bluuebunny/crossref_metadata_2025 huggingface dataset:
Induction of recurrent experimental allergic encephalomyelitis with myelin basic protein
Authors: Panitch Hillel , Ciccone Carlo
Published: January 2005

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

Complex partial status epilepticus (CPSE) has rarely been described in children. We have recently studied four girls, aged 1 to 4 years, who presented with CPSE. Their seizures were characterized by decreased level of consciousness, lack of response to familiar persons, diminished response to pain, staring, slow visual tracking, eye deviation, picking at nearby objects, and lip smacking. Three patients developed focal clonic activity during their seizures and one progressed to a generalized motor seizure after 4 hours of CPSE. Two patients had ictal electroencephalograms demonstrating temporpoccipital poly spikes and slow waves.
Acute thallium poisoning: Toxicological and morphological studies of the nervous system
Authors: Davis Larry E. , Standefer James C. , Kornfeld Marchio , Abercrombie David M. , Butler Cooley
Published: January 2005

Nine days following ingestion of 5 to 10 gm of thallium nitrate, a young man died with severe cranial and peripheral neuropathy, anuria, and heart failure. Ultrastructural examination of nerves obtained on days 7 and 9 demonstrated axonal degeneration with secondary myelin loss. Axons were swollen and contained distended mitochondria and vacuoles. Thallium levels in more than twenty organs and body fluids ranged from below 1.0 to 178 μg/gm; concentrations in twenty areas of the nervous system ranged from 29 to 140 μg/gm. The highest brain levels of thallium were found in gray matter. In the thalamus, 87% of the thallium was present in cell sap. Tissue concentrations of thallium did not parallel those reported for potassium, suggesting that thallium distribution differs from potassium distribution in human beings.
Alzheimer disease: Evidence for selective loss of cholinergic neurons in the nucleus basalis
Authors: Whitehouse Peter J. , Price Donald L. , Clark Arthur W. , Coyle Joseph T. , DeLong Mahlon R.
Published: January 2005

The nucleus basalis of Meynert provides diffuse cholinergic input to the neocortex. When compared with an age‐ and sex‐matched control, the nucleus basalis from a patient with Alzheimer disease demonstrated substantial reduction of neurons. Loss of this neuronal population may represent an anatomical correlate of the well‐documented cholinergic derangement in Alzheimer disease.
Voluntary and visual control of the vestibuloocular reflex after cerebral hemidecortication
Authors: Sharpe James A. , Lo Alex W.
Published: January 2005

Horizontal vestibuloocular reflex (VOR) function was studied in five patients after cerebral hemidecortication. In darkness, VOR gain contralateral to the decorticate hemisphere was slightly higher than ipsilateral gain. Voluntary modulation of the reflex by attempted fixation of imaginary targets in darkness increased this VOR asymmetry. Voluntary cancellation of the ipsilateral VOR was better than cancellation of the contralateral VOR. Voluntary enhancement of the contralateral VOR was better than enhancement of the ipsilateral VOR. Visual control of the reflex while viewing foveal targets further increased the VOR asymmetry. Defective visual modulation corresponded to paresis of ipsilateral smooth pursuit. Abnormal voluntary responses in darkness indicate that cerebral control of the reflex can be independent of the pursuit system. The hemispheres may use a corollary discharge of eye position in the orbit and a head velocity signal to direct smooth eye movements toward the perceived location of objects. One hemisphere regulates ipsilateral smooth eye movements that achieve voluntary and visual control of the VOR.
Emery‐dreifuss humeroperoneal muscular dystorphy: An X‐linked myopathy with unusual contractures and bradycardia
Authors: Hopkins Linton C. , Jackson Joseph A. , Elsas Louis J.
Published: January 2005

Clinical, electromyographic, and muscle biopsy findings in the two largest known families with emery‐Dreifuss humieroperoneal muscular dystorphy indicate that this is an X‐linked recessive muscle disease with stereotyped clinical manifestations but with variable pathological and electromyographic characteristics. Elbow contractures, involvement of humeral muscles, Hyporeflexia, and abnorman electorcardiograms are present in our patients. The disorder is associated with a potentially lethal cardiac arrhythmia that should be managed by pacemaker insertion. The skeletal muscle disease is slowly progressive and is usually not life threatening. Muscle biopsy commonly shows type I fiver atrophy. Electromyogrphy usually indicates myopathy, though the classic findings of myopathy may not be present in every muscle.
The lateral, third, and fourth ventricle choroid plexus of the dog: A structural and ultrastructural study
Authors: Gomez Daniel G. , Potts D. Gordon
Published: January 2005

