bluuebunny/crossref_metadata_2025 huggingface dataset:
Hypothalamic hypothyroidism causing spastic paraplegia: Recovery following thyroid medication
Authors: Jackson Ivor M. D. , Papapetrou Peter D. , Hills John R. , Kassirer Marilyn
Published: January 2005

A 63‐year‐old woman with longstanding spastic paraplegia and neurological evidence of long tract disturbance was found to have hypothyroidism, partial diabetes insipidus, hyperprolactinemia, and gonadotropin deficiency of hypothalamic origin. Replacement therapy with thyroxine and prednisone induced complete remission of the neurological abnormalities. The association of spastic paraplegia with hypothalamic insufficincy has not been reported previously. The possibility of hypothalamic disease should be considered in cases of spastic paraplegia of unknown cause.
Failure to demonstrate papovavirus tumor antigen in human cerebral neoplasms
Authors: Greenlee John E. , Becker Laurence E. , Narayan Opendra , Johnson Richard T.
Published: January 2005

Cell cultures derived from 80 brain tumors failed to show the intranuclear tumor (T) antigen common to cells transformed by simian virus 40 (SV40) or BK or JC viruses.
Nonsensory neglect
Authors: Watson Robert T. , Miller Bayard D. , Heilman Kenneth M.
Published: January 2005

Five monkeys trained to perform with the extremity contralateral to a stimulus had unilateral neglect induced by frontal and reticular formation lesions. Postoperatively the performance of the animals was abnormal only on ipsilateral stimulation, which suggests that the mechanism underlying neglect in these subjects is not deafferentation or sensory inattention but a defect of intention.
The malignancy of dementias
Authors: Go Rodney C. P. , Todorov Alexandre B. , Elston Robert C. , Constantinidis Jean
Published: January 2005

Survival times were determined for 982 patients admitted over a ten‐year period to the Geneva Psychiatric Clinic. Patients with dementia had one‐third the life expectancy of controls. Among patients classed as having Alzheimer disease, the longest survival times were in those with neurofibrillary tangles involving the neocortex, while those lacking this anatomical abnormality had the shortest survival times. Except for women with Alzheimer disease, patients with dementia had less than 10% life expectancy from the time of their admission to the clinic compared to the life expectancy of Geneva's population.
Identification of speech lateralization by intracarotid injection of methohexital
Authors: Willmore L. James , Wilder B. Joe , Mayersdorf Assa , Ramsay R. Eugene , Sypert George W.
Published: January 2005

Speech lateralization was assessed in 25 patients with epilepsy by direct carotid injection or carotid catheterization from a femoral approach. Hemispheric anesthesia was induced by intracarotid injection of the rapidly acting barbiturate methohexital. This technique allowed same‐day study of both hemispheres, permitted repeated injection of either carotid artery without accumulation of methohexital sufficient to cause systemic effects, and added little to the total time required for three‐vessel cerebral angiography. Unilateral seizures were induced by methohexital injection in 4 patients. No other complication secondary to the methohexital study was observed.
Progressive dialysis encephalopathy
Authors: Lederman Richard J. , Henry Charles E.
Published: January 2005

The clinical features of 42 patients with the only recently recognized and generally fatal neurological syndrome of progressive dialysis encephalopathy are reviewed and the electroencephalographic and neuropathological findings are summarized. Despite apparently successful hemodialysis, these patients develop a wide spectrum of neurological abnormalities. Of these, sudden onset of hesitant, nonfluent speech is the most characteristic and usually the earliest sign. Both dysphasic and dysarthric elements are found, though the former predominate. Myoclonus, dementia, seizures, and gait difficulty are also seen in the majority of these patients. EEGs are more abnormal than would be expected for the clinical severity, with some type of high‐voltage spike‐wave pattern intermixed with abundant slow activity. The combination of clinical and EEG features in the appropriate setting is virtually diagnostic. Transient episodes with variable periods of complete or partial remission have been recognized. Neuropathological changes are surprisingly mild and nonspecific. The cause is uncertain; current speculation focuses on aluminum as the offending neurotoxin. Treatment remains unsatisfactory.
Treatment of elevated intracranial pressure in reye syndrome
Authors: Trauner Doris A. , Brown Frederick , Ganz Edward , Huttenlocher Peter R.
Published: January 2005

Continuous intraventricular monitoring of intracranial pressure appears to be a useful aid in the management of patients with Reye syndrome, especially those in stage 3 or 4 coma, in which the mortality rate remains high. Elevations in intracranial pressure can be treated promptly and before the patient shows clinical signs of deterioration. Careful attention to adequate airway care is essential. The use of paralytic agents to reduce pressure secondary to muscle movement is useful. The minimum dose of mannitol required can be carefully titrated, and sudden pressure changes can be combated by release of small amounts of cerebrospinal fluid.
HLA antigens in the Landry‐Guillain‐Barré syndrome and chronic relapsing polyneuritis
Authors: Stewart G. J. , Pollard J. D. , McLeod J. G. , Wolnizer C. M.
Published: January 2005

