infarction in preselected cortiacal regions may facilitate the study of behavioral, functional, and structural consequences of acute and chronic stroke.
Tissue culture studies of neurofibromatosis: Effects of axolemmal fragments and cyclic adenosine 3′,5′‐monophosphate analogues on proliferation of schwann‐like and fibroblast‐like neurofibroma cells
Authors: Sobue Gen , Sonnenfeld Kenneth , Rubenstein Allen E. , Pleasure David
Published: January 2005

Six dermal neurofibromas obtained from 5 patients with neurofibromatosis were dissociated and the cells were plated on polylysine‐coated glass. Two principal cell types were observed in the cultures: elongated and bipolar Schwann‐like cells (SLCs), and polymorphic flattened fibroblast‐like cells (FLCs). Indirect immunofluorescence demonstrated that SLCs expressed surface laminin but not surface fibronectin; FLCs expressed surface fibronectin but were only weakly positive for surface laminin. Tritiated thymidine autoradiography demonstrated that cultured SLCs proliferated slowly (labeling index, 0.7 to 4.0%), whereas FLCs divided more rapidl%(labeling index, 7.5 to 26.4%). Axolemmal fragments prepared from human or rat central nervous system specimens adhered to SLCs derived from each of the 6 neurofibromas, but not to FLCs. Axolemmal fragments induced a marked proliferative response of SLCs from 2 of the 6 neurofibromas but had no effect on proliferation of SLCs from the other 4 neurofibromas or FLCs from any of the 6 neurofibromas. In one patient from whom 2 neurofibromas were obtained, SLCs from one neurofibroma responded to axolemmal fragments, while SLCs from the other did not. Treatment of the cultures with 0.1 mM cyclic adenosine 3′,5′‐monophosphate (cAMP) analogue, 8‐bromo cAMP, caused marked inhibition of proliferation of both SLCs and FLCs derived from all 6 neurofibromas. The same concentration of another cAMP analogue, dibutyryl cAMP, inhibited proliferation of SLCs but not of FLCs.
Hypoglycemia masquerading as cerebrovascular disease (hypoglycemic hemiplegia)
Authors: Wallis William E. , Donaldson Ivan , Scott Russell S. , Wilson John
Published: January 2005

Hypoglycemia produced hemiplegia with right‐sided predilection in 16 patients initially suspected of having suffered a stroke. Fifteen patients had no demonstrable brain disease, and the hemiplegia cleared rapidly once the hypoglycemia was corrected. Invasive investigations such as carotid arteriography are not required in most patients. The features of hypoglycemia hemiplegia suggest that a selective neuronal vulnerability and not underlying focal brain disease is responsible in most cases.
Neurological complications in infants and children with acquired immune deficiency syndrome
Authors: Belman Anita Lesgold , Ultmann Monica H. , Horoupian Dikran , Novick Brian , Spiro Alfred J. , Rubinstein Arye , Kurtzberg Diane , Cone‐Wesson Barbara
Published: January 2005

Neurological complications occurred in 6 children, aged 6 months to 5 years, with acquired immune deficiency syndrome who were followed for 14 months. The most frequent manifestations included encephalopathies, acquired microcephaly, and pyramidal tract signs. Computed tomographic examinations showed variable degrees of cortical atrophy with ventricular dilatation and calcification. Electrophysiological abnormalities were demonstrated. Two children had documented central nervous system infections. Neurological deterioration resulted in dementia in 3 children. Cognitive impairment and developmental delays were evident in the other 3. Postmortem examination of the 3 children who died showed subacute cytomegalovirus encephalitis in 1; nonspecific hemispheric white matter changes, calcific vasopathy of the basal ganglia, and striking bilateral corticospinal tract degeneration in the second; and extensive calcific vasopathy of the basal ganglia and frontal centrum semiovale, and bilateral attenuation of the frontopontine and corticospinal tracts in the third.
Diabetic autonomic dysfunction, blood pressure, and sleep
Authors: Guilleminault Christian , Mondini Susanna , Hayes Boyd
Published: January 2005

We measured serially the blood pressure of 7 normal controls and 11 Type I insulin‐dependent and 6 Type II non‐insulin‐dependent diabetic patients during sleep. All subjects underwent 2 or 3 nights of monitoring during which blood pressure was measured with an arteriosonde, a Doppler‐type system with a cuff that automatically inflates every 15 minutes. All 7 normal controls, 3 of 11 Type I, and all 6 Type II diabetic patients had normal results from bedside tests of autonomic function. The Type I patients had abnormal systolic blood pressure measurements throughout the night. Unlike normal controls and Type II diabetics, their blood pressure did not rise progressively in the early morning hours. During rapid eye movement (REM) sleep, their blood pressure measurements were significantly higher and lower than values measured during non‐REM sleep, in sharp contrast to those in the other two groups, whose REM sleep–related blood pressure values were slightly above the preceding non‐REM sleep measurements.
Joseph's disease: Clinical and pathological studies in a Japanese family
Authors: Yuasa Tatsuhiko , Ohama Eisaku , Harayama Hiromi , Yamada Mitsunori , Kawase Yasuhiro , Wakabayashi Masatoshi , Atsumi Tetsushi , Miyatake Tadashi
Published: January 2005