The morphology of the choroid plexuses of the lateral, third, and fourth ventricles in dogs was studied by histological and ultrastructural techniques after in situ fixation. Most portions of the plexus in the lateral ventricle showed a parallel arrangement of capillaries, producing a fine corrugation of the overlying layer of epithelial cells. Relatively large amounts of connective tissue separated the capillaries from the epithelium. The fourth ventricle choroid plexus showed short capillary loops projecting into the ventricle and more intimately covered by epithelium. Smaller amounts of connective tissue separated the capillaries from the epithelium. The choroid plexus of the third ventricle showed characteristics seen in the plexuses of both the lateral and fourth ventricles. The total surface of the choroid plexuses in the dog averaged 10.7 cm2, of which 55% was fourth ventricle choroid plexus.
A family affected with intestinal polyposis and gliomas
Authors: Todd David W. , Christoferson Lee A. , Leech Richard W. , Rudolf Lou
Published: January 2005

The third affected family with Turcot syndrome is presented and the problems associated with the occurrence of familial aggregates of cancer are discussed. Turcot syndrome represents the unique and discrete occurrence of polyposis coli with glioblastoma multiforme, medulloblastoma, or both.
The effect of stimulus orientation on the visual evoked potential in multiple sclerosis
Authors: Camisa John , Mylin Leland H. , Bodis‐Wollner Ivan
Published: January 2005

Visual evoked potential (VEP) latency was measured in 89 patients with multiple sclerosis (MS) using both a checkerboard stimulus and an alternating vertical grating. Thirty‐seven patients had abnormal VEP latencies to the check stimulus, but 63 were classified as abnormal when the grating stimulus was employed. In an additional 22 MS patients, each eye was tested with a checkerboard stimulus, a vertical grating, and a horizontal grating to determine if edge orientation of the stimuli was responsible for the difference between grating and check diagnostic yields. The resulting diagnostic differences could be explained by taking into account the sinusoidal components of these stimuli. After equating gratings and checks for the fundamental Fourier component, each eye of 15 of the MS patients was retested with gratings in three orientations: vertical, horizontal, and oblique. Nine of the 15 patients had an orientation‐dependent abnormality of VEP latency. The results suggest that MS causes an orientational imbalance in the human visual system and that VEP testing with stimuli in more than one orientation can increase the diagnostic yield in MS patients.
Systemic and pulmonary vascular pressures during generalized seizures in sheep
Authors: Bayne L. L. , Simon R. P.
Published: January 2005

The systemic vascular response to seizures in known but information on the hemodynamics of the pulmonary circulation is lacking, clouding an understanding of the pathophysiology of postical pulmonary edema. We investigated this problem using electrically induced single seizures and bicuculline‐induced status epilecticus in halothaneanesthetized sheep. Aortic, pulmonary arterial, and left atrial pressures were recorded throughout the seizure activity, and the responses of the systemic and pulmonary pressures were compared. All monitored pressures rose precipitously with the onset of seizures. In all types of seizure the pulmonary pressure elevation was brief, lasting only one‐third as long as did the systemic. Pulmonary microvascular pressures rose well above the level of plasma oncotic pressure. Pulmonary vascular pressures were elevated in proportion to the duration of the seizure stimulus, while the systemic response did not so vary. Cervical spinal cord transection abolished all systemic and pulmonary vascular response to seizures, demonstrating ultimate neural mediation.
Isolation of herpes simplex virus type 1 in recurrent (Mollaret) meningitis
Authors: Steel J. Griffith , Dix Richard D. , Baringer J. Richard
Published: January 2005

Herpes simplex virus type 1 (HSV‐1) was isolated from the cerebrospinal fluid of a patient with benign recurrent aseptic meningitis (Mollaret meningitis). HSV‐1 may be one of several causes for this rare syndrome. The isolation of HSV‐1 from our patient serves to broaden the range of herpesvirus‐associated neurological disease.
Anterior decompression of the spine for metastatic epidural cord compression: A promising avenue of therapy?
Authors: Siegal Tali , Siegal Tzony , Robin Gordon , Lubetzki‐Korn Isabelle , Fuks Zvi
Published: January 2005

Most metastatic epidural tumors arise in a vertebral body and invade the anterior epidural space. Therefore, it is logical to decompress the spine anteriorly and not by traditional laminectomy. Surgical decompression is indicated if relapse occurs after radiotherapy and further radiation cannot be administered, if there is neurological deterioration during radiotherapy, and when histological diagnosis of the primary tumor is lacking. This pilot study consists of eleven consecutive anterior decompressions of the spine performed in nine patients. In seven instances other treatment modalities had been exhausted, and in four patients a tissue diagnosis was lacking. Before operation eight of the patients were nonambulatory, four of them paraplegic. Following decompression all but one patient became ambulatory. At operation the main bulk of the compressing tumor was found anterior or anterolateral to the cord. Spine stabilization was done when stability was a problem. Wound infection in one patient was the only postoperative complication. The encouraging outcome of our management prompts us to suggest that anterior decompression of the spine should be considered more often in metastatic compression of the cord and cauda equina.
Sylvian aqueduct syndrome caused by unilateral midbrain lesion
Authors: Auerbach Sanford H. , De Piero Theslee J. , Romanul Flaviu
Published: January 2005

A patient developed a syndrome of head tilt, limited upgaze, and convergence‐retractory nystagmus. Pathological examination revealed a single 0.6 cm diameter metastatic tumor nodule in the right tegmentum of the midbrain extending from the posterior commissure to a plane through the lower end of the lateral geniculate body. The unique features are the lesion's small size and the fact that it was unilateral.
Association between rise in serum sodium and central pontine myelinolysis
Authors: Norenberg Michael D. , Leslie Kevin O. , Robertson Andrew S.
Published: January 2005

Twelve hyponatremic patients with central pontine myeliolysis (CPM) showed a rise in serum sodium levels 3 to 10 days (mean, 6) prior to the development of CPM. The increase exceeded 20 mEq/L within 1 to 3 days and was then sustained for an additional 3 to 5 days. In addition, 11 of the 12 CPM patients achieved a sodium value of 147 mEq/L or greater during the period of sodium elevation. The rise in sodium frequently coincided with administration of saline solutions, diuretics, steroids, and lactulose. In contrast, 9 hyponatremic patients who did not have CPM showed sodium increases that were significantly less of slower (or both) following treatment of hyponatremia. Our findings suggest that CPM may be caused by a too rapid or excessive rise in serum sodium from a hyponatremic baseline.
Primary cerebellopontine progressive multifocal leukoencephalopathy diagnosed premortem by cerebellar biopsy
Authors: Jones H. Royden , Hedley‐Whyte E. Tessa , Freidberg Stephen R. , Kelleher Joseph E. , Krolikowski John
Published: January 2005

A subacute progressive cerebellar brainstem syndrome developed in a patient with systemic lupus erythematosus in remission. Cerebellar biopsy documented the diagnosis of progressive multifocal leukoencephalopathy (PML). Data from this patient and 10 others in the literature emphasize the need to consider this diagnosis when ataxia develops in any patient with underlying malignancy, chronic infection, or other disease that involves immunological incompetence. Although the ataxic form of PML is not of nosological relevance, early diagnosis may eventually hav therapeutic importance.
Glial outgrowth along spinal nerve roots in amyotrophic lateral sclerosis
Authors: Ghatak Nitya R. , Nochlin David
Published: January 2005

Formation of glial bundles in the proximal portion of the ventral nerve roots is described in a 51‐year‐old patient with the sporadic form of amyotrophic lateral sclerosis (ALS). Although the bundles were relatively fewer, they were identical in morphology and distribution to those consistently found in Werdnig‐Hoffmann disease (WHD). The occurrence of glial bundles in ALS, albeit rare, indicates that this phenomenon is not a unique feature of WHD. Similar changes have been observed in several other unrelated conditions, always in association with degeneration of neurons or axons. Thus, outgrowth of astrocytes in the form of glial bundles should be considered a special type of astrocytic reaction at the interface of the central and peripheral nervous systems.
Inborn errors of metabolism
Authors: Kolodny Edwin H. , Cable William J. L.
Published: January 2005

Inborn errors of metabolism often cause neurological dysfunction. These disorders are most common in childhood, but adult‐onset forms with a different clinical presentation are encountered, examples being Pompe disease, Tay‐Sachs disease, metachromatic leukodystrophy, Gaucher disease, and Maroteaux‐Lamy disease. In the evaluation of a patient with a possible inborn error of metabolism, simple screening tests may aid in the diagnosis and provide direction for more comprehensive laboratory analysis. In most cases, diagnosis can be established without a brain biopsy through biochemical variants of inherited metabolic disorders are being recognized through wider application of screening tests, improved specificity of laboratory analysis, cell complementation experiments, and the identification of enzyme activator factors. Accurate diagnosis is important for medical management, determining prognosis, and genetic counseling.
Presidential address: Thirty years of progress and problems in clinical neurology
Authors: Rowland Lewis P.
Published: January 2005

Research has transformed clinical neurology in the past thirty years, but we are faced with a paradox. Advances in basic sciences and better training for clinical investigators have accelerated the practical application of new knowledge. Now, just when neurological sciences are moving so rapidly, economic and other problems have threatened clinical investigation. It will take ingenuity and steadfast effort to continue research that is directly related to neurological diseases.
Neuroaxonal dystrophy in young adults: A clinicopathological study of two unrelated cases
Authors: Williamson Keith , Sima Anders, A. F. , Curry Bernadette , Ludwin Samuel K.
Published: January 2005

The clinical and pathological features of two unrelated sporadic cases of neuroaxonal dystrophy occurring in young adults are described. Initial clinical manifestations in both patients were those of primary psychiatric disorders. They subsequently developed extrapyramidal symptoms, dementia, cerebellar ataxia, and corticospinal dysfunction, but neither demonstrated myoclonic seizures. In one patient the diagnosis was made before death by brain biopsy. On pathological examination both patients showed a generalized distribution of spheroids within the central nervous system and, in one, in the peripheral nerves. In both patients, Lewy bodies were demonstrated in the pigmented brainstem nuclei. In addition, one patient showed ultrastructurally verified Lewy bodies in the cerebral cortex. Although these two cases could be considered examples of juvenile neuroaxonal dystrophy, we prefer to regard them as transitional forms in a disease spectrum with classic infantile neuroaxonal dystrophy and Hallervorden‐Spatz disease at the two extremes.
Regional cerebral blood flow and glucose metabolism following transient forebrain ischemia
Authors: Pulsinelli William A. , Levy David E. , Duffy Thomas E.
Published: January 2005

Progressive brain damage after transient cerebral ischemia may be related to changes in postischemic cerebral blood flow and metabolism. Regional cerebral blood flow (rCBF) and cerebral glucose utilization (rCGU) were measured in adult rats prior to, during (only rCBF), and serially after transient forebrain ischemia. Animals were subjected to 30 minutes of forebrain ischemia by occluding both common carotid arteries 24 hours after cauterizing the vertebral arteries. Regional CBF was measured by the indicator‐fractionation technique using 4‐iodo‐[14C]‐antipyrine. Regional CGU was measured by the 2‐[14C]deoxyglucose method. The results were correlated with the distribution and progression of ischemic neuronal damage in animals subjected to an identical ischemic insult. Cerebral blood flow to forebrain after 30 minutes of moderate to severe ischemia ( < 10% control CBF) was characterized by 5 to 15 minutes of hyperemia; rCBF then fell below normal and remained low for as long as 24 hours. Postischemic glucose utilization in the forebrain, except in the hippocampus, was depressed below control values at 1 hour and either remained low (neocortex, striatum) or gradually rose to normal (white matter) by 48 hours. In the hippocampus, glucose utilization equaled the control value at 1 hour and fell below control between 24 and 48 hours. The appearance of moderate to severe morphological damage in striatum and hippocampus coincided with a late rise of rCBF above normal and with a fall of rCGU; the late depression of rCGU was usually preceded by a period during which metabolism was increased relative to adjacent tissue. Further refinement of these studies may help identify salvageable brain after ischemia and define ways to manipulate CBF and metabolism in the treatment of stroke.
Periodic paralysis and the sodium‐potassium pump
Authors: Layzer Robert B.
Published: January 2005

Analysis of the pathophysiology of hypokalemic paralysis, as it occurs in barium poisoning, chronic potassium deficiency, and thyrotoxicosis, suggests that these disorders may have a similar mechanism. An increased ratio of muscle sodium permeability to potassium permeability reduces the ionic diffusion potential, while the resting membrane potential is sustained by an increase of Na‐K pump electrogenesis. The result is that potassium entry (the sum of active and passive influx) exceeds potassium efflux; this causes a large shift of extracellular potassium into muscle until the Na‐K pump turns off, leading to depolarization and paralysis. The primary defect in familial hypokalemic periodic paralysis, as in the example of barium poisoning, may be a marked reduction of muscle permeability to potassium.
Encephalomyeloneuropathy with ganglionitis of the myenteric plexuses in the absence of cancer
Authors: Horoupian Dikran S. , Kim Yehum
Published: January 2005

A 55‐year‐old woman presented with rapidly progressive brainstem dysfunction which led to death within a month. She also had constipation for three weeks, and barium enema showed ileus. Subacute encephalomyelitis predominantly involving the medulla and pons correlated with the patient's initial symptoms. In addition, ganglionitis of the myenteric plexuses explained the constipation and ileus. Ganglioradiculoneuropathy was another finding. The presence of abundant neuronophagia in the brainstem, dorsal root ganglia, and myenteric plexuses‐raised the speculation that a putative virus, toxic agent, or immune reaction possessed special affinity for neurons and ganglion cells. The neuropathological findings were similar to paraneoplastic changes, but no neoplasm was found.
A variant form of metachromatic leukodystrophy without arylsulfatase deficiency
Authors: Hahn Angelika F. , Gordon Bruce A. , Feleki Vera , Hinton George G. , Gilbert Joseph J.
Published: January 2005

The clinical, pathological, and biochemical findings in a young woman with a new variant of metachromatic leukodystropy (MLD) are reported. The patient showed slow early development and deteriorated further during her first two decades. Nerve conductions were slow, and a sural nerve biopsy showed features of a sulfatide lipidosis. Urinary sulfatide excretion was comparable to that of patients with classic MLD, yet in vitro activity of arylsulfatase A and B and cerebroside sulfatase activity were normal. Skin fibroblasts cultured in medium supplemented with 3H‐labeled sulfatide showed accumulation of labeled sulfatide in large amounts, implying a defect in sulfatide hydrolysis in vivo in spite of intact enzyme activity in vitro.
The role of the limbic system in experiential phenomena of temporal lobe epilepsy
Authors: Gloor Pierre , Olivier André , Quesney Luis F. , Andermann Frederick , Horowitz Sandra
Published: January 2005

Experiential phenomena occurring in spontaneous seizures or evoked by brain stimulation were reported by 18 of 29 patients with medically intractable temporal lobe epilepsy who were investigated with chronic, stereotaxically implanted intracerebral electrodes. The phenomena mainly consisted of perceptual (visual or auditory) hallucinations or illusions, memory flashbacks, illusions of familiarity, forced thinking, or emotions. Experiential phenomena did not occur unless a seizure discharge or electrical stimulation involved limbic structures. For such phenomena to occur, seizure discharge or electrical stimulation did not have to implicate temporal neocortex. This was true even for perceptual experiential phenomena. Many experiential responses elicited by electrical stimulation, particularly when applied to the amygdala, were not associated with electrical afterdischarge. Limbic activation by seizure discharge or electrical stimulation may add an affective dimension to perceptual and mnemonic data processed by the temporal neocortex, which may be required for endowing them with experiential immediacy.
Treatment of neuroectodermal brain tumors
Authors: Shapiro William R.
Published: January 2005

Prospective clinical trials support the view that malignant neuroectodermal tumors should be treated vigorously using a multimodal approach that includes surgical resection, high‐dose radiation therapy, and prolonged maintenance chemotherapy. Less malignant astrocytomas and oligodendrogliomas should be treated by resection and irradiation. Laboratory research on neuroectodermal tumors has advanced rapidly, providing new data on tumor cell biology and the pharmacology of chemotherapy that directly influence the therapeutic options.
The localizing value of depth electroencephalography in 32 patients with refractory epilepsy
Authors: Spencer Susan S. , Spencer Dennis D. , Williamson Peter D. , Mattson Richard H.
Published: January 2005

Clinical seizure manifestations, physical examination, radiological studies, neuropsychological tests, and scalp and depth electroencephalographic (EEG) studies were done to localize seizure foci in 32 patients, 23 of whom have undergone surgery with more than one year of follow‐up. Of 16 patients with unlocalized scalp EEGs, depth EEG detected a consistent focal seizure onset in 3. Of 15 patients with localized scalp EEGs, depth EEG revealed multiple foci in 3 and inaccurate localization of the focus by scalp EEG in 4. Therefore, localization by scalp EEG was inaccurate in 10 of 31 patients. When depth EEG revealed a consistent focal seizure onset and this localization was used to determine the surgical resection site, good to excellent results were obