bluuebunny/crossref_metadata_2025 huggingface dataset:
Chronic idiopathic anhidrosis
Authors: Low P. A. , Fealey R. D. , Sheps S. G. , Su W. P. D. , Trautmann J. C. , Kuntz N. L.
Published: January 2005

We describe the cases of eight patients with chronic idiopathic anhidrosis. These patients were heat intolerant and became hot, flushed, dizzy, dyspneic, and weak but did not sweat when the ambient temperature was high or when they exercised. Four patients had preganglionic sudomotor lesions and in the remaining 4 the lesion appeared to be postganglionic. The patients did not have orthostatic hypotension, other evidence of generalized autonomic failure, or symptomatic somatic neuropathy. One patient regained thermoregulatory sweat function and no patient's condition progressed to generalized autonomic failure. Chronic idiopathic anhidrosis appears to be distinctly different from other autonomic neuropathies that tend to carry much poorer prognoses.
Buccofacial apraxia without aphasia due to a right parietal lesion
Authors: Kramer Joel H. , Delis Dean C. , Nakada Tsutomu
Published: January 2005

Buccofaciai praxis in right‐handers is thought to be mediated by the left hemisphere. We present the case of a right‐handed man without family history of sinistrality who exhibited a buccofacial apraxia following a unilateral right parietal infarction. Other findings such as intact language, visuospatial impairment, and hemi‐inattention are more characteristic of right posterior lesions.
Influenza B virus model of Reye's syndrome in mice: The effect of aspirin
Authors: Davis Larry E. , Green Carl L. , Wallace Judith M.
Published: January 2005

Aspirin is a possible cofactor with influenza B virus in Reye's syndrome. In an attempt to determine whether single or multiple doses of acetylsalicylic acid (ASA) alter mortality in an influenza B virus mouse model of Reye's syndrome, either ASA (300 to 667 mg/kg) or saline was given intraperitoneally to 3‐week‐old Balb/c mice. Two hours later, one median lethal dose (LD50) of influenza B/Lee virus was given intravenously. Mortality was not significantly increased by the single ASA dose. Blood ASA levels rose as high as 68 mg/dl. The average ASA blood levels in mice 12 hours after receiving the virus plus 667 mg/kg ASA was 22.6 mg/dl, which was significantly higher than the 3.4 mg/dl for mice who received saline plus ASA. No significant increase in mortality occurred in mice given 67 mg/kg ASA twice daily for 7 days and one LD50 dose of virus on day 4. We conclude that a single dose of ASA at up to half the median lethal dose or multiple doses of ASA do not significantly increase mortality in this influenza virus model of Reye's syndrome. However, influenza B virus infection appears to interfere with ASA metabolism.
Alternative single anticonvulsant drug therapy for refractory epilepsy
Authors: Schmidt Dieter , Richter Klaus
Published: January 2005

Fifty‐nine patients with chronic generalized toniclonic or partial seizures refractory to the maximally tolerated daily dosage of single‐drup therapy with carbamazepine, phenytoin, phenobarbital, or primidone subsequently received single‐drug therapy with another one of these primary anticonvulsant drugs. Alternative single‐drug therapy resulted in complete seizure control or more than 75% seizure reduction for 18 patients (31%). Side effects disappeared in 16 patients (27%). Patients with a good therapeutic response often had epilepsy of recent onset. Alternative single‐drug therapy is beneficial for chronic refractory epilepsy and should be considered before a second drug is permanently added.
Glutamate and the pathophysiology of hypoxic–ischemic brain damage
Authors: Rothman Steven M. , Olney John W.
Published: January 2005

Information obtained over the past 25 years indicates that the amino acid glutamate functions as a fast excitatory transmitter in the mammalian brain. Studies completed during the last 15 years have also demonstrated that glutamate is a powerful neurotoxin, capable of killing neurons in the central nervous system when its extracellular concentration is sufficiently high. Recent experiments in a variety of preparations have shown that either blockade of synaptic transmission or the specific antagonism of postsynaptic glutamate receptors greatly diminishes the sensitivity of central neurons to hypoxia and ischemia. These experiments suggest that glutamate plays a key role in ischemic brain damage, and that drugs which decrease the accumulation of glutamate or block its postsynaptic effects may be a rational therapy for stroke.
Phenytoin neuropathy: Structural changes in the sural nerve
Authors: Ramirez José A. , Mendell Jerry R. , Warmolts John R. , Griggs Robert C.
Published: January 2005

Phenytoin has been implicated as a causative agent in peripheral neuropathy, although structural changes in nerve have not been characterized. A 47‐year‐old man was seen with clinical and electrophysiological signs of peripheral neuropathy after 30 years of phenytoin administration. Despite a modest dose of phenytoin (300 mg/day) blood levels were 31 to 38 μg/ml. A sural nerve biopsy showed a loss of large myelinated nerve fibers and a nonrandom clustered distribution of segmental demyelination and remyelination. The latter findings were accompanied by axonal shrinkage. Sixteen months after phenytoin was stopped, the patient's clinical and electrophysiological findings reflected improvement. These data indicate that long‐term phenytoin administration can cause a reversible neuropathy characterized by axonal shrinkage and secondary demyelination.
Cerebral toxoplasmosis complicating the acquired immune deficiency syndrome: Clinical and neuropathological findings in 27 patients
Authors: Navia Bradford A. , Petito Carol K. , Gold Jonathan W. M. , Cho Eun‐Sook , Jordan Barry D. , Price Richard W.
Published: January 2005

We reviewed the clinical, neuroradiological, and serological findings in 27 patients with cerebral toxoplasmosis complicating the acquired immune deficiency syndrome, 19 of whom were also analyzed neuropathologically. The clinical manifestations of this disorder varied, ranging from headache and fever to coma. However, the characteristic presentation included focal neurological symptoms and signs, usually of subacute onset. In addition, two‐thirds of the patients exhibited more generalized cerebral dysfunction with confusion and lethargy. The computed tomographic (CT) scan most commonly revealed ring contrast enhancement, which appeared to correlate best with the histological presence of vascular proliferation and inflammation surrounding the abscesses. However, in 5 patients the CT scan revealed either homogeneous enhancement or no enhancement, and in 3 patients the scans were negative. In general, CT scans underrepresented the number of lesions eventually documented pathologically. Double‐dose contrast administration and preliminary experience with magnetic resonance imaging suggested that these techniques were superior to standard CT scanning in detecting Toxoplasma lesions. All patients were seropositive for IgG antibody against Toxoplasma gondii in blood, both before the onset of illness and at the time of presentation, although titers in some patients were as low as 1:8 and most patients did not exhibit rising titers. Prompt therapy resulted in rapid clinical improvement, documented by CT scan, associated with the development of an organizing tissue response in the host and elimination of free organisms. Response to treatment was sufficiently rapid in most patients to allow a trial of therapy as the favored approach to diagnosis.
Regional distribution of amino acid transmitters in postmortem brains of presenile and senile dementia of Alzheimer type
Authors: Sasaki Hidenao , Muramoto Osamu , Kanazawa Ichiro , Arai Heii , Kosaka Kenji , Iizuka Reiji
Published: January 2005

We measured the concentrations of glutamate, asparate, and γ‐aminobutyrate (GABA) in cortical and subcortical areas by an enzymatic fluorometric method in 9 patients with histologically verified Alzheimer's disease and senile dementia of the Alzheimer type (AD/SDAT), and in 10 age‐matched normal control subjects. When compared with controls, the concentration of glutamate in cortical and subcortical areas of AD/SDAT brains was significantly reduced. The concentrations of aspartate and GABA were also reduced in several areas, but the magnitude of reduction was less than that of glutamate. This apparent reduction of glutamate in postmortem brains might have a bearing on pathophysiological mechanisms in AD/SDAT.
Effect of thymectomy on blood T‐cell subsets in myasthenia gravis
Authors: Cox Adele , Lisak Robert P. , Skolnik Paul , Zweiman Burton
Published: January 2005

We studied peripheral blood mononuclear T‐cell subsets in 23 patients with myasthenia gravis who had undergone thymectomy at least one year before study. An increase in the T4+/T8+ (helper‐inducer/suppressor‐cytotoxic cell) ratio was seen, similar to that previously reported in nonthymectomized patients with myasthenia gravis. Six patients were studied before and after thymectomy and no consistent alteration in the pattern of T‐cell subsets was detected. We were not able to demonstrate a quantitative effect of thymectomy on peripheral blood mononuclear T‐cell subsets in patients with myasthenia gravis.
Somatosympathetic reflex unilateral sweating and pupillary dilatation in a paraplegic man
Authors: Saper Clifford B. , DeMarchena Octavio
Published: January 2005

Sympathetic spinal reflex responses to painful stimuli have been studied in experimental animals, but have rarely been demonstrated in human beings. We report the case of a paraplegic man with a high thoracic sensory level to pain, who developed unilateral pupillodilatation and sweating from the head to the midthoracic region as a response to rib fractures below the sensory level. This regional sympathetic reflex response was similar in many ways to that seen in spinally transected animals. Regional sympathetic reflex responses may provide a clinically useful sign of a painful and perhaps dangerous condition which is located below the analgesic level in spinal cord–injured patients.
Isolation of P2 protein–reactive T‐cell lines from human blood
Authors: Burns James , Krasner L. Jill , Rostami Abdolmohammad , Pleasure David
Published: January 2005

Human T‐cell lines reactive with the peripheral nerve myelin, protein, P2 protein, were isolated from the peripheral blood of 4 normal persons and 1 patient with Guillain‐Barré syndrome. These predominantly helper phenotype T‐cell lines were isolated and maintained in vitro by antigen stimulation followed by culture with interleukin 2. Myelin basic protein–reactive T cells were also isolated in parallel from the same subjects as antigen specificity controls. T cells recognizing myelin basic protein did not respond to P2 protein, nor did P2‐reactive cells respond to myelin basic protein. These findings suggest that a potential for autoimmune reactivity with peripheral nervous system myelin antigens may exist for both normal persons and some patients with neurological disease.
Cortical potentials related to voluntary and passive finger movements recorded from subdural electrodes in humans
Authors: Lee B. I. , Lüders H. , Lesser R. P. , Dinner D. S. , Morris H. H.
Published: January 2005

Movement‐related potentials were recorded from subdural electrodes placed on the precentral and postcentral cortex in 3 patients undergoing operation for intractable epilepsy. With self‐initiated index finger movement, a negative potential of 25 to 50 microvolts in amplitude, preceding onset of the electromyographic activity by 60 to 95 ms (or onset of movement by 150 to 230 ms), was recorded from the hand somatosensory postrolandic area in all 3 patients. A similar potential preceding the movement was recorded from the precentral hand motor area in one subject who was the only patient in whom the precentral electrodes were placed on the hand motor area. Following active and passive movements, a clearly defined positivity (18 to 32 ms after a photometer trigger) that reversed phase across the central fissure was recorded. The premovement potentials are most probably generated by pyramidal tract neurons and motor‐function‐related neurons located in the post‐ and prerolandic areas. The postmovement positivity is most probably due to short‐latency kinesthetic reafferent activation of the posterior bank of the central fissure (equivalent to P2 of the somatosensory evoked potentials).
Neurological manifestations in xeroderma pigmentosum
Authors: Mimaki Takashi , Itoh Naoyuki , Abe Jiro , Tagawa Tetsuzo , Sato Kenji , Yabuuchi Hyakuji , Takebe Hiraku
Published: January 2005