Forty‐four patients with inflammatory demyelinating polyneuritis (22 with Landry‐Guillain‐Barré syndrome, 6 with subacute polyneuritis, and 16 with chronic relapsing polyneuritis) were typed for genetic markers in and around the HLA region of chromosome 6. Patients with chronic relapsing polyneuritis showed a definite association with HLA‐AW30 and AW31 and probable associations with HLA‐B8, HLA‐DW3, and glyoxalase I. No significant associations were demonstrated with the Landry‐Guillain‐Barré syndrome although an increase in glyoxalase I was significant if combined with the results of typing in chronic relapsing polyneuritis. The total patient group showed significant increases in HLA‐AW30, HLA‐AW31, and HLA‐DW3. The results support the view that HLA‐linked genetic factors influence susceptibility to chronic relapsing polyneuritis and may contribute to the differences in clinical patterns observed in inflammatory demyelination of the peripheral nervous system.
Measurement of regional cerebral blood volume by emission tomography
Authors: Grubb Robert L. , Raichle Marcus E. , Higgins Carol S. , Eichling John O.
Published: January 2005

The technique of positron emission tomography was used to measure cerebral blood volume (CBV) in 10 normal right‐handed human volunteers following inhalation of trace quantities of cyclotron‐produced, 11C‐labeled carbon monoxide. In scans obtained 4 cm above the orbitomeatal line, CBV was 4.3 ml per 100 gm of tissue, whereas in scans obtained 8 cm above the orbitomeatal line, CBV was significantly less (3.3 ml per 100 gm; p < 0.001). This difference reflects the greater proportion of gray matter in the lower scan. Furthermore, the CBV was significantly larger (p < 0.001) in the left cerebral hemisphere in the tomographic scans obtained 4 cm above the orbitomeatal line. These scans include the region of the superior surface of the temporal lobe (planum temporale), which is thought to be larger in individuals with left cerebral dominance for speech. This observation is the first in vivo demonstration of a structural correlate of a known functional difference in the cerebral hemispheres of man.
Recurrent seizures induced by cortical iron injection: A model of posttraumatic epilepsy
Authors: Willmore L. James , Sypert George W. , Munson John B.
Published: January 2005

A single injection of 5 or 10 μl of ferrous or ferric chloride into rat or cat sensorimotor cortex resulted in chronic recurrent focal paroxysmal electroencephalographic discharges as well as behavioral convulsions and electrical seizures. Iron‐filled macrophages, ferruginated neurons, and astroglial cells surrounded the focus of seizure discharge. Recurrent focal epileptiform discharges caused by cortical injection of iron salts suggests that the development of human posttraumatic epilepsy may depend, in part, on neurochemical alterations induced by the principal metallic ions found in whole blood.
Changes in brain weights during the span of human life: Relation of brain weights to body heights and body weights
Authors: Dekaban Anatole S. , Sadowsky Doris
Published: January 2005

More than 20,000 autopsy reports from several general hospitals were surveyed for the purpose of selecting brains without a pathological lesion that had been weighed in the fresh condition. From this number, 2,773 males and 1,963 females were chosen for whom body weight, body height, and cause of death had been recorded. The data were segregated into 23 age groups ranging from birth to 86+ years and subjected to statistical evaluation.

Overall, the brain weights in males were greater than in females by 9.8%. The largest increases in brain weights in both sexes occurred during the first 3 years of life, when the value quadruples over that at birth, while during the subsequent 15 years the brain weight barely quintuples over that at birth. Progressive decline in brain weight begins at about 45 to 50 years of age and reaches its lowest values after age 86 years, by which time the mean brain weight has decreased by about 11% relative to the maximum brain weight attained in young adults (about 19 years of age). Computed regression lines for brain weights versus body heights and body weights and for ratios of brain weights to body heights and weights versus age groups show clearly differential rates of change in brain weights which are less affected by sex.
Inheritance of the enzyme defect in a new hexosaminidase deficiency disease
Authors: Johnson William G. , Chutorian Abe M.
Published: January 2005

A new form of hexosaminidase deficiency disease is characterized clinically by mild, juvenile‐onset, very slowly progressive cerebellar ataxia with macular cherry‐red spots and absence of other findings. Biochemically there is striking hexosaminidase deficiency in serum, leukocytes, and fibroblasts. Hexosaminidase B appears absent, but hexosaminidase A–like and S–like activity is present on strach‐gel electrophoresis.

We studied hexosaminidase in leukocytes and serum from members of an affected patient's family and traced the enzyme defect through four generations. Leukocyte heat‐stabile hexosaminidase in obligate and presumptive carriers was depressed both in specific activity (nanomoles per milligram of protein per hour) and as a percentage of total hexosaminidase. The carrier state was expressed in serum, but overlap with controls made this test unreliable. The similarity of these carriers to carriers of Sandhoff disease suggests that the disorders may be closely related, perhaps as allelic mutations of the hexosaminidase β subunit. Those involved with screening for Tay‐Sachs disease should be aware that persons with an increased percentage of hexosaminidase A—that is, a decreased heat‐stabile fraction—may be carriers of hexosaminidase deficiency diseases.
Venous angioma of the optic chiasm
Authors: Fermaglich Joseph , Kattah Jorge , Manz Herbert
Published: January 2005

A 30‐year‐old man presented with decreased vision in the right eye of three weeks' duration. Examination indicated a chiasmal syndrome and evidence of subarachnoid hemorrhage. CAT scan showed a large suprasellar mass. Surgical intervention confirmed the presence of a globular lesion filled with blood clots involving mainly the right side of the optic chiasm. Following removal of the clots, the chiasm regained its shape and anatomical landmarks. Biopsy of the mass demonstrated a venous angioma located within the optic chiasm as well as recent and old bleeding. Following surgery the patient did well and recovered vision in the right eye, though a left homonymous hemianopia persisted.
Electron microscopic studies of cerebrospinal fluid sediment in demyelinating disease
Authors: Herndon Robert M. , Kasckow John
Published: January 2005

