bluuebunny/crossref_metadata_2025 huggingface dataset:
Identification and characterization of an endogenous chemotactic ligand specific for FPRL2
Authors: Migeotte Isabelle , Riboldi Elena , Franssen Jean-Denis , Grégoire Françoise , Loison Cécile , Wittamer Valérie , Detheux Michel , Robberecht Patrick , Costagliola Sabine , Vassart Gilbert , Sozzani Silvano , Parmentier Marc , Communi David
Published: December 2004

Chemotaxis of dendritic cells (DCs) and monocytes is a key step in the initiation of an adequate immune response. Formyl peptide receptor (FPR) and FPR-like receptor (FPRL)1, two G protein–coupled receptors belonging to the FPR family, play an essential role in host defense mechanisms against bacterial infection and in the regulation of inflammatory reactions. FPRL2, the third member of this structural family of chemoattractant receptors, is characterized by its specific expression on monocytes and DCs. Here, we present the isolation from a spleen extract and the functional characterization of F2L, a novel chemoattractant peptide acting specifically through FPRL2. F2L is an acetylated amino-terminal peptide derived from the cleavage of the human heme-binding protein, an intracellular tetrapyrolle-binding protein. The peptide binds and activates FPRL2 in the low nanomolar range, which triggers intracellular calcium release, inhibition of cAMP accumulation, and phosphorylation of extracellular signal–regulated kinase 1/2 mitogen-activated protein kinases through the Gi class of heterotrimeric G proteins. When tested on monocytes and monocyte-derived DCs, F2L promotes calcium mobilization and chemotaxis. Therefore, F2L appears as a new natural chemoattractant peptide for DCs and monocytes, and the first potent and specific agonist of FPRL2.
A novel C‐terminal sequence from barley polyamine oxidase is a vacuolar sorting signal
Authors: Cervelli Manuela , Caro Oriana Di , Penta Alessandra Di , Angelini Riccardo , Federico Rodolfo , Vitale Alessandro , Mariottini Paolo
Published: December 2004

Barley contains two different isoforms of flavin‐containing polyamine oxidase (BPAO1 and BPAO2). We have previously demonstrated that BPAO2 is a symplastic protein in barley leaves. On the contrary, maize polyamine oxidase (MPAO), the best characterized member of this enzyme class, is apoplastic. Comparison of the derived amino‐acid sequences of BPAO2 and MPAO has revealed that both precursor proteins include a cleavable N‐terminal signal peptide of 25 amino acid residues, but the barley enzyme shows an extra C‐terminal extension of eight amino acids. By means of MPAO engineering with BPAO2 C‐terminal tail (MPAO‐T) and exploiting transient expression in Nicotiana tabacum protoplasts, we demonstrate that this oligopeptide is a signal for protein sorting to the plant vacuole. The vacuolar sorting of MPAO‐T was saturable. Specific mutations of the C‐terminal tail were constructed to determine which amino acid residues of this novel propeptide affect proper protein sorting. No consensus sequence or common structural determinant is required for the intracellular retention of the MPAO‐T protein, but a gradual lowering of the efficiency was observed as a result of progressive deletion of the C‐terminus.
Domestic strain: a hindrance in rehabilitation?
Authors: Östlund Gunnel , Cedersund Elisabet , Hensing Gunnel , Alexanderson Kristina
Published: December 2004

Long‐term sick leave due to musculoskeletal disorders has increased, especially for women. The aim of this paper was to explore women's and men's descriptions of the private arena in relation to rehabilitation and sickness absence. This study is part of a larger project focusing on consequences of sick leave. Individual interviews were conducted in 1997 and 1998 with 20 women and men who had been long‐term sick‐listed in 1985 with musculoskeletal diagnoses. The data were analysed using Grounded theory in combination with gender analysis, which involved separately comparing men's and women's statements in order to find similarities and differences. According to the interviewees, factors in the private arena were closely associated with the rehabilitation process. Women described themselves as being responsible for the domestic work while men more often ‘helped out’ when asked to do so. Women and men described similar strategies to facilitate domestic work. Three dimensions of domestic life were identified, comprising division of domestic work and division of responsibility for domestic life and the amount of socio‐emotional support or isolation at home. Using these dimensions, a ‘domestic strain model’ was developed. Our study indicates that long‐term sick‐listed women experiencing domestic strain would rather stay at home than return to work. Domestic strain involves inequities in the division of work and responsibilities and a lack of socio‐emotional support at home. However, the model of domestic strain needs further empirical testing.
Gender, muscle, and velocity comparisons of mechanomyographic and electromyographic responses during isokinetic muscle actions
Authors: Cramer Joel T. , Housh Terry J. , Weir Joseph P. , Johnson Glen O. , Berning Joseph M. , Perry Sharon R. , Bull Anthony J.
Published: December 2004