Joseph's disease is a hereditary ataxia found among descendants of Portuguese from the Azores Islands. We describe the clinical and pathological features of 4 members of a Japanese family who were diagnosed as having Joseph's disease. The illness began with cerebellar ataxia between the ages of 18 and 45 years. Nystagmus, dysarthria, and pyramidal signs were early manifestations. External ophthalmoplegia, dystonia and/or athetotic movements, and muscular atrophy appeared in the late stages. Neuropathological findings in one patient revealed degeneration of the dentatorubral and pallidoluysian systems, substantia nigra, pontocerebellar system, Clarke's column and spinocerebellar tracts, and anterior horn cells, as well as the cranial nuclei in the brainstem. Neurons in the inferior olivary nuclei, Purkinje's and granule cells, the cerebral cortex, thalamus, and striatum were spared. Involvement of the dentatorubral and pallidoluysian systems seems to be a characteristic feature of this disease in Japan.
Normal myelination of regenerating peripheral nerve sprouts despite circulating antibodies to galactocerebroside in rabbits
Authors: Stoll Guido , Reiners Karlheinz , Schwendemann Günther , Heininger Kurt , Toyka Klaus V.
Published: January 2005

Rabbits were immunized with galactocerebroside and a crush lesion was created in the tibial nerve before the onset of experimental allergic neuritis. Normal regeneration and myelination of distal peripheral nerve sprouts occurred and was identical to that of controls, although circulating antigalactocerebroside antibodies were present and nerve roots showed typical signs of beginning experimental allergic neuritis.
Abnormal platelet glutamate dehydrogenase activity and activation in dominant and nondominant olivopontocerebellar atrophy
Authors: Sorbi Sandro , Tonini Stefano , Giannini Emiliana , Piacentini Silvia , Marini Paolo , Amaducci Luigi
Published: January 2005

Glutamate dehydrogenase (GDH) activity and its allosteric modulation by purine nucleotides were studied in platelet preparations from 4 patients with a nondominant form of adult‐onset olivopontocerebellar atrophy (OPCA) and in affected and nonaffected members of two families with a dominant form of OPCA. A partial deficiency of GDH activity (40 to 50% of control values) was present in 3 patients with nondominant OPCA and in 2 patients, father and son, with a dominant form of OPCA. Platelet GDH from these patients and controls was regularly inactivated by 2 mM guanosine‐5′‐triphosphate (GTP) and simulated one‐ to twofold by 2 mM adenosine‐5′‐diphosphate (ADP). In the presence of 0.2% Triton X‐100, the activating effect of ADP was enhanced four‐ to sixfold. The partial deficiency in maximum catalytic activity observed in these patients persisted under all conditions used for enzyme assay. In affected members, but not in one unaffected member of another family with a dominant type of OPCA, GDH activity was in the control range but was not activated by ADP in either the presence or absence of Triton. These results suggest that there may be at least two possible alterations of GDH in patients with OPCA: one which decreases the maximum catalytic activity and one which impairs the regulatory properties of the enzyme. Furthermore, this study suggests that platelet GDH determination in patients with OPCA may provide a simple and useful tool to classify these disorders and to understand the basic pathophysiological mechanisms involved.
Blink‐induced saccadic oscillations
Authors: Hain T. C. , Zee D. S. , Mordes M.
Published: January 2005

A patient with a neurodegenerative disease had abnormal saccades only when he blinked. These saccades were hypermetric and were followed immediately, without any intersaccadic interval, by a large, oppositely directed saccade (dynamic overshoot). To explain these findings, we hypothesize that a blink‐related neural signal can modulate the activity of pause cells that normally inhibit saccadic burst neurons during fixation. In pathological circumstances, abnormal function of pause cells could lead to large‐amplitude saccadic oscillations. In normal subjects, blinks could induce short bursts of low‐amplitude flutter.
Benign versive or circling epilepsy with bilateral 3‐cps spike‐and‐wave discharges in late childhood
Authors: Gastaut Henri , Aguglia Umberto , Tinuper Paolo
Published: January 2005

Twenty‐eight of 920 patients seen between the ages of 8 and 20 years for a first seizure manifested versive or circling events associated with bilateral rhythmic 3‐cps spike‐and‐wave discharges. In 21 (75%), the seizures appeared between the ages of 10 and 14 years. Paroxysmal adversion or gyration was always toward the same side in a given patient. “Break of contact” during the ictus was found in 70% of the seizures, and secondary generalization occurred in half. Ictal electroencephalographic recordings failed to show a focal hemispheric origin. Additional features were: absence of prior major diseases; normal results of neurological and neuroradiological examinations; excellent response to anticonvulsant treatment with sodium valproate and/or phenobarbital; frequent family history of epileptic seizures (25% of patients); and association with generalized seizures (57.2% of patients). We conclude that versive or circling epilepsy with bilateral rhythmic 3‐cps spike‐and‐wave discharges represents a benign form of primary generalized epilepsy in late childhood.
What determines the latency and amplitude of stationary peaks in far‐field recordings?
Authors: Kimura Jun , Kimura Akio , Ishida Tetsuro , Kudo Yutaka , Suzuki Shoji , Machida Masafumi , Matsuoka Hiroo , Yamada Thoru
Published: January 2005

In 20 radial nerves from 10 healthy persons, a referential derivation from the tip of the first or second digit registered two biphasic stationary peaks, PI‐NI and PII‐NII, following stimulation of the nerve in the forearm. These two peaks occurred slightly before the arrival of the propagating impulse at the wrist and at the base of the digit, respectively. With stepwise reduction of stimulation from a maximal to a threshold intensity, the far‐field potential decreased in amplitude linearly with the near‐field potential recorded at the junction of the volume conductor. Abduction of the first digit or flexion of the second and third digits altered the waveform and, to a lesser degree, the latency of the farfield peaks. However, these changes appeared in an inconsistent manner, which we were unable to characterize. We conclude that in far‐field recording a stationary peak results at the border of the adjoining volume conductors in proportion to the magnitude of the propagating axonal volley approaching the boundary.