Cerebrospinal fluid specimens from 31 patients with multiple sclerosis (MS) and 2 with progressive multifocal leukoencephalopathy (PML) were subjected to ultracentrifugation, and the resulting pellets were examined in an electron microscope. Cell types seen in the pellets included lymphocytes, occasional plasma cells, polymorphonuclear leukocytes, monocytes, eosinophils, lipid‐laden macrophages, and fibroblasts. The most interesting noncellular elements were extracellular myelin fragments, recognizable by their characteristic alternation of major dense lines and intraperiod lines. Myelin fragments were seen in the CSF from 7 of 9 patients with MS in exacerbation involving areas other than the optic nerve. These fragments were not observed in 4 specimens from patients with acute attacks manifested by optic neuritis. Myelin fragments were present in 1 of the 2 patients with PML. These observations indicate that a portion of the myelin destruction seen in MS and PML occurs extracellularly, with release of myelin fragments and degradation products into the CSF.
Cerebrospinal fluid findings in asymptomatic patients with reactive serum fluoresecent treponemal antibody absorption tests
Authors: Traviesa Daniel C. , Prystowsky Stephen D. , Nelson Barbara J. , Johnson Kenneth P. , Clavan Barbara S.
Published: January 2005

It is common to examine the cerebrospinal fluid in untreated or inadequately treated asymptomatic patients with a reactive serum fluorescent treponemal antibody absorption (FTA‐ABS) test before initiating antibiotic therapy for syphilis. This prospective study evaluated the usefulness of such examination. Four hundred thirty‐two patients over 40 years old, reporting for annual physical examination, had a serum FTA‐ABS test. Thirty‐seven (8.6%) patients and 2 of 4 spouses were reactive repeatedly. Of the 39 patients with reactive tests, 7 had a history of pencillin therapy for syphilis, 5 had received heavy metal therapy, and 27 had no history of syphilis. These 39 patients had a neurological examination, serum VDRL, Treponema pallidum immobilization (TPI), and repeat FTA‐ABS tests by two other laboratories. The TPI test was reactive in 30 (77%). Four had nonspecific neurological signs. Routine CSF examination (cells, total protein, VDRL, glucose, IgG%) on 30 patients with a history of inadequate treatment had a low diagnostic yield. Two patients had an unexplained total protein elevation (57 and 61 mg/dl) and 1 had a mildly increased IgG% (15%). All cell counts, VDRL tests, and glucose levels were normal. Agarose electrophoresis demonstrated one or more CSF immunoglobulin bands in 10 (36%) of 28 patients, possibly representing an immunological marker of past or latent central nervous system infection.
Juvenile amaurotic idiocy (neuronal ceroid lipofuscinosis) and lymphocyte fingerprint profiles
Authors: Baumann Robert J. , Markesbery William R.
Published: January 2005

Lymphocytes from 3 children with a form of juvenile amaurotic idiocy (characterized by retinal blindness, progressive dementia, and extrapyramidal motor disturbance) were studied by electron microscopy. Numerous fingerprint profiles (FP) were found in vacuolated lymphocytes from all 3 patients and an asymptomatic younger sibling of 1 patient who subsequently became symptomatic. We propose that the combination of this clinical picture and vacuolated lymphocytes with FP is sufficiently distinctive for clinical and research purposes until the biochemical defect is discovered. Wider utilization of ultrastructural study of lymphocytes should increase the number of children diagnosed and allow detection of asymptomatic patients.
Recovery from primary pontine hemorrhage
Authors: Payne Harlan A. , Maravilla Kenneth R. , Levinstone Alan , Heuter John , Tindall RichardS. A.
Published: January 2005

A patient with primary pontine hemorrhage showed typical ocular bobbing with only mild alteration of mental status. Serial CT scans showed resolution of the hemorrhage and correlated with clinical improvement.
Neurotoxicity of intrathecal gentamicin: A case report and experimental study
Authors: Watanabe Itaru , Hodges Glenn R. , Dworzack David L. , Kepes John J. , Duensing George F.
Published: January 2005

Distinctive lesions occurred in the brainstem of a 59‐year‐old patient who had had recent Pseudomonas aeruginosa meningitis treated with parenteral and intrathecal gentamicin sulfate. The lesions were multiple, minute, and discrete, and were characterized by loss of axons, spongiosis, axonal swelling with frequent calcification, loss of astroglia and oligodendroglia, and slight inflammatory response. These lesions were restricted to the myelinated fiber bundles of the pons and mesencephalon.

Because similar lesions can occur with other intrathecally administered medications and emboli to the brain, an experimental study in rabbits was done. Similar lesions were produced in normal adult rabbits after a single intracisternal injection of gentamicin sulfate with or without preservative at doses equivalent to 50 and 100 times the human therapeutic dose. Lesions were not seen after injection of normal saline, preservative, or gentamicin sulfate with preservative at doses equivalent to 1 and 10 times the human therapeutic dose. A direct relationship was observed between the cerebrospinal fluid concentrations of gentamicin, brain tissue concentrations of gentamicin, and occurrence of the lesions.
Multiple sclerosis in the faroe islands: I. Clinical and epidemiological features
Authors: Kurtzke John F. , Hyllested Kay
Published: January 2005

Intensive search over the past five years for all cases of multiple sclerosis (MS) on the Faroe Islands since about 1920 has revealed 25 cases among native‐born resident Faroese up to 1977. All but 1 patient had clinical onset of MS between 1943 and 1960; 1 case began in 1970. Four cases of MS in Faroese with prolonged foreign residence and 5 among Danish‐born Faroese were excluded.

The 24 included cases with onset between 1943 and 1960 meet all critèria for a point‐source epidemic. The median year of onset was 1949. The cumulative risk of MS for Faroese in 1940 was 8.7 per 10,000. All 14 early‐onset cases (1943 to 1949) were in patients 11 to 45 years old in 1940; all but 2 late‐onset cases (1952 to 1960) were in persons aged up to 10 years old in 1940. British troops occupied the Faroes in large numbers for five years beginning in April, 1940. During the war, all but 3 patients resided in locations where the troops were stationed, and these 3 also had direct contact with the British.

We conclude that there was an epidemic of MS on the Faroes and that the disease was probably introduced by the British troops (or their baggage). If so, then MS on the Faroes is a transmissible disease, most likely infectious; but only about 1 in 500 of the exposed individuals were clinically affected.
Clinical and biochemical aspects of trichopoliodystrophy
Authors: Grover Warren D. , Johnson Waine C. , Henkin Robert I.
Published: January 2005

The clinical and biochemical evaluation of 6 patients with trichopoliodystrophy indicates that the disease process can begin in utero and is related to a selective abnormality in copper metabolism. Examination of 2 infants on the first day of life revealed abnormal neurological signs, a characteristic hair abnormality, and elevated levels of copper and ceruloplasmin. Decreased hepatic copper levels and increased urinary copper excretion were documented during the first week. The 2 neonates demonstrated a progressive decrease in blood copper levels in the first month of life. Four infants identified at ages 2 to 11 months had low values for blood copper and ceruloplasmin. All infants had progressive neurological dysfunction, and 4 of the 6 died at ages ranging form 2 1/2 months to 5 1/2 years. Parenteral copper therapy achieved normal blood and hepatic copper levels in 1 patient, but the copper values in the cerebral cortex and white matter were significantly decreased compared to control specimens.
Postvaccinial encephalomyelitis without cutaneous vaccination reaction
Authors: Rockoff A. , Spigland I. , Lorenstein B. , Rose A. L.
Published: January 2005

A 5‐year‐old girl developed a progressive febrile neurological illness consisting of right focal seizures, right hemiparesis, and stupor evolving over a period of six weeks. During the month preceding the onset of her illness she had received two apparently unsuccessful smallpox vaccinations without a skin lesion. Elevation of cerebrospinal fluid gamma globulin and findings on brain biopsy were consistent with postinfectious encephalitis, and a simultaneous increase in serum vacinia antibody titer suggested that the illness was postvaccinial encephalitis. Clinically, the child developed a severe extrapyramidal movement disorder during the acute phase followed by nine months of stuporous unresponsiveness, yet subsequently made a substantial recovery.
Endoneurial lipid composition of normal human sural nerve
Authors: Brown Mark J. , Iwamori Masao , Kishimoto Yasuo , Ostroff Stephen M. , Moser Hugo W. , Asbury Arthur K.
Published: January 2005

The endoneurial lipid composition was determined in 7 human sural nerves from subjects without known neurological disease. The nerves were morphologically normal, with myelinated fiber density of 7,710 ± 1,210/mm2 (mean ± SD). Endoneurium contained 30.7 ± 1.5 mg total lipid per 100 mg of dry weight. Cholesterol was the predominant lipid (9.1 ± 1.0 mg). Cholesterol ester was present only in very small quantities (0.2 ± 0.1 mg). Cerebroside concentration was 4.5 ± 0.3 mg, and sulfatide was 1.5 ± 0.3 mg. The most abundant phospholipid was sphingomyelin (4.3 ± 0.3 mg), followed by phosphatidylethanolamine (4.1 ± 0.2 mg), phosphatidylcholine (3.8 ± 0.2 mg), and phosphatidylinositol‐phosphatidylserine (1.7 ± 0.2 mg). Eighty‐one percent of cerebroside nonhydroxy fatty acids were long chain (C20 to C26), and 67% were saturated. Technological advances now permit microchemical lipid analysis of human sural nerve biopsies in cases of neuropathy. When compared with our normative data, such studies may further the understanding of peripheral nerve disorders.
Atrial myxoma with intellectual decline and cerebral growths on CT scan
Authors: Frank Robert A. , Shalen Philip R. , Harvey Danny G. , Berg Leonard , Ferguson Thomas B. , Schwartz Henry G.
Published: January 2005

A woman with left atrial myxoma had progressive disturbance of intellectual function, headache, and prominent constitutional symptoms. The absence of stroke is noteworthy. Multiple high‐density lesions with contrast enhancement were seen by CT scan, suggesting metastatic neoplasms, hematomas, or hemorrhagic infarcts. Serial study showed progressive enlargement of the lesions. The only cerebral lesion examined histologically proved to be an organizing hematoma, originating most likely from adjacent small arteries found to be plugged by embolic myxoma.
A hypothetical explanation of saccadic oscillations
Authors: Zee David S. , Robinson David A.
Published: January 2005

Eye movements in a patient with saccadic oscillations (ocular flutter) were recorded and analyzed. Findings were related to recent microelectrode studies in the monkey pontine reticular formation which have identified three types of premotor neurons related to saccadic eye movements: burst, tonic, and pause cells. We incorporated these cell types into a hypothetical circuit that generates saccades by rapidly driving the eye to a designated orbital position rather than preprogramming a distance for movement.