Xeroderma pigmentosum is an unusual neurocutaneous disorder. Recent studies have classified patients with xeroderma pigmentosum into 10 groups by somatic cell hybridization methods. In this report we describe 32 patients with Group A xeroderma pigmentosum, including 1 patient with an atypical case, who were assessed for neurological complications. Of these patients, 17 had microcephaly, 13 short stature, and 21 mental retardation. In patients over 7 years of age, sensorineural deafness and spinocerebellar signs such as nystagmus, dysarthria, tremor, and ataxia were frequently observed; no patients below 7 years of age had such neurological complications. Electroencephalographic studies revealed abnormal slow and low voltage background activity. Two patients had focal abnormal discharges, one of whom developed versive seizures. Cranial computed tomographic scans revealed abnormalities, including ventricular dilatation, cerebral atrophy, cerebellar and brainstem atrophy, and cranial bone thickening. A patient with an atypical case of Group A xeroderma pigmentosum had less skin and neurological involvement, and higher levels of postultraviolet colony‐forming ability and host cell reactivation than did a typical Group A case. It is possible that these less severe cytological findings are responsible for the less severe skin lesions and neurological complications noted clinically.
Primary central nervous system lymphoma related to Epstein‐Barr virus in a patient with acquired immune deficiency syndrome
Authors: Rosenberg Neil L. , Hochberg Fred H. , Miller George , Kleinschmidt‐DeMasters B. K.
Published: January 2005

The study of a patient suggested a relationship between Epstein‐Barr virus infection and primary lymphoma of the central nervous system in the acquired immune deficiency syndrome. Deoxyribonucleic acid preparations from tumor tissue contained 30 to 100 copies of Epstein‐Barr virus genome per cell when hybridized with a probe consisting of the Bam‐HI K fragment of Epstein‐Barr virus strain FF41. This hybridization study suggests that induction of this patient's central nervous system lymphoma was related to Epstein‐Barr virus infection.
Phenytoin: Mechanisms of its anticonvulsant action
Authors: Yaari Yoel , Selzer Michael E. , Pincus Jonathan H.
Published: January 2005

Phenytoin is a major anticonvulsant drug that is very effective in controlling a wide variety of seizure disorders while impairing neurological function little, if at all. Early work suggested the hypothesis that the drug's effects were due to a selective block of high‐frequency neuronal activity. This theory is reevaluated in the light of accumulated observations on the effects of phenytoin in many neuronal and synaptic preparations. Most of these observations can be explained by a use‐ and frequency‐dependent suppression of the sodium action potential by phenytoin, with a consequent filtering out of sustained high‐frequency neuronal discharges and synaptic activity. The molecular mechanism for this is a voltage‐dependent blockade of membrane sodium channels responsible for the action potential. Through this action, phenytoin obstructs the positive feedback that underlies the development of maximal seizure activity, while normal brain activity, proceeding at lower neuronal firing rates, is spared its depressant action. Other mechanisms of action that may contribute to the drug's efficacy and selectivity are also discussed.
The incidence and prevalence of multiple sclerosis in Newfoundland and Labrador, 1960–1984
Authors: Pryse‐Phillips W. E. M.
Published: January 2005

A study of the incidence and prevalence of multiple sclerosis on the island of Newfoundland between 1960 and 1984 has yielded a current overall prevalence rate of 55.2 per 100,000, but wide variation is noted in the rates for different parts of the island (range, 15.9 to 105.0 per 100,000). The annual incidence rates for St. John's and the Avalon region of Newfoundland show cyclical variation on an approximate five‐ or six‐year cycle. Suggestive evidence for a temporal link between varying incidence rates in these areas and recurrent local outbreaks of canine distemper was obtained.
Epitopes of immunoreactive myelin basic protein in human cerebrospinal fluid
Authors: Whitaker John N. , Gupta Manjula , Smith Olive F.
Published: January 2005

To define in more detail the features of the immunoreactive myelin basic protein (MBP) present in cerebrospinal fluid (CSF) of humans following acute injury to central nervous system myelin, the epitopes of MBP recognized by three different antisera, each capable of detecting immunoreactive MBP in CSF, were examined. All three antisera reacted well with human MBP and human MBP peptide 45–89. Only in radioimmunoassays in which the MBP peptide 45–89 served as the radioligand could clearly elevated values of immunoreactive MBP be measured in CSF specimens from 5 patients with multiple sclerosis during or immediately after an exacerbation. The two antisera that reacted well with MBP peptide 80–89 resulted in higher levels of immunoreactive MBP measured in CSF. An epitope present in human MBP peptide 80–89 but sharing a conformation with both MBP and MBP peptide 45–89 is present in CSF following acute central nervous system myelin damage in multiple sclerosis.
HLA study of Japanese patients with Crutzfeldt‐Jakob disease: Significant association with HLA‐DQw3
Authors: Kuroda Yasuo , Kaneoka Hidetoshi , Shibasaki Hiroshi , Kume Shuji , Yamaguchi Masaya
Published: January 2005

To demonstrate genetic susceptibility to Creutzfeldt‐Jakob disease (CJD), 10 Japanese patients with pathologically proved CJD and 6 patients with clinically definite CJD were tissue typed for 48 class I and 14 class II human leukocyte antigen (HLA) specificities. The patients were scattered throughout Japan and there were no consanguineous relationships. Among 62 HLA specificities, HLA‐DRw53 and DQw3 were found in all pathologically proved CJD cases. When the frequencies of the HLA antigens in the 16 CJD cases were compared to those of normal Japanese, a highly significant (p < 0.005) increase was found for HLA‐DQw3 in the CJD group. The present findings provide evidence for genetic control of susceptibility to CJD in humans.
Atypical presentation and neuropathological studies in 3‐hydroxy‐3‐methylglutaryl–CoA lyase deficiency
Authors: Zoghbi Huda Y. , Spence J. Edward , Beaudet Arthur L. , O'Brien William E. , Goodman Clay J. , Gibson Kenneth M.
Published: January 2005

A 6½‐month‐old male offspring of consanguineous Egyptian parents was first seen because of fever, somnolence, vomiting, right focal motor seizures, right hemiparesis, elevated transaminase levels, hyperammonemia, and acidosis. A computed tomographic scan of the head suggested swelling of the left cerebral hemisphere, and an electroencephalogram indicated left frontotemporal abnormalities, but brain biopsy demonstrated diffuse white matter spongiosis and gliosis. Subsequently, urine organic acid analysis and enzyme assays were diagnostic of 3‐hydroxy‐3‐methylglutaryl–coenzyme A lyase deficiency.
Localization and effects of neuropeptide Y, vasoactive intestinal polypeptide, substance P, and calcitonin gene–related peptide in human temporal arteries
Authors: Jansen Inger , Uddman Rolf , Hocherman Max , Ekman Rolf , Jensen Kai , Olesen Jes , Srienholm Pia , Edvinsson Lars
Published: January 2005

Nerve fibers containing neuropeptide Y (NPY), vasoactive intestinal polypeptide (VIP), substance P (SP), and calcitonin gene–related peptide (CGRP) were seen in the adventitia or at the adventitia–media border of the human temporal artery. Pharmacological experiments on isolated temporal artery segments revealed that NPY potentiated the vasoconstrictor responses to noradrenaline, but had no vasoconstrictor ability or only a small vasoconstrictor ability per se. VIP, peptide histidine methionine 27 (PHM‐27), SP, neurokinin A (NKA), and CGRP potently relaxed vessels precontracted by prostaglandin F2α, the relative potency being CGRP > SP > NKA  VIP  PHM‐27. The amount of relaxation varied between 67 and 91% of the prostaglandin F2α–induced contraction. The peptide effects were not antagonized by classic adrenergic or cholinergic blockers, suggesting interactions via separate receptor sites.
Acute high‐dose methotrexate neurotoxicity in the rat
Authors: Phillips Peter C. , Thaler Howard T. , Berger Claudia A. , Fleisher Martin , Wellner Daniel , Allen Jeffrey C. , Rottenberg David A.
Published: January 2005

Intravenous high‐dose methotrexate chemotherapy may produce acute, subacute, or chronic neurotoxicity in patients with cancer. Acute encephalopathies following high‐dose methotrexate treatment are recognized with increasing frequency. This study describes a model of acute high‐dose methotrexate neurotoxicity in the rat characterized by a profound dose‐dependent depression of cerebral glucose metabolism in association with behavioral and electroencephalographc abnormalities. Alterations in the amino acid profile, similar to those described in cancer patients after high‐dose methotrexate treatment, were observed in the absence of biochemical evidence of systemic organ toxicity. This model facilitates the study of the biochemical mechanisms of antifolate neurotoxicity in humans and permits the evaluation of potential therapeutic interventions.
Subclasses of event‐related potentials: Response‐locked and stimulus‐locked components
Authors: Goodin Douglas S. , Aminoff Michael J. , Mantle Mary M.
Published: January 2005

The manner in which information processing proceeds and the relationship of the components of the cerebral “event‐related” evoked potential (ERP) to the presumed stages of such processing are uncertain. We studied these relationships by recording long‐latency evoked potentials from the scalp in conjunction with electromyographic (EMG) recording in experiments in which subjects were required to make a motor response to one of two auditory stimuli. The cerebral response was averaged from the tone onset (stimulus‐synchronized average) and the EMG onset (response‐synchronized average). We found that N2 was quite distinct from the other ERP components (P165 and P3) in its lack of a close relationship to the motor response required in a sensory discrimination task. This suggests that, contrary to general belief, the P165‐N2‐P3 components of the ERP are not generated in an orderly sequence, but rather are generated separately and are possibly related to different cognitive processes.
Cell surface markers for diagnosis of central nervous system involvement in lymphoproliferative diseases
Authors: Ernerudh Jan , Olsson Tomas , Berlin Gösta , Gustafsson Bertil , Karlsson Hans
Published: January 2005

To diagnose lymphoproliferative central nervous system (CNS) involvement we have used monoclonal antibodies in an immunocytochemical method for differentiation of cells in cerebrospinal fluid (CSF) and peripheral blood. The cell distribution in 9 patients with B‐cell lymphoma and 7 patients with chronic lymphatic leukemia was compared to that in a group of patients with aseptic meningitis. Most patients with neoplastic CNS involvement showed a high proportion of CSF B cells (OKB2+ and/or OKB7+) and a concurrently low proportion of CSF T cells (anti‐Leu 1 +). Proliferating cells expressing transferrin receptor (OKT9 labeled) were increased in the CSF of 2 patients with neoplastic CNS involvement. In 2 patients with infectious CNS complications, the cell distribution in CSF did not differ from that in patients with aseptic meningitis. Patients with leukemia who had no CNS symptoms, and also 1 patient with meningitis and blood—brain barrier damage, showed a normal cell distribution in CSF despite high B‐cell numbers in the peripheral blood. This indicates a selective passage of leukocytes into the CNS and/or local proliferation.
4‐Aminopyridine improves clinical signs in multiple sclerosis
Authors: Stefoski D. , Davis F. A. , Faut M. , Schauf C. L.
Published: January 2005

Twelve temperature‐sensitive male patients with multiple sclerosis and 5 normal men were monitored before, during, and after the intravenous injection of 7 to 35 mg of 4‐aminopyridine (4‐AP) in 1‐to 5‐mg doses, every 10 to 60 minutes. Static quantitative perimetry, flicker‐fusion frequency, visual acuity, and videotaped neurological examinations were performed. Ten of the 12 patients showed mild to marked improvement. Vision improved in 7 patients, oculomotor function in 5, and motor function (power, coordination, gait) in 5. Improvements developed gradually within minutes of drug injection at doses as low as 2 mg, and gradually reversed around 2 to 4 hours after the peak drug effect. No effects were observed in 5 patients given saline injections. No serious side effects occurred in either the normal subjects or the patients receiving 4‐AP. It is concluded that 4‐AP lessens multiple neurological deficits in multiple sclerosis and, furthermore, that the K+ channel is functional in demyelinated central nervous system axons in humans. The improvements with 4‐AP are substantial enough to be of transient therapeutic benefit in selected patients.
Irradiation of meningiomas of the intracranial anterior visual pathway
Authors: Kupersmith Mark J. , Warren Floyd A. , Newall Joseph , Ransohoff Joseph
Published: January 2005

Twenty patients with progressive visual loss caused by intracranial meningiomas involving the anterior visual pathway were treated with irradiation alone (n = 4) or with irradiation combined with tumor excision (n = 16). Improvement in visual performance occurred in 13 patients; 2 patients experienced temporary improvement; and 5 others maintained stable visual function for up to 9.5 years (mean follow‐up, 4.0 years). Radiotherapy seemed to have no effect in 2 patients. One patient experienced temporary improvement, but eventually died from spread of tumor. Only one definite complication of radiotherapy occurred and was related to excessive radiation dose (greater than 7,000 cGy). Irradiation in the range of 5,000 to 5,000 cGy was effective either alone or in combination with surgical excision. Serial assessment of visual acuity and fields allowed us to demonstrate and quantify improvement in our patients following radiotherapy; in contrast, follow‐up computed tomographic studies rarely demonstrated reduced tumor size.
Cerebrospinal fluid monoaminergic metabolites are elevated in adults with Down's syndrome
Authors: Kay Arthur D. , Schapiro Mark B. , Riker Amy K. , Haxby James V. , Rapoport Stanley I. , Cutler Neal R.
Published: January 2005

Under conditions of rest and a low monoamine diet, brain monoamine activity was examined in young (<35 years) and old (>35 years) adults with Down's syndrome and in control subjects by measuring the cerebrospinal fluid (CSF) and plasma concentrations of the neurotransmitter norepinephrine, and of 5‐hydroxyindoleacetic acid (5‐HIAA), homovanillic acid (HVA), and 3‐methoxy 4‐hydroxyphenylglycol (MHPG), the respective metabolites of the neurotransmitters serotonin, dopamine, and norepinephrine. There were no age‐related differences in metabolite concentrations in either the Down's syndrome or control subjects. CSF concentrations of 5‐HIAA, HVA, and norepinephrine were significantly higher in young subjects with Down's syndrome as compared with young controls, and CSF concentrations of 5‐HIAA and norepinephrine were significantly higher, by twofold or more, in old subjects with Down's syndrome as compared with older controls. The results suggest that monoamine turnover and brain functional activity involving monoamines is elevated in Down's syndrome, and that the early neuropathological changes in the disorder are not associated with a monoamine deficit.
IgG heavy‐chain (Gm) allotypes in demyelinating polyneuropathy associated with MAG‐binding monoclonal IgM autoantibodies
Authors: Kahn Sidney N. , Pandey Janardan P.
Published: January 2005

IgG heavy‐chain (Gm) allotypes were determined in 11 Caucasian patients with the syndrome of demyelinating polyneuropathy associated with monoclonal IgM autoantibodies with affinity for myelin‐associated glycoprotein. The frequency of Gm phenotypes in the patient group was significantly different from that seen in a normal Caucasian population.
Postherpetic gustatory flushing and sweating
Authors: Drummond Peter D. , Boyce Geoffrey M. , Lance James W.
Published: January 2005

An 11‐year‐old girl who had suffered right facial herpes zoster at the age of 6 years was left with anesthetic scars in the distribution of the third division of the trigeminal nerve. Since then, certain tastes provoked flushing and sweating localized to the scarred areas, lasting for 10 to 15 minutes after a latency of a few seconds. The response was evoked most readily from the ipsilateral posterior section of the tongue and was virtually abolished by local administration of anesthesia to the tongue. It remained unaltered after blockade of the sphenopalatine and stellate ganglia but was diminished by blockade of the mandibular nerve. Thermoregulatory sweating and flushing were diminished in the scarred areas. Patchy destruction of sympathetic fibers, which are known to accompany peripheral trigeminal nerve branches, and reinnervation of the affected areas by parasympathetic fibers that normally mediate salivation may explain the phenomenon. It is thus analogous to the gustatory flushing and sweating that may follow damage to the auricultemporal nerve in the region of the parotid gland (Frey's syndrome).
3‐O‐Methyldopa and the response to levodopa in Parkinson's disease
Authors: Nutt John G. , Woodward William R. , Gancher Stephen T. , Merrick Dawn
Published: January 2005

Plasma 3‐O‐methyldopa (3OMD) concentrations in parkinsonian patients treated with levodopa on a long‐term basis reflect daily levodopa dosage and do not vary markedly during the day. Oral challenges with 3OMD reduce the clinical response to levodopa infusions, but 3OMD is no more potent than phenylalanine in this regard. These observations, plus the fact that 3OMD makes a small contribution to the total concentration of large neutral amino acids competing with levodopa for transport at the blood‐‐brain barrier, support the contention that 3OMD is not an important determinant of clinical response to levodopa.
Lambert‐Eaton myasthenic syndrome: II. Immunoelectron microscopy localization of IgG at the mouse motor end‐plate
Authors: Fukuoka Tadahiro , Engel Andrew G. , Lang Bethan , Newsom‐Davis John , Vincent Angela
Published: January 2005

The autoimmune origin of the Lambert‐Eaton myasthenic syndrome (LEMS) was documented by passive transfer of its electrophysiological features from humans to mice with IgG. Freeze‐fracture electron microscopy has demonstrated a loss of active‐zone particles in human LEMS and in its mouse passive transfer model. These data imply that the active zones are targets of the pathogenic LEMS autoantibodies. Immunolocalization of the antibodies has been hindered, however, by a paucity of active‐zone particles (about 50/μm2 normally and still lower in LEMS) and by diffusion artifacts in the immunoperoxidase method. To obviate these problems, we employed sensitive avidin–biotin detection systems, both peroxidase and ferritin labels, and quantitative immunoelectron microscopy and end‐plate morphometry. We compared mice treated with LEMS IgG, control IgG, and no IgG. In all mice, nonspecific background staining was found in the basal lamina covering the muscle fibers and Schwann cells. When a single 10‐mg dose of IgG was injected intravenously, IgG samples from 12 patients produced significant immunostaining of the mouse active zones; from 7 patients they did not. Higher doses of intraperitoneally injected IgG (20 mg, three times a day for 2 days, or 10 mg/day for 15 days) from each of 4 patients (3 of whose IgG previously transferred LEMS to mice) caused significant immunostaining of mouse active zones: (1) the mean density (no./μm presynaptic membrane length) of positive active zones was 0.91 in the immunoferritin study and 0.72 in the immunoperoxidase study (control values, 0.12 and 0.02); and (2) 43% of the ferritin particles in the primary cleft were concentrated at the active zones and the rest were scattered randomly (control value, 5.3%). The findings indicate that LEMS IgG binds to the active zones of the presynaptic membrane.
Physiological effects of 4‐aminopyridine on demyelinated mammalian motor and sensory fibers
Authors: Bowe Constance M. , Kocsis Jeffery D. , Targ Elizabeth F. , Waxman Stephen G.
Published: January 2005

The selective response of demyelinated sensory fibers to 4‐aminopyridine (4‐AP) has been proposed as a mechanism underlying the reported paresthesias that complicate the use of this potassium‐channel blocking agent in clinical trials for the treatment of multiple sclerosis and neuromuscular disorders. To identify differences in the electrophysiological response of specific fiber types to the application of 4‐AP, rat ventral and dorsal spinal roots, demyelinated by intrathecal injections of lysophosphatidylcholine, were examined in vitro before and during potassium‐channel blockade. The compound action potentials of demyelinated ventral roots showed a prominent postspike negativity associated with a broadening of single action potentials following application of 4‐AP. Under similar conditions, whole root responses of demyelinated dorsal root axons also developed a late negativity, but individual fibers were observed to fire repetitively in response to a single stimulus. The data support the hypothesis that the prominent sensory dysfunctions reported in clinical trials of 4‐AP are due to the selective response characteristics of sensory fibers.
Regional cerebral blood flow in down's syndrome
Authors: Melamed Eldad , Mildworf Bracha , Sharav Tereza , Belenky Lev , Wertman Eliahu
Published: January 2005

Regional cerebral blood flow was measured in 14 patients with Down's syndrome, in 46 patients with Alzheimer's disease and senile dementia of the Alzheimer type, and in 114 age‐matched controls, using the xenon 133 inhalation technique. Cerebral blood flow was reduced in 13 of 14 Down's patients by a mean of 16.8 ± 2.5% from expected age‐matched normal values. Degrees of regional cerebral blood flow reduction did not differ among the frontal, temporal, parietal, and occipital regions in both cerebral hemispheres. The regional cerebral blood flow Decemberreases were similar in magnitude and pattern to those in Alzheimer patients. These findings constitute an additional similarity between the two disorders.
Supratentorial malignant gliomas in childhood: A review of fifty cases
Authors: Dropcho Edward J. , Wisoff Jeffrey H. , Walker Russell W. , Allen Jeffrey C.
Published: January 2005

From 1977 to 1986, 50 children aged 15 months to 18 years were treated for supratentorial malignant gliomas at the Memorial Sloan‐Kettering Cancer Center and the New York University Medical Center. Thirteen patients had glioblastoma multiforme, 29 had anaplastic astrocytomas, and 8 had malignant gliomas. In 10 patients the tumor evolved from a low‐grade lesion. Seven patients, including 2 patients with neurofibromatosis, developed multiple primary malignant neoplasms. The median time to tumor progression after surgery was 31 weeks, with local recurrence representing the mode of treatment failure in nearly all patients. Notable clinical features included symptomatic leptomeningeal metastasis (13 patients) and intratumoral hemorrhage (9 patients). The estimated median survival time for all 50 patients was 98 weeks, with a 3‐year survival rate of 32%. A trend toward longer survival was seen in patients 12 years of age or younger at diagnosis. There was no apparent correlation between survival and tumor histology or tumor location. Recommendations for management are presented.
Search for antibodies to galactocerebroside in the serum and cerebrospinal fluid in human demyelinating disorders
Authors: Rostami A. M. , Burns J. B. , Eccleston P. A. , Manning M. C. , Lisak R. P. , Silberberg D. H.
Published: January 2005

To determine if galactocerebroside (GalC) is a target antigen in the human demyelinating disorders multiple sclerosis, Guillain‐Barré syndrome, and chronic demyelinating inflammatory polyneuropathy, we examined the serum and cerebrospinal fluid from patients with these disorders and from control subjects using four assay systems. In none of these assays could we detect significant differences in anti‐GalC antibody titer between patients with demyelinating diseases and normal subjects or patients with demyelinating diseases and normal subjects or patients with other neurological disorders. Our data suggest that there is no humoral immune response to GalC in human demyelinating disorders.
Elevated serotonin and reduced dopamine in subregionally divided Huntington's disease striatum
Authors: Kish Stephen J. , Shannak Kathleen , Hornykiewicz Oleh
Published: January 2005

We measured the rostrocaudal distribution of serotonin, dopamine, and their metabolites in Huntington's disease striatum (caudate and putamen). Mean levels of serotonin or 5‐hydroxyindoleacetic acid were elevated in most striatal subdivisions, whereas concentrations of dopamine or its metabolite homovanillic acid were slightly to markedly reduced. Dopamine and serotonin were at control levels in the nucleus accumbens and substantia nigra. Whereas the above‐normal serotonin can most likely be accounted for by striatal atrophy, the reduced dopamine suggests either a marked down regulation of nigrostriatal dopamine neurons or an actual reduction in the arborization of the striatal dopamine neurons. As experimental animal data suggest, the relative excess of striatal serotonin or one of its metabolites may facilitate the neurodegenerative process in Huntington's disease striatum.
Chronic muscle weakness caused by Borrelia burgdorferi meningoradiculitis
Authors: Wokke J. H. J. , de Koning J. , Stanek G. , Jennekens F. G. I.
Published: January 2005

A 19‐year‐old man developed chronic weakness of the lower limbs as the predominant manifestation of Borrelia burgdorferi infection of the nervous system. Spirochetes were demonstrated in the cerebrospinal fluid. The condition resolved following intravenous penicillin treatment.
Positron emission tomography studies of cerebral glucose metabolism in chronic partial epilepsy
Authors: Abou‐Khalil Bassel W. , Siegel George J. , Chris Sackellares J. , Gilman Sid , Hichwa Richard , Marshall Robert
Published: January 2005

Positron emission tomography (PET) performed with {18F}‐2‐fluoro‐2‐deoxy‐D‐glucose ({18F}FDG) was used to measure local cerebral metabolic rate for glucose (lCMRGlc) interictally in 31 patients with chronic partial epilepsy and 16 age‐matched normal subjects. Hypometabolic zones were visualized in 25 patients (81%). Cortical lCMRGlc in hypometabolic zones was within 2 standard deviations of the mean for normal temporal cortex in all but 8 patients. However, in 24 patients asymmetry between the hypometabolic cortex and homologous contralateral cortex was more than 2 standard deviations above the mean cortical asymmetry for normals. There was good correlation between hypometabolic zones and electroencephalogram (EEG) foci in patients with unilateral well‐defined EEG foci. Diffuse or shifting EEG abnormalities were often associated with normal PET scans. Of 28 patients who underwent magnetic resonance imaging, 10 showed focal temporal lobe abnormalities corresponding to focal hypometabolism. While the {18F}FDG PET scan cannot currently localize an epileptogenic zone independently, the absence of focal hypometabolism or its presence contralateral to a presumed EEG focus suggests the need for additional electrophysiological data.
Alzheimer‐related neuronal protein A68: Specificity and distribution
Authors: Wolozin Benjamin , Davies Peter
Published: January 2005

We previously reported that the monoclonal antibody Alz‐50 recognizes a protein (A68) with an apparent molecular weight of 68,000 daltons in the brains of patients who died with Alzheimer's disease, but not in brain tissue from individuals free of neurological disease. We now report that substantial quantities of this protein are found in brain tissue only from patients with Alzheimer's disease, Although trace amounts of A68 may be detected in cases of Guam—Parkinson dementia complex and Pick's disease, accumulation of A68 in neuronal perikarya and particularly in neuronal processes appears to be a unique feature of Alzheimer's disease. A68 can readily be detected in cerebrospinal fluid after concentration and partial purification. These data, combined with the quantitation of immunoreactivity in other neurological diseases, suggest the usefulness of detection of A68 in cerebrospinal fluid for diagnosis of Alzheimer's disease.
Naltrexone decreases self‐injurious behavior
Authors: Herman Barbara H. , Kathryn Hammock M. , Bs Ann Arthur‐Smith, , Egan James , Chatoor Irene , Werner Alisa , Zelnik Nathaniel
Published: January 2005

The effect of naltrexone (0.5, 1.0, 1.5, and 2.0 mg/kg) on the frequency of self‐injurious behavior (SIB) was investigated in three male adolescents. The frequency of total SIB was reduced significantly in all three subjects; dose‐dependent Decemberreases (at 0.5, 1.0, and 1.5 mg/kg) in SIB frequency were observed in the two mentally retarded subjects. These data suggest a role for opioid peptides in SIB.
Alpha‐glucosidase deficiency and basilar artery aneurysm: Report of a sibship
Authors: Makos Mignon M. , McComb Rodney D. , Hart Michael N. , Bennett Donald R.
Published: January 2005

Glycogen deposition in vascular smooth muscle has been demonstrated previously in alpha‐glucosidase deficiency but has not been clinically significant. Three sons of healthy, nonconsanguineous parents developed progressive proximal muscular weakness secondary to alpha‐glucosidase deficiency. Each patient developed a fusiform basilar artery aneurysm, which was complicated by fatal rupture in two patients and a cerebellar infarction in the third. Postmortem examination demonstrated severe vacuolation of skeletal muscle, liver, and vascular smooth muscle with accumulation of periodic acid‐Schiff–positive, diastase‐sensitive material. In the surviving brother, similar glycogen deposition was demonstrated in the smooth muscle of the superficial temporal artery. Basilar artery aneurysm formation in this sibship may be a consequence of alpha‐glucosidase deficiency.
Total lymphoid irradiation in multiple sclerosis: Blood lymphocytes and clinical course
Authors: Cook Stuart D. , Devereux Corinne , Troiano Raymond , Zito George , Hafstein Marino , Lavenhar Marvin , Hernandez Enrique , Dowling Peter C.
Published: January 2005

We have found a significant relationship between blood lymphocyte count and prognosis in 45 patients receiving either total lymphoid irradiation or sham irradiation for chronic progressive multiple sclerosis. Patients with sustained lymphocyte counts less than 900 mm−3 for prolonged periods after treatment showed less rapid progression over the ensuing 3 years than did patients with multiple sclerosis who had lymphocyte counts above this level (p < 0.01). Our results suggest that a simple laboratory test, the absolute blood lymphocyte count, may serve as a valuable barometer for monitoring the amount of immunosuppressive therapy needed to prevent progression in patients with multiple sclerosis, and possibly other autoimmune diseases.
Unilateral asterixis due to ipsilateral lesions in the pons and medulla
Authors: Peterson Donald I. , Peterson Gordon W.
Published: January 2005

Unilateral asterixis was ipsilateral to the brainstem lesions in 2 patients. The finding of unilateral asterixis has been consistently associated with structural neurological disease, but contrary to previous reports it is not a reliable localizing or lateralizing finding.
Visual evoked potentials in patients with neuropathy and macroglobulinemia
Authors: Barbieri S. , Nobile‐Orazio E. , Baldini L. , Fayoumi Z. , Manfredini E. , Scarlato G.
Published: January 2005

Visual evoked potentials were studied in 11 patients with neuropathy and macroglobulinemia. The P100 latency was increased bilaterally in 5 of the 6 patients whose IgM M‐proteins reacted with myelin‐associated glycoprotein (MAG) and in 1 of the other patients. In patients whose M‐protein bound to MAG, abnormal visual evoked potentials correlated with the presence of the M‐protein in the cerebrospinal fluid. Subclinical involvement of the central nervous system is frequent in patients with neuropathy and anti‐MAG M‐proteins and may be due to the binding of M‐proteins to central nervous system myelin.
Borderzone ischemia
Authors: Leblanc Richard , Lucas Yamamoto Y. , Tyler Jane L. , Diksic Mirko , Hakim Antoine
Published: January 2005

Positron emission tomography was used to measure cerebral perfusion and metabolism in 7 patients with severe carotid stenosis. None of the patients had sustained a major stroke, and all had a normal neurological examination except 1 patient with a mild neurological deficit from a capsular lacuna. Cerebral blood flow (CBF), cerebral blood volume, the cerebral blood flow/cerebral blood volume ratio, oxygen metabolism, and the fractional extraction of oxygen by the brain were measured in the cerebral cortex of both hemispheres in the anterior and middle cerebral artery distributions, in the borderzone regions between the anterior and middle cerebral arteries (anterior borderzone), and between the middle cerebral and posterior cerebral arteries (posterior borderzone). Results obtained in patients were compared, using Student's t test, to those obtained in 6 neurologically normal, elderly volunteers. Cerebral blood flow and the cerebral blood flow/cerebral blood volume ratio were both significantly decreased (p < 0.025 and p < 0.005, respectively) in the anterior borderzone ipsilateral to the carotid stenosis. In the same region there was a tendency toward a rising fractional extraction of oxygen with the oxygen metabolism maintained in the low‐normal range. Cerebral blood flow was also significantly decreased (p < 0.05) in the contralateral anterior borderzone. Hemodynamic and metabolic variables in other vascular territories were not significantly different from values obtained in the control group. These data indicate that the anterior borderzone may be selectively vulnerable to severe carotid stenosis, which produces, in this region, diminished perfusion, lowered hemodynamic reserve, and a tendency towards a rising fractional extraction of oxygen.