The purpose of this study was to examine the responses of peak torque (PT), mean power output (MP), mechanomyographic (MMG) and electromyographic (EMG) amplitude and mean power frequency (MPF) of the vastus lateralis (VL), rectus femoris (RF), and vastus medialis (VM) in males and females during maximal, concentric isokinetic muscle actions. Subjects performed maximal leg extensions at 60° s−1, 120° s−1, 180° s−1, 240° s−1, 300° s−1, 360° s−1, 420° s−1, and 480° s−1. No gender differences were obser‐ved, but there were muscle‐specific differences for the patterns of MMG MPF, EMG amplitude, and EMG MPF. The MP and MMG amplitude increased to 180–240° s−1, plateaued, and then decreased to 480° s−1. MMG MPF for the VL and VM remained unchanged to 300° s−1, but then increased to 480° s−1. The EMG amplitude for the RF and EMG MPF for the VL decreased across velocity. Overall, these findings indicated that there were muscle‐specific, velocity‐related differences in the associations among motor control strategies (EMG amplitude and MPF) and the mechanical aspects of isokinetic muscular activity (MMG amplitude and MPF).
SOME WILSON LOOPS IN GRAVITATIONAL METRIC FIELDS
Authors: SHAO D. , SHAO L. , NODA H. , SHAO C. G.
Published: December 2004

We calculated some Wilson loop functionals in a static sphere-symmetrical diagonal metric field, the Robertson–Walker (RW) metric field and a gravitational metric field established by cosmic string, respectively, and calculated the leading term W(2) and the term W(4) of a Wilson loop in Kerr metric field. Using the direction change of vector when it is parallel transported in the metric field of cosmic string, the cone symmetry of the metric field is demonstrated.
Normal cerebrospinal fluid protein, increased intracranial pressure, and the guillain‐barré syndrome
Authors: Sullivan Richard L. , Reeves Alexander G.
Published: January 2005

A patient who had the Guillain‐Barré syndrome (GBS) with associated increased intracranial pressure (ICP) and papilledema is described. The cerebrospinal fluid protein level and electrophoretic pattern were normal. This indicates that abnormal or excessive CSF protein is not the cause of the ICP and papilledema associated with GBS.
An immunological and cytogenetic investigation of tuberous sclerosis
Authors: Roos Raymond P. , Kirkpatrick Charles , Gadoth Natan , Berman Wulfred , Rary Jack
Published: January 2005

Patients with tuberous sclerosis were studied to investigate the pathophysiological basis for hamartoma and neoplasia development in this disease. Chromosome banding, aberration following G‐2 x‐ray exposure, and the frequency of sister chromatid exchange were normal. Mean IgM levels were significantly elevated above those of random institutionalized control patients but not above cottage‐mate controls. Although 4 out of 10 patients did not respond to any of the skin test antigens used, tuberous sclerosis patients and controls had similar responses to in vitro lymphocyte transformation. This study failed to reveal any cytogenetic abnormalities or conclusive evidence of immune deficiency in tuberous sclerosis.
Ataxia and nystagmus induced by injection of local anesthetics in the neck
Authors: de Jong Paul T. V. M. , de Jong J. M. B. Vianney , Cohen Bernard , Jongkees Leonard B. W.
Published: January 2005

Vertigo, ataxia, and nystagmus were induced in animals and man by injecting local anesthetics into the neck. This presumably interrupted the flow of afferent information from neck muscle and joint receptors. Ataxia in man was associated with a broad‐based, staggering gait; hypotonia of the ipsilateral arm and leg; and a strong sensation of ipsilateral falling or tilting. Nystagmus was stronger in the rabbit and cat than in the monkey and was not induced in man. In the rabbit, section of the cervical roots on one siEN reactivated signs of a previous labyrinthectomy, regardless of the side of operation. Neck‐afferent nystagmus was not dependent on the cerebellum, and the activity responsible for it appeared to ascend ipsilaterally through ventral portions of the cervical spinal cord. The data demonstrate the dependence of neck‐afferent nystagmus on the vestibular system and suggest that in the presence of previous vestibular lesions, neck joint or muscle disorders may be a cause of clinical vertigo, ataxia, or nystagmus.
Axonal transport disturbances in vincristine‐induced peripheral neuropathy
Authors: Green Louis S. , Donoso J. Alejandro , Heller‐Bettinger Irene E. , Samson Fred E.
Published: January 2005

Fast axoplasmic transport of labeled proteins as well as the ultrastructure was studied in the vagus nerves of cats treated chronically with vincristine sulfate (VCR) and in normal nerves exposed in vitro to vincristine. Transport was partially blocked in nerves of some neuropathic cats, and nerves of all neuropathic animals showed a small accelerated transport peak, probably reflecting a reactive or regenerative proces. In vitro, vincristine induced a dose‐related partial blockage of fast axoplasmic transport, with concurrent disappearance of microtubules and appearance of paracrystals.
ACTH and neuromuscular transmission: Electrophysiological in vitro investigation of the effects of corticotropin and an ACTH fragment on neuromuscular transmission
Authors: Birnberger K. L. , Rüdel R. , Struppler A.
Published: January 2005

The effects of corticotropin (ACTH) and the polypeptide fragment ACTH 4‐10 on neuromuscular transmission were studied in rat phrenic nerve–diaphragm preparations in vitro. ACTH decreased the quantum content of end‐plate potentials (epps) and increased the transmission failure rate. The frequency of miniature end‐plate potentials (mepps) was increased. The results indicate that ACTH acts directly on the presynaptic terminal. The findings could explain the transient decrease in muscle strength often observed during corticotropin therapy for myasthenia gravis, and they suggest that the therapeutic effect of ACTH is indirectly mediated. The only marked effect produced by the ACTH fragment was an increase in the mepp frequency; quantum content and failure rate of epps remained unchanged. Since ACTH 4‐10 is unable to stimulate cortisone release, its clinical efficacy seems unlikely.
The long‐term prognosis in untreated cerebral aneurysms: I. The incidence of late hemorrhage in cerebral aneurysm: A 10‐year evaluation of 364 patients
Authors: Winn H. Richard , Richardson Alan E. , Jane John A.
Published: January 2005

Three hundred sixty‐four patients who suffered a subarachnoid hemorrhage from an aneurysm in either the posterior communicating or anterior communicating artery and who were not surgically treated have been followed for up to 21 years in order to determine the frequency of subsequent hemorrhage. For patients surviving six months: (1) rebleeding occurs on the average of 3.5% per year during the first decade; (2) the mortality associated with a late rebleed is 67%; (3) high blood pressure and female sex predispose to late rebleeding in posterior communicating artery aneurysms; (4) young patients take longer to rebleed than older patients but do so at the same rate; and (5) increase in aneurysm size as judged by routine arteriography six months after the initial hemorrhage did not correlate with rebleeding, although an increase in size had occurred in all patients who were studied angiographically at the time of their late rebleed.
Subacute measles encephalitis complicating Hodgkin's disease in an adult
Authors: Wolinsky Jerry S. , Swoveland Peggy , Johnson Kenneth P. , Baringer J. Richard
Published: January 2005

A progressive neurological illness characterized by myoclonus, motor and sensory deficits, and lethargy occurred in a patient with Hodgkin's disease and was fatal within two months. A focal inclusion cell encephalitis was demonstrated by immunohistological means to be due to measles virus. Measles encephalitis must be considered a potential opportunistic agent in the immune‐compromised host.
Ethanol and spinal presynaptic inhibition in man
Authors: Ashby Peter , Carlen Peter L. , MacLeod Stuart , Sinha Prakash
Published: January 2005

Ethanol, 0.65 gm per kilogram of body weight, was administered orally to 6 normal subjects. The mean blood ethanol levels ranged from 0.74 to 0.99 gm per liter over a subsequent 40‐minute testing period. The proportion of the soleus motoneuron pool activated by the Achilles tendon reflex was reduced. Vibratory inhibition of the monosynaptic reflex was used as an estimate of spinal presynaptic inhibition. It was unaffected by ethanol.
The EEG manifestations of chronic ethanol abuse: Relation to cerebral cortical atrophy
Authors: Newman S. E.
Published: January 2005

Eleven chronic alcoholic patients without other nonneurological or traumatic disease were evaluated by a simultaneous electroencephalogram and computerized axial tomogram. The findings suggested that chronic abusers of approximately 60 years of age or less may have a normal EEG despite the presence of cerebral cortical atrophy or dementia. In alcoholics over 60 years of age, the greater the severity of cerebral cortical atrophy, the greater the slowing in background frequency of the EEG. Voltage diminution and slow‐wave transients also occurred more frequently in the older patients. The incidence of EEG abnormalities was greater than the incidence of CAT scan evidence for cerebral cortical atrophy in alcoholics over 60 years of age with dementia.
Experimental analysis of EMG feedback in treating dystonia
Authors: Bird Bruce L. , Cataldo Michael F.
Published: January 2005

Recent clinical reports have suggested that electromyographic feedback offers promise as a palliative therapy for neuromuscular disorders. This study utilized a single‐subject experimental analysis to evaluate the effects of EMG feedback training on a 20‐year‐old man with adult‐onset idiopathic dystonia to achieve control of orofacial dysfunctions. Feedback training produced reductions in levels and variability of tension in facial and forearm muscles, as measured by EMG. Reliable blind observations of facial control indicated that feedback training enabled the patient to produce normal facial relaxation in extraclinical environments.
Neoplastic angioendotheliosis: Ultrastructural study and review of the literature
Authors: Petito Carol K. , Gottlieb Geoffrey J. , Dougherty John H. , Petito Frank A.
Published: January 2005

Neoplastic angioendotheliosis is a rare disorder characterized by progressive multifocal neurological deficit and dementia. The principal pathological findings are multiple infarcts in the brain and spinal cord and the microscopical demonstration of proliferated neoplastic cells, primarily within the lumens of arteries, arterioles, and capillaries. Vessels of the central nervous system are the most severely affected and appear to be the primary site of neoplastic proliferation. Systemic intravascular and focal extravascular proliferation can also be identified. The endothelial origin of the neoplastic cell with a gradation of changes from normal to neoplastic is demonstrated by the electron microscopical study of involved meningeal vessels. Our patient and those reported in the literature all demonstrate involvement of the central nervous system. In addition, an elevated cerebrospinal fluid protein level is present in all patients for whom that information is available, and fever and renal involvement are frequently present. Steroid therapy is generally ineffective. If the disease is suspected clinically, our studies suggest that meningeal biopsy is the diagnostic procedure of choice to help determine whether chemotherapy should be instituted.
Juvenile type of distal and segmental muscular atrophy of upper extremities
Authors: Sobue I. , Saito N. , Iida M. , Ando K.
Published: January 2005

Seventy‐one cases of distal and segmental muscular atrophy of the upper extremities with juvenile onset were studied. The clinical features consisted of: juvenile onset, male preponderance, unique distribution of the muscular atrophy in the hand and forearm, tendon reflexes hypoactive in most cases but hyperactive in some, no definite sensory disturbances, no involvement of the cranial nerves, and autonomic nerve disorders in the affected region. There was rapid progression during the 2 to 3 years after onset with a slowly progressive course thereafter. There were no abnormal laboratory findings except for electrophysiological and morphological findings of the affected muscles. The site of lesion was surmised to be from the C5 to T1 spinal segments with intramedullary involvement. The cause is unknown.
Juvenile parkinsonism: A patient with possible primary striatal dysfunction
Authors: Naidu Sakkubai , Wolfson Leslie I. , Sharpless Nansie S.
Published: January 2005

A 15‐year‐old boy who initially manifested dystonic features and later developed classic parkinsonism is described. Cerebrospinal fluid homovanillic acid levels were normal or slightly elevated both before and after administration of probenecid. The patient responded favorably to treatment with levodopa and carbidopa. The normal or slightly elevated cerebrospinal fluid homovanillic acid levels contrast with the low levels usually seen in adult parkinsonian patients. The data on this patient suggest direct neostriatal involvement rather than depletion of neurons of the substantia nigra. Juvenile parkinsonism may have at least two distinct pathological forms, but they have similar clinical features and a similar response to treatment.
Blue rubber bleb nevus syndrome with CNS involvement and thrombosis of a vein of galen malformation
Authors: Waybright E. A. , Selhorst J. B. , Rosenblum W. I. , Suter C. G.
Published: January 2005

A patient is described with the dermatological features of blue rubber bleb nevus syndrome (BRBNS), focal seizures, and lateralized neurological signs. CAT scan demonstrated a nonenhanced density in the region of the vein of Galen. Neuropathological examintion showed that this density was a clot within a vein of Galen malformation. Hemangiomas that grossly resembled the skin lesions of BRBNS were seen on the cerebral surface. Many of these hemangiomas were thrombosed and overlay patchy zones of infarction. Numerous vascular malformations of varying histological types were also found within the brain and systemic organs.

Bluish, compressible, often raised hemangiomas of the skin should alert physicians to the BRBNS and the potential for vascular malformations to occur within the brain as well as systemic organs. Diagnosis of BRBNS involving the brain may assist in interpretation of radiographic findings. The tendency of these malformations to thrombose may account for focal neurological deficits.
Neurological manifestations of hereditary hemorrhagic telangiectasia (rendu‐osler‐weber disease): Report of 2 cases and review of the literature
Authors: Román Gustavo , Fisher Marc , Perl Daniel P. , Poser Charles M.
Published: January 2005

Two cases of hereditary hemorrhagic telangiectasia (HHT) with neurological involvement are presented. One patient had multiple vascular malformations including telangiectasias of the brain, medulla, and spinal cord and a berry aneurysm of the internal carotid artery; she also had a large cerebellar abscess, presumably reflecting the presence of a pulmonary arteriovenous fistula. The second patient had an idiopathic subarachnoid hemorrhage.

In more than 200 reported patients with HHT involving the nervous system, 61% had lesions secondary to a pulmonary arteriovenous fistula (cerebral hypoxemia, paradoxical and septic emboli, and brain abscess). The findings emphasize the need for early surgical correction of such fistulas. In 36% of the patients with neurological involvement and HHT, vascular malformations of the brain and spinal cord were documented, and in 3%, portal‐systemic encephalopathy was noted. Multiple lesions were frequent.

HHT should be considered a generalized vascular dysplasia (universal or systemic angiomatosis), and not simply a benign mucocutaneous disease.
Psychiatric disturbances in neurological patients: Detection, recognition, and Hospital Course
Authors: Depaulo J. R. , Folstein M. F.
Published: January 2005

The Mini‐Mental State and General Health Questionnaire were used to detect cognitive defects and emotional disturbances, respectively, in 126 consecutively admitted neurological patients. The Mini‐Mental State was revalidated in this sample as a measure related to cerebral disorder. Sixty‐seven percent of the patients tested had cognitive defect, emotional disturbance, or both. Thirty percent of the patients with psychiatric disturbance were not recognized by their physician. The majority of psychiatric disturbances persisted at the time of discharge.

Since psychiatric disturbances are common among neurological inpatients and are frequently unrecognized, further evaluation of the psychiatric training of neurologists is needed. Simple screening tests may help to increase the recognition of psychiatric disturbance. It is concluded that the Board requirement for neurologists to know psychiatry accurately reflects “the nature of their case material.”
A Behavioral analysis of complete unilateral section of the pyramidal tract at the medullary level in macaca mulatta
Authors: Schwartzman Robert Jay
Published: January 2005

Ten Macaca mulatta monkeys were operantly conditioned to perform three motor paradigms designed to evaluate single and combination finger movements. Eight of these monkeys were retested after left medullary pyramidotomy; 2 monkeys underwent left medullary pyramidotomy prior to conditioning. All animals were tested for three years after operation. Monkeys with a completely sectioned medullary pyramid could, with time, perform difficult motor paradigms that required: (1) both individual and combination finger movements; (2) proximal upper extremity motor control; (3) thumb and index finger pincer grasp; and (4) the ability to preprogram and then execute a precision hand movement. The greater the extent of pyramidal tract destruction, the longer the time necessary for recovery of both discrete finger movement and pincer grasp, the greater the effort needed to attain recovery of hand function, and the weaker the affected musculature. The 2 animals in which pyramidotomy of at least 70% of the tract preceded efforts at operant conditioning learned and performed difficult motor paradigms. In all animals, neurological examination revealed that the most enduring and functionally most important deficit that interferes with hand function following pyramidotomy is loss of contactual hand orienting responses and failure of reflex sensorimotor adjustments.
Brain dopamine receptor stimulation and the relief of parkinsonism: Relationship between bromocriptine and levodopa
Authors: Rinne U. K. , Marttila R.
Published: January 2005

The relationship between brain dopamine receptor stimulation by bromocriptine or levodopa and the relief of parkinsonism was studied in 24 patients with Parkinson disease. Bromocriptine, 30 mg daily for 20 weeks, elicited an improvement in the parkinsonian clinical features, but this was less than the subsequent improvement with levodopa and benserazide, 800 mg and 200 mg daily, respectively. There was a negative correlation between the pretreatment severity of the disease or changes in cerebrospinal fluid homovanillic acid (HVA) and improvement in parkinsonian disability during bromocriptine treatment. Furthermore, it was found that clinical improvement and HVA responses in the cerebrospinal fluid after dopamine receptor stimulation by bromocriptine may predict the clinical response to levodopa.
Multiple small cavernous angiomas of the brain with increased intracranial pressure
Authors: Tindall Richard S. A. , Kirkpatrick Joel B. , Sklar Fred
Published: January 2005

Small vascular malformations of the central nervous system are generally considered cryptic, or silent, because they are an incidental but frequent finding at autopsy. When they are symptomatic, these malformations have been associated with intracranial hemorrhage or seizures. The patient reported here had multiple small cavernous angiomas associated with a syndrome resembling pseudotumor cerebri. The increased intracranial pressure apparently was produced by multifocal areas of cerebral edema without associated hydrocephalus.
Bell's palsy and secondary syphilis: CSF spirochetes detected by immunofluorescence
Authors: Davis Larry E. , Sperry Sally
Published: January 2005

Although Bell's palsy is usually idiopathic, occasional cases may have an identifiable infectious cause. When facial paralysis results from syphilis, it usually develops during the tertiary meningovascular stage. We report a 30‐year‐old man with secondary syphilis who developed facial paralysis associated with acute syphilitic meningitis. Spirochetes were identified in the cerebrospinal fluid by immunofluorescence using standard reagents from the fluorescent treponemal antibody absorption (FTA‐ABS) test. Patients with Bell's palsy should be screened for syphilis with a blood FTA‐ABS test, and treatment with corticosteroids should be considered only after an infectious cause has been excluded.
Progressive dystonia with marked diurnal fluctuation
Authors: Ouvrier Robert A.
Published: January 2005

Three children, 2 of them siblings, developed a progressive dystonic condition in the first decade of life. The symptoms and signs were milder in the mornings or after a daytime nap but worsened greatly later in the day. One patient became unable to walk by evening. Investigations in 2 patients revealed a reduced concentration of cerebrospinal fluid homovanillic acid. The 2 more severely affected patients showed dramatic and sustained improvement with levodopa therapy. The condition bears a clinical resemblance to some reported cases of juvenile Parkinson's disease but is probably a separate disorder.
Destruction of the internal sagittal stratum and normal smooth pursuit
Authors: Sharpe James A. , Deck John H. N.
Published: January 2005

Analysis of smooth pursuit by infrared oculography showed normal horizontal smooth eye movement gain in a patient with a unilateral parietooccipital hematoma that involved the internal sagittal stratum and the adjacent tapetum and visual radiation. Normal pursuit toward the lesion is attributed to commissural transfer of visual information from the normal cerebral hemisphere to the intact extrastriate cortex of the visually deprived hemisphere. This pathophysiological correlation indicates that the occipitomesencephalic projections of the internal sagittal stratum and the peristriate interhemispheric connections of the tapetum are not necessary for smooth pursuit system function.
The L‐dopa on‐off effect in parkinson disease: Treatment by transient drug withdrawal and dopamine receptor resensitization
Authors: Direnfeld L. , Spero L. , Marotta J. , Seeman P.
Published: January 2005

It has been suggested that patients with Parkinson disease partially compensate for neuron loss by developing denervation supersensitivity, and, if so, that prolonged levodopa (L‐dopa) therapy might lead to desensitization. As a preliminary test of this hypothesis, and in order to study whether it was possible to “resensitize” a patient who had already presumbly been desensitized by previous L‐dopa therapy, a patient who had become unpredictably responsive to L‐dopa was investigated.

The patient had been taking L‐dopa for eight years and had exhibited severe dyskinesia‐akinesia oscillation (“on‐off” phenomenon) before the study. There was no consistent response to his hourly doses of Prolopa (L‐dopa and benserazide in a 4:1 ratio). He was first lowered, over 33 days, to 20% of his original Prolopa dose. The dosage was then increased until a consistent response were observed. The three main results achieved were, first, overall reduction by 64% of the daily requirement for L‐dopa; second, conversion from a previously unpredictable to a predictable response to each dose of L‐dopa; and, third, change in his movement fluctuations to a pattern more typical of “end‐of‐dose” akinesia than the “on‐off” phenomenon. The results support the idea of dopamine receptor resensitization upon reduction of the L‐dopa dosage.
Inhibition of the evoked release of acetylcholine by the porphyrin precursor δ‐aminolevulinic acid
Authors: Bornstein Joel C. , Pickett Jackson B. , Diamond Ivan
Published: January 2005

The effects of δ‐aminolevulinic acid (ALA) on neuromuscular transmission were studied. High concentrations (0.6 to 18 mM) of ALA caused significant reductions in the amplitudes of curarized end‐plate potentials (epps). Changing the ratio of calcium to magnesium in the bathing solution allowed the quantal content of the epps to be directly measured. Under these conditions, ALA reduced the quantal content of epps without affecting the depolarization produced by a single quantum of acetylcholine. It was concluded that ALA, in high concentrations, inhibits the release of acetylcholine evoked by a nerve impulse but is unlikely to be the cause of the neurological defects of acute porphyria.
Pheochromocytoma causing exercise‐related headache and pulmonary edema
Authors: Paulson George W. , Zipf Robert E. , Beekman Jerome F.
Published: January 2005

A 13‐year‐old hypertensive girl with a history of severe headaches following exercise developed circulatory collapse after “squat jumps.” Pulmonary edema was present without evidence of a cerebral mass. The patient died with increased intrased intracranial pressure despite decompressive craniectomy. Neuropathological evaluation revealed diffuse cerebral edema and a pheochromocytoma in the right adrenal gland. Examination of urine revealed elevation of vanillylmandelic acid levels.
Metabolism of dimethoxymethyl phenobarbital (Eterobarb) in patients with epilepsy
Authors: Goldberg Mark A. , Gal Joseph , Cho Arthur K. , Jenden Donald J.
Published: January 2005

The metabolism of dimethoxymethyl phenobarbital (DMMP) was investigated in 6 epileptic patients using deuterium‐labeled drug measured by integrated gas chromatography/mass spectrometry. Following an oral dose of 120 mg, none of the parent compound appeared in serum, urine, or saliva in spite of a measurement sensitivity of less than 0.05 nmol/ml. A metabolite, monomethoxymethyl phenobarbital (MMP), was detected in all patients, with peak serum concentrations averaging 1.96 nmol/ml at 1.3 hours. Phenobarbital was the major metabolite detected, and it reached peak levels in 6 to 12 hours. Two of the patients were continued on DMMP therapy for several months and then administered a pulse dose of deuterated drug. Again no DMMP was detectable. Steady‐state drug administration had no effect on the kinetics of metabolism of either MMP or phenobarbital although peak serum concentrations were lower for both metabolites. In the urine of 5 patients an unidentified labeled fragment was detected that does not appear in urine from patients taking phenobarbital. From these pharmacokinetic studies it appears likely that the anticonvulsant activity of DMMP results from its metabolic conversion to phenobarbital.
Thalamic degeneration in X‐chromosome–linked copper malabsorption
Authors: Iwata Makoto , Hirano Asao , French Joseph H.
Published: January 2005

Thalamic degeneration was present in 5 autopsied cases of X‐chromosome–linked copper malabsorption (X‐cLCM), Menkes' kinky hair disease. Among the thalamic nuclei, those in the formatio paraventricularis, intralamellaris, and extralamellaris were spared. The nuclei projecting to the granular cortices had severe neuronal depopulation. The thalamic nuclei that send axons to the agranular cortices were less often and less severely involved. The thalamic afferent system was intact except for degeneration of the red nucleus. Cerebral cortical lesions varied from case to case and usually were less marked than thalamic neuronal changes.
Hyperexcitability of motor and sensory neurons in neuromyotonia
Authors: Lance James W. , Burke David , Pollard John
Published: January 2005

Two members of a family with a neuropathy resembling Charcot‐Marie‐Tooth disease were unable to relax their muscles after voluntary contraction. Muscle spasm often outlasted voluntary contraction by 30 seconds or more before subsiding into myokymia and fasciculations. The posture of the hand during muscle spasm resembled that of tetany, and Trousseau's and Chvostek's signs were present although no abnormality of calcium or magnesium metabolism could be demonstrated. Muscle spasms ceased during medication with carbamazepine, 600 mg daily.

Nerve stimulation, electromyography, and regional neuromuscular blockade with curare indicated that the condition originated in peripheral nerve, while microneurographic recordings showed that sensory as well as motor fibers were hyperexcitable. Sural nerve biopsy revealed axonal degeneration involving myelinated and unmyelinated fibers. It was concluded that the neural hyperexcitability is caused by a membrane defect resulting in a low threshold for excitation throughout the length of the axon.
Chronic lead intoxication: Effects on developing optic nerve
Authors: Tennekoon G. , Aitchison C. S. , Frangia J. , Price D. L. , Goldberg A. M.
Published: January 2005

The effects of chronic lead intoxication were studied by examining the optic nerves of mice given lead‐containing mother's milk from the day 1 to day 21 of life. Biochemical assays for myelin basic protein, 2′,3′‐cyclic nucleotide phosphodiesterase, and cerebroside sulfotransferase showed that the total amount of myelin produced by the lead‐exposed animals was decreased. Lead exposure did not alter the number of oligdendroglia or the relationship between axons and myelin sheaths. The hypomyelination was paralleled by a reduction in size of optic axons in the lead‐exposed animals. The data suggest that chronic exposure to lead in developing mice results in a primary effect on neurons and that hypomyelination is secondary to reduction in axon size.
Transmission of chronic spongiform encephalopathy with kuru plaques from humans to small rodents
Authors: Tateishi Jun , Ohta Michiya , Koga Makoto , Sato Yuji , Kuroiwa Yoshigoro
Published: January 2005

An unusual case of spongiform encephalopathy was transmitted directly from a human to rats and mice. After serial passages, incubation periods were shortened to about six months in rats and four months in mice. Clinical symptoms were similar in rats and mice, including ruffled fur, arched back, bradykinesia, and hind limb paralysis. Pathologically, a spongy state, proliferation of astrocytes, and neuronal changes were observed. Electron microscopic observation of the parietal cortex of rats and mice disclosed many membrane‐bound vacuoles in the neuropil, predominantly in dendrites. In the pons of mice, intramyelinic vacuoles and accumulation of extracellular fluid were prominent. The clinicopathological symptoms of the affected animals resembled those in other experimental spongiform encephalopathies, especially scrapie.
The natural history of peripheral neuropathy in primary systemic amyloidosis
Authors: Kelly John J. , Kyle Robert A. , O'Brien Peter C. , Dyck Peter J.
Published: January 2005

The records of 31 patients with primary systemic amyloidosis and peripheral neuropathy seen during a 17‐year period were analyzed to define the natural history of the neuropathy. Patients tended to be older men with a painful, distal, symmetrical sensorimotor neuropathy and prominent autonomic features. Loss of pain and temperature sensation was frequently more striking than loss of mechanoreception. Renal, cardiac, hematological, and gastrointestinal dysfunction often overshadowed the neuropathy. Clinical, neurophysiological, and histopathological studies pointed to axonal degeneration with predominant but not exclusive involvement of small myelinated and unmyelinated fibers. The neuropathy was progressive in all patients with or without treatment, but death was typically due to supervening medical complications.
Periodic lateralized epileptiform discharges in infants and children
Authors: PeBenito Rhandy , Cracco Joan B.
Published: January 2005

Clinical and electroencephalographic data on 7 infants and children with periodic lateralized epileptiform discharges (PLEDS) in their electroencephalograms were reviewed. The waveform, periodicity, and transitory nature of these discharges were similar to those reported in adults. In children, as in adults, PLEDs reflect severe underlying brain dysfunction which is often associated with metabolic and structural abnormalities. In adults, PLEDs are usually associated with altered states of consciousness and with acute unilateral cerebral lesions; in children, however, they often occur with little or no alteration in consciousness and with chronic, diffuse lesions of the central nervous system.
The effect of metrizamide on the electroencephalogram: A prospective study in 61 patients
Authors: Ropper Allan H. , Chiappa Keith H. , Young Robert R.
Published: January 2005

A prospective study was made of changes in the electroencephalogram after the use of metrizamide for myelography and posterior fossa cisternography in 61 patients (62 studies). The EEG changed in 21 patients (34%); 3 of these had had previously abnormal records, and the abnormalities were accentuated on the second recording. Eight developed paroxysmal activity, including spike and wave in 1. EEG changes appeared 24 to 48 hours after the use of metrizamide in 6 patients whose intervening EEGs were normal. The percentage of records that changed tended to increase as metrizamide was brought to more rostral levels. Symptoms following the use of metrizamide were reviewed in 58 patients. Although there were no seizures in the present series, seizures did occur with subsequent use of metrizamide in 3 other patients at our institution.

Previous reports may have underestimated the frequency of EEG changes after metrizamide since these may require 24 to 48 hours to develop. The appearance of such activity suggests that the use of metrizamide in patients with known seizure disorders, the anatomical level to which metrizamide is brought during radiography, removal of contrast medium, and further investigation of premedication with anticonvulsants all require careful consideration when use of metrizamide is being contemplated.
Search for viral particles and virus‐specific products in idiopathic Parkinson disease brain material
Authors: Schwartz Jerome , Elizan Teresita S.
Published: January 2005

Virological studies were performed on brain material from 9 patients with idiopathic Parkinson disease and 3 matched controls. Electron microscopy and indirect immunofluorescent study were carried out directly on autopsy material from substantia nigra, caudate nucleus, and hypothalamus and on primary tissue culture explants of these brain areas grown alone or in cocultivation with virus‐susceptible indicator cell lines. All studies were negative for the presence of viral particles, viral inclusions, or cellular changes suggestive of virus infection. All materials were negative for antigens of herpes simplex type 1, influenza A/NWS, and measles (Edmonston strain) viruses. Within the limits of these techniques, there is no evidence at this time that viruses are an important etiological agent in idiopathic Parkinson disease.
Seizures and related epileptiform activity in hippocampus transplanted to the anterior chamber of the eye: Modulation by cholinergic and adrenergic input
Authors: Freedman Robert , Taylor David A. , Seiger Ake , Olson Lars , Hoffer Barry J.
Published: January 2005

Transplants of rat hippocampus into the anterior chamber of the eye of a host animal were used to assess the effects of cholinergic and adrenergic neuronal inputs on the generation and duration of seizure activity. Cholinomimetics initiated both seizures and hypersynchronous neuronal activity in the transplants. Cyclic guanosine monophosphate (GMP) derivatives and isobutyl methylxanthine elicited similar changes. Reflex activation of the cholinergic parasympathetic input to the iris and transplant by illumination of the ipsilateral retina also induced seizures or increased the rate of penicillin‐induced interictal spike discharge. Application of β‐adrenergic agonists inhibited interictal spikes and paroxysmal depolarizing shifts induced by penicillin. Fluorescence histochemical studies showed that host sympathetic adrenergic fibers derived from the ground plexus of the iris invaded the transplant to form fine varicose nerve terminals. Activation of these adrenergic afferents to the transplant diminished both the amplitude and frequency of penicillin‐induced epileptiform activity. Epileptiform activity in hippocampal occular transplants is strongly modulated by cholinergic and adrenergic neuronal inputs, with the former exerting a facilatory influence and the latter, an inhibitory effect.
Cockayne syndrome: Unusual neuropathological findings and review of the literature
Authors: Soffer Dov , Grotsky Harold W. , Rapin Isabelle , Suzuki Kinuko
Published: January 2005

Two siblings with Cockayne syndrome (CS) are described and the literature on the subject is briefly reviewed. Of particular interest were the unusual neuropathological findings in 1 of the patients. These included microcephaly, white matter atrophy with patchy loss of myelinated fibers, calcifications of the basal ganglia, occasional ferrugination of cerebral and cerebellar neurons, and severe cerebellar degeneration. Findings not previously reported in CS were proliferation of extremely bizarre astrocytes, neurofibrillary tangles, and pigmentation of the globus pallidus. We conclude that brain involvement in CS is a result of primary degeneration in the central nervous system rather than being secondary to angiopathy or normal pressure hydrocephalus, as previously suggested.
Bacteroides meningitis successfully treated with metronidazole
Authors: Peterson Donald I. , Voorhees Elwood G. , Elder Harvey A.
Published: January 2005

Bacteroides meningitis without associated cerebral abscess occurred in an elderly man who had a long history of chronic otitis media. Anaerobic cultures of the cerebrospinal fluid revealed bacteroides organisms. Sensitivity testing showed them to be resistant to penicillin and chloramphenicol but sensitive to metronidazole. Appropriate therapy with this agent eradicated the causative organisms and produced rapid clinical improvement.
Language deficits after apparent clinical recovery from childhood aphasia
Authors: Woods Bryan T. , Carey Susan
Published: January 2005

Twenty‐seven children with childhood injury to the left hemisphere were tested for language function and compared with appropriate controls. Eleven children had incurred their lesions before the age of 1 year, 16 afterward. The group with perinatal injury to the left hemisphere did not show a specific aphasic deficit even though they were mildly cognitively imparired. The group of children with later injury to the left hemisphere showed aphasic deficits if the orginal injury had caused a language defect; otherwise the left hemisphere injury was not associated with specific disturbances in language function. The average age at time of lesion in those children who had recovered from aphasia was 4.7 years. We conclude that even when childhood aphasia results from a unilateral nonprogressive lesion, recovery of language is less complete than has been generally supposed.
Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (leigh disease)
Authors: Devivo Darryl C. , Haymond Morey W. , Obert Kathleen A. , Nelson James S. , Pagliara Anthony S.
Published: January 2005

Autopsy examination confirmed the diagnosis of subacute necrotizing encephalomyelopathy (SNE) in a 7‐monthold male infant who underwent several metabolic studies before death. Intermittent lactic acidemia and fumaric aciduria, an extreme hyperglycemic response to an intravenous bolus of alanine, and an elevated total body flux rate of glucose (58.4 μmoles · kg−1 · min−1) suggested a disturbance in the oxidative decarboxylation of pyruvate. Enzymological studies of postmortem samples revealed low nonactivated pyruvate dehydrogenase activity in liver (19.4%) and brain (53.8%). The lowest brain pyruvate dehydrogenase activities were noted in the midbrain and pontine regions. Supramaximal activation of the hepatic pyruvate dehydrogenase complex (135% of control values) occurred in vitro. Spontaneous reactivation following in vitro inactivation of the complex with adenosine triphosphate was significantly less (p < 0.02) in the patient's samples compared to controls. The biochemical defect was not apparent in fibroblasts. These enzymological observations point to an in vivo defect in the activation mechanism of the pyruvate dehydrogenase complex as the biochemical disturbance in SNE. The findings suggest that dichloroacetate may be beneficial in treating SNE.
Cerebral infarcts with arterial occlusion in neonates
Authors: Barmada M. A. , Moossy J. , Shuman R. M.
Published: January 2005

Among 592 infants examined at autopsy during a four‐year period, 32 (5.4%) had cerebral infarcts. Excluded were cases of traumatic hemorrhages and softening, periventricular leukomalacia, venous lesions, and any mass, including encephaloceles, with arterial distortion and infarction. Histological abnormalities were similar to those of infarcts in adults. Relatively advanced histopathological changes in some infants living only a few hours indicated that some infactions may have occurred in utero. The most common cause of arterial occlusion was embolization, with sepsis and disseminated intravascular coagulation playing a major role. The brains of term neonates were more frequently involved than those of premature infants. Multiple small infarcts occurred more often in premature infants. In most cases autonomic dysfunction with prolonged apnea, episodic seizures, and metabolic acidosis were the major associated clinical features, rather than focal neurological deficits. Similar cerebral infarcts in infants who survive with less severe systemic complications may lead to porecephaly, hemiplegia, mental and motor retardation, and recurrent seizures.
Acute intoxication with sodium valproate
Authors: Chadwick D. W. , Cumming W. J. K. , Livingstone I. , Cartlidge N. E. F.
Published: January 2005

Two epileptic patients developed an acute toxic encephalopathy consisting of altered behavior, deteriorating seizure control, and confusion while taking sodium valproate alone. Serum valproate levels were greater than 100 μg/ml in both. Toxic symptoms resolved when the dose of valproate was reduced, with a consequent fall in serum concentration of the drug.