Physiological measurements suggest that this neural network is unstable and that the burst neurons must be tonically inhibited to prevent saccadic oscillations during periods of fixation. Pause cells, which discharge tonically except during saccades, when they pause, appear to inhibit burst cells and prevent such saccadic oscillations. Analysis of our patient's behavior indicates that many types of saccadic oscillations can be explained and classified by assuming an abnormality of pause cell control over saccadic burst neurons.
Degeneration of the cerebellar system in X‐chromosome—linked copper malabsorption
Authors: Iwata Makoto , Hirano Asao , French Joseph H.
Published: January 2005

Examination of the cerebellar system of 5 autopsied patients with X‐chromosome–linked copper malabsorption led to the discovery that among cerebellar afferent systems, only the dorsal spinocerebellar tract showed consistent degenerative changes. Cerebellar cortical lesions comprised granule cell loss and Purkinje cell abnormalities that included nuclear and cytoplasmic degenerative changes with cell loss and deficient dendritic arborization as well as the presence of somatic sprouts. Perisomatic baskets were often absent in the area of sprouting Purkinje cells. Cerebellar cortical topographical analysis revealed relative sparing of the caudal portion of the cerebellum. The nodulus was intact in all patients. The dentate nucleus and the superior cerebellar peduncle were preserved. The red nucleus was consistently degenerated.
Patterns of incidence of brain tumors in children
Authors: Gold Ellen B. , Gordis Leon
Published: January 2005

A population‐based community‐wide study of the incidence of brain tumors in children from 1960 through 1974 was conducted in the Baltimore area. Incidence rates in boys declined from 1960 to 1974 and were higher in whites than in blacks in all age groups except children under 5 years. A peak in incidence rates was observed in white 5‐to 9‐year‐olds and in blacks from 0 to 4 years of age. In addition, incidence rates tended to be higher in boys than in girls at all ages except at puberty—the 10‐ to 14‐year‐old age group—when rates in girls exceeded those in boys. The findings suggest that both host and environmental factors may be involved in the cause of brain tumors in children.
Familial arteriovenous malformation
Authors: Snead O. Carter , Acker James D. , Morawetz Richard
Published: January 2005

A family is reported in which 3 siblings, 2 with the same and the third with a different father, were demonstrated to have cerebral arteriovenous malformations.
Metals in spinal cord tissue of patients dying of motor neuron disease
Authors: Kurlander Harold M. , Patten Bernard M.
Published: January 2005

To evaluate the role of toxic metals in causing motor neuron disease (MND), we used a photon‐excited, energydispersive x‐ray analytical system to measure the metal content of spinal ventral horn tissue. Specimens were taken from the cervical and lumbar enlargements of 7 patients who died of MND and the results compared with those found in 12 control patients. Anterior horn lead levels were elevated in MND patients compred to controls (mean, 40.7 μg/gm versus 14.6 μg/gm; p <0.05) and lead levels correlate with the duration of illness (r = +0.84, p < 0.05). Only 2 MND patients had detectable manganese levels (72.3 and 132.2 μg/gm) whereas 1 control had detectable manganese (14.3 μg/gm). One MND patient had 244 μg/gm selenium, but 3 controls had levels of 180, 58, and 62. Patients with the histories of greatest environmental exposure to metals during life exhibited the highest tissue levels of metals after death; despite chelation therapy for about a year, high levels remained in their tissue.
Evaluation of the insulin receptor in myotonic dystrophy
Authors: Festoff B. W. , Moore W. V.
Published: January 2005

Only 2 of 6 patients with myotonic dystrophy had glucose intolerance and hyperinsulinemia. All, however, had markedly reduced insulin binding to specific receptors on circulating monocytes. A significant difference in receptor affinity for insulin was observed, but the difference in affinity was not as marked as that in the amount of 125I‐insulin bound per cell, which was sixfold greater in control cells. No evidence for a circulating factor competing with insulin for binding sites could be demonstrated. These alterations in binding did not directly correlate with glucose intolerance or hyperinsulinemia and are consistent with a postulated generalized membrane defect. Study of the insulin receptor in this disorder may uncover regulatory mechanisms in normal and pathological conditions, including myotonic dystrophy.
Failure of chronic dorsal column stimulation in multiple sclerosis
Authors: Rosen James A. , Barsoum Adib H.
Published: January 2005

In view of the favorable claims of Cook and others, an open trial of chronic dorsal column stimulation was undertaken in 9 patients who had moderately advanced multiple sclerosis (MS) but were still ambulatory. No objective improvement was noted by either author during the postoperative observation period of 6 to 37 months; the occasional subjective improvement was ascribed to placebo effect. This procedure can no longer be recommended for MS patients.
Lorazepam in status epilepticus
Authors: Walker Jonathan E. , Homan Richard W. , Vasko Michael R. , Crawford Isaac L. , Bell Rodney D. , Tasker William G.
Published: January 2005

Lorazepam, a dichloro‐3‐hydroxy‐1,4‐benzodiazepine, has been shown to be a potent anticonvulsant in animal models of epilsepsy and has minimal depressant effects on respiration and circulation in humans. The effects of this compound were studied in status epilepticus. Twenty‐five patients were given intravenous lorazepam during status epilepticus of varying cause. Four or 8 mg of the drug controlled status in 22 of the 25 patients. Although single seizures recurred in 5 of the 22 patients, none experienced recurrence of status during a prolonged follow‐up period. Transient respiratory arrest occurred in 1 patient, but no other significant complications were observed. Studies of plasma drug levels suggest that most patients attain good seizure control at concentrations between 30 and 100 ng per milliliter. Clinical observations indicate that repetitive injections are not required for continuing control of seizures in patients whose seizures are initially controlled. Lorazepam appears to be an effective and safe drug for treatment of status epilepticus, with a duration of control longer than that achieved with diazepam.
Infantile neuroaxonal dystrophy and giant axonal neuropathy: Are they related?
Authors: Begeer J. H. , Houthoff H. J. , Van Weerden T. W. , De Groot C. J. , Blaauw E. H. , Le Coultre R.
Published: January 2005

Light and electron microscopic findings from two sural nerve biopsies obtained at a one‐year interval from a patient with the clinical features of Seitelberger's disease are described. Ballooned axons with accumulations of membranous profiles, vesicles, mitochondria, and a homogeneous center were present, and there were masses of 90 A filaments in endothelial, endoneurial, perineurial, and Schwann cells. These pathological alterations were less prominent in the second nerve biopsy, which showed a more pronounced decrease in myelinated fibers. The case shows that a generalized increase of 90 A filaments in structures of the peripheral nervous system is not a phenomenon exclusively occurring in patients with giant axonal neuropathy and, furthermore, that it may be a transitory feature.
Skin fibroblast microtubular network in Alzheimer disease
Authors: Harper C. G. , Buck D. , Gonatas N. K. , Guilbert B. , Avrameas S.
Published: January 2005

A preliminary observation from another laboratory recently suggested that a systemic microtubular defect may exist in Alzheimer disease. To investigate this hypothesis, we obtained skin biopsies from 4 patients with Alzheimer disease and 2 age‐matched controls Fibroblast cultures were established and the tubulin networks examined using immunoadsorbent purified antitubulin antibody and the indirect immunoperoxidase technique as well as electron microscopy. The cells were also examined after treatment with vinblastine and at senescence.

The microtubular network appeared as delicate fibers radiating From the perinuclear region toward the cytoplasmic margins. No differences were recognized between the controls and the fibroblast cultures from patients with Alzheimer disease. Microtubules were not visualized following incubation with vinblastine, but positively staining intracytoplasmic paracrystalline inclusions were noted. No abnormalities of microtubules were recognized in the electron microscopic examinations. These findings suggest that the neurofibrillary neuronal degeneration of Alzheimer disease is not a manifestation of a systemic disorder of the microtubular network.
Multisystem triglyceride storage disorder with impaired long‐chain fatty acid oxidation
Authors: Angelini Corrado , Philippart Michel , Borrone Carla , Bresolin Nereo , Cantini Marcello , Lucke Sandra
Published: January 2005

A five‐year‐old girl presented with congenital ichthyosis, hepatosplenomegaly, vacuolized granulocytes (Jordans' anomaly), and myopathy. Pathological, ultrastructural, and biochemical studies revealed nonlysosomal, multisystemic triglyceride storage. The cultured fibroblasts had increased uptake but decreased oxidation of labeled oleate. The patient failed to produce ketone bodies on fasting. A medium‐chain triglyceride diet reversed the hepatomegaly. These studies are all consistent with a partial defect in the catabolism of long‐chain fatty acids. This newly identified syndrome is presumably transmitted as an autosomal recessive trait.
Familial stroke syndrome associated with mitral valve prolapse
Authors: Rice G. P. A. , Boughner D. R. , Stiller C. , Ebers G. C.
Published: January 2005

In a 27‐member family, 8 individuals had mitral valve prolapse (MVP) by echocardiography. Four of them suffered a total of eight cerebral ischemic events in the first four decades of life. In none of these patients could risk factors for stroke, other than MVP, be found. Stroke did not occur in individuals free of MVP. This report draws attention to a previously unrecognized cause of familial stroke which strengthens the relationship between MVP and premature stroke.
Cold face test in the assessment of trigeminal‐brainstem‐ vagal function in humans
Authors: Khurana Ramesh K. , Watabiki Sadakiyo , Hebel J. R. , Toro Rodrigo , Nelson Erland
Published: January 2005

Study of the reflex heart rate response in humans to apneic facial immersion (simulated diving) and its modifications showed that bradycardia caused by simple application of cold compresses to the face (cold face test) correlated well with that produced by the simulated diving reflex. Bilateral application of cold stimulus to the individual divisions of the trigeminal nerve revealed the ophthalmic division to be the most sensitive pathway for this reflex. The cold face test was standardized in 50 normal individuals and further validated in 10 patients by comparison with the simulated diving reflex, the Valsalva maneuver, and administration of atropine. Patients with diabetes mellitus, brainstem stroke, multiple sclerosis, or Shy‐Drager syndrome developed less than normal bradycardia or minimal tachycardia in response to the cold facial stimulus. The cold face test is a novel, simple, safe, and economical test of the integrity of trigeminal‐brainstem‐vagal reflex pathways, can be utilized practically to assess vagal and brainstem dysfunctions, and has the special advantage of being applicable even in an uncooperative or comatose patient.
The multisensory physiological and pathological vertigo syndromes
Authors: Brandt Thomas , Daroff Robert B.
Published: January 2005

Vertigo occurs with either physiological stimulation or pathological dysfunction of any of the three stabilizing sensory systems: vestibular, visual, and somatosensory. The physiological syndromes, induced by intersensory or intrasensory mismatches, include motion sickness and space sickness as well as height, visual, somatosensory, auditory, head‐extension, and bending‐over vertigo. This review emphasizes the relationship between these physilological forms of vertigo and the pathological clinical vertigo syndromes.
Pure axonal neuropathy: Nerve xenografts and clinicopathological study of a family with peripheral neuropathy, hereditary ataxia, focal necrotizing encephalopathy, and spongy degeneration of brain
Authors: Appenzeller O. , Kornfeld Mario , Atkinson Ruth
Published: January 2005

Three family members had the unusual combination of severe peripheral neuropathy, atypical hereditary ataxia, spongy degeneration of cerebral hemispheres, and cerebellar and brainstem foci of necrotizing encephalopathy, proved pathologically in one autopsied case. A sural nerve from a patient, devoid of myelinated fibers, was transplanted into thymectomized, lethally irradiated, and bone marrow reconstituted adult mice. A normal number of myelinated fibers was present in the grafts eleven weeks later. Eighteen weeks after grafting, mice were reconstituted with syngeneic thymus to return immunocompetence. Schwann cells in the graft were rejected and axons became totally denuded of myelin after thymus reconstitution. The peripheral neuropathy is thus due to axonal disease since human Schwann cells were capable of normally myelinating regenerating mouse axons. A puzzling feature after rejection was the absence of myelin debris containing macrophages in the grafts. It is suggested that part of the rejection process in this model is mediated by antibody rather than by cellular mechanisms.
Neuropathy and mitochondrial myopathy
Authors: Peyronnard Jean‐Marie , Charron Louise , Bellavance Andrè , Marchand Luc
Published: January 2005

It has been stated that peripheral neuropathy can be a feature of so‐called ophthalmoplegia‐ plus syndrome, but to date one case of hypertrophic neuropathy has been reported. This study is concerned with the clinical, electrophysiology, and pathological observations made in a 37‐year‐old man with progressive extyernal ophthalmcmetric studi9ees of the sural nerve revealed a marked loss of large myelinated fibers and an occasional degenerating axon. Residual fibers had disproportionately thin myelin sheaths in reelation to axon calibedrs. In contrast to the muscle biopsy findings, no mitochondrial paracrystaline inclusions were observed in the nerve. However, the number of mitochondria per square micron of Schwann cell cytoplasm was wlevated when compared with values obtained from normal subjects and a patient with a chronic neuropathy. These findings may indicate an alteration of mitochondrial function common to muscle fibers and Schwann cels which, in nerves, could lead to axon loss and abnormality of myelination.
Parkinson disease, dementia, and alzheimer disease: Clinicopathological correlations
Authors: Boller Fraņois , Mizutani Tomohiko , Roessmann Uros , Gambetti Pierluigi
Published: January 2005

Clinical records and neuropathological specimens from 36 patients with autopsy‐demonstrated idiopathic Parkinson disease (PD) were reviewed independently and the results compared. Nine (31%) of the 29 patients with adequate clinical data had severe dementia and 7 (24%) had mild dementia. The cerebral cortex showed senile plaques and fibrillary tangles in 15 of the 36 patients (42%). These changes were found in all 9 patients with severe dementia, in 3 of the 7 with mild dementia, and in 3 of the 13 patients with normal mental status.

The prevalence of pathologically established Alzheimer changes and dementia among the patients with PD (33%) was over six times that found in an age‐matched population (5.1%). Survival after the onset of PD with Alzheimer disease was shorter than in PD without Alzheimer disease.
Acute parotitis and hyperamylasemia following whole‐brain radiation therapy
Authors: Cairncross J. Gregory , Salmon Jane , Kim Jae‐Ho , Posner Jerome B.
Published: January 2005

Parotitis, an infrequent, previously unreported complication of whole‐brain radiation therapy, was observed in 4 patients. The acute symptoms, which include fever, dry mouth, pain, swelling, and tenderness, are accompanied by hyperamylasemia. Among 10 patients receiving whole‐brain irradiation, 8 hac serum amylase elevations without symptoms. Both acute parotitis and asymptomatic hyperamylasemia result from irradiation of the parotid glands.
The cubital tunnel syndrome: Treatment with simple decompression
Authors: Miller Robert G. , Hummel Errett E.
Published: January 2005

Twelve patients with progressive cubital tunnel syndrome were treated with simple decompression of the ulnar nerve. The clinical and electrophysiological responses to treatment are described.
Heart rate changes in convulsive and nonconvulsive neonatal apnea
Authors: Fenichel Gerald M. , Olson Barbara J. , Fitzpatrick Jeri E.
Published: January 2005

One hundred twelve apneic spells of at least 4 seconds' duration were monitored in 15 neonates; 77 episodes were associated with convulsive discharges on the electroencephalogram and 35 were not. Convulsive apnea did not occur in conjunction with bradycardia. Nonconvulsive apnea of less than 20 seconds' duration occurred in the presence of variable heart rates, but nonconvulsive apnea of 20 seconds or longer was nearly always associated with bradycardia.
Lymphocyte responsiveness to acetylcholine receptor in rats with experimental autoimmune myasthenia gravis
Authors: Noguchi Sadako A. , Drachman Daniel B. , Adams Robert N. , Hirsch Robert L.
Published: January 2005

We examined the time course of lymphocyte responsiveness to acetylcholine receptor (AChR) in rats with experimental autoimmune myasthenia gravis (EAMG). Rats were immunized with purified torpedo AChR. At intervals of one to eight weeks later, lymphocytes from the lymph nodes and spleen were cultured with purified torpedo AChR and rat muscle extract containing AChR. Lymphocyte responsiveness (stimulation index) was determined from uptake of 3H‐labeled thymidine by the cultured cells. The response of lymphocytes to torpedo antigen began earlier and rose more rapidly than that to the homologous (rat) antigen. Lymph node cells responded more promptly than spleen lymphocytes. The stimulation indexes peaked at four to six weeks while antibodies to both antigens continued to rise. Delineation of this pattern of lymphocyte responsiveness sheds further light on the pathogenesis of the autoimmune response in EAMG and will be useful in the future design of immunotherapeutic strategies.
Progressive central nervous system deterioration: A complication of advanced chronic lung disease of prematurity
Authors: Ellison Patricia H. , Farina Matthew A.
Published: January 2005

Fifteen premature infants with severe chronic lung disease of prematurity were treated with ventilatory assistance and continuous positive airway pressure for at least 28 days. Fourteen developed progressive central nervous system deterioration following right heart failure and pulmonary hypertension. Two patterns of deterioration were observed: (1) cerebral, with loss of developmental milestones, increasing hypotonia, and eventual EEG deterioration and death; and (2) brainstem, with progressive apnea, bradycardia, or both, and sudden death or irreversible apnea.
Acne fulminans with inflammatory myopathy
Authors: Noseworthy J. H. , Heffernan L. P. , Ross J. B. , Sangalang V. E.
Published: January 2005

An 18‐year‐old man with acne fulminans developed an acute inflammatory myositis, an association not previously reported. The condition resolved with treatment using systemic corticosteroids.
Progressive white matter destruction following irradiation of an extracranial neoplasm
Authors: Rottenberg David A. , Horten Bruce , Kim Jae‐Ho , Posner Jerome B.
Published: January 2005

Although numerous “cures” have been reported following surgical extirpation of symptomatic foci of cerebral radiation necrosis, delayed progressive white matter destruction and neurological deterioration may occur in some patients who survive for prolonged periods after operation. The postoperative appearance on CT scans of hypodensity within heavily irradiated white matter structures at a distance from the initial radionecrotic focus or operative site suggests continuing radiation‐induced tissue injury and a poor prognosis. Anticipated survival as well as administered radiation dose must be taken into account when “safe” radiation thresholds are calculated.
C‐6 Glioma Growth in Rats: Suppression with a β‐Adrenergic Agonist and a Phosphodiesterase Inhibitor
Authors: Chelmicka‐Schorr Ewa , Arnason Barry G. W. , Holshouser Sidney J.
Published: January 2005

Rat C‐6 glioma possesses a β‐adrenergic receptor which, when activated, raises intracellular levels of cyclic adenosine monophosphate (cAMP) in cultured C‐6 glioma cells. The present study shows that growth of C‐6 glioma is suppressed in rats treated with the β‐adrenergic agonist isoproterenol. Addition of papaverine, a cAMP phosphodiesterase inhibitor, to the treatment schedule augments this effect. Pharmacological agents that elevate intracellular cAMP levels may retard the growth of neural tumors in vivo.
Altered protein composition of isolated human cortical neurons in alzheimer disease
Authors: Selkoe Dennis J.
Published: January 2005

To characterize the protein composition of degenerating neurons in Alzheimer disease, enriched fractions of isolated cortical neurons from postmortem Alzheimer brain were compared by ultrastructural and biochemical techniques to neuronal isolates from aged normal controls and from patients with the nonfibrillary degenerative dementia, Huntington disease. Electron microscopy of isolated neurons showed well‐preserved organelles, including nuclear membranes, mitochondria, endoplasmic reticulum, ribosomes, and lipofuscin. Abundant paired helical filaments (PHF) were preserved in affected Alzheimer perikarya. In both sporadic and familial Alzheimer disease, sodium dodecylsulfate‐polyacrylamide gel electrophoresis revealed marked augmentation of a 20,000 mw protein (P20) in neuronal fractions from affected cortex compared to Huntington disease patients and controls. P20 was commonly increased in hippocampal, temporal, and frontal neurons but not in cortex free of PHF. The augmentation of P20 in Alzheimer disease samples appeared to correlate with the presence of PHF‐bearing neurons in the fractions. The increase in P20 in Alzheimer disease was observed in the presence of proteolysis inhibitors, in reverse‐sieved samples, and in whole cortical homogenates. Neurofilament proteins, tubulins, and a 50,000 mw protein showed no changes. In Huntington disease, marked augmentation of a 49,000 mw protein was found in striatal but not cortical homogenates. The relationship of these findings to the biochemistry of brain fibrous proteins and to the molecular pathology of Alzheimer disease is discussed.
Time modulation of spike‐and‐wave activity in generalized epilepsy
Authors: Kellaway Peter , Frost James D. , Crawley James W.
Published: January 2005

Nineteen patients, aged 4 to 44 years, with generalized epilepsy and generalized 3 Hz spike‐and‐wave activity, were monitored for 24 or 36 hours on one to nine occasions to determine the time distribution of ictal and interictal electroencephalographic discharges and the consistency of the time patterns observed. Two patients with a low incidence of spike‐and‐wave activity had a random pattern of occurrence; the other 17 showed one of three types of time modulation. The latter can be explained by the interaction of two modulative processes, one with a period of about 100 minutes and the other of approximately 24 hours. The pattern observed depends on the phase angle of the two cyclic processes. Treatment with ethosuximide altered the pattern of time modulation, and eventually, when the spike‐and‐wave activity was reduced to a low level, the time distribution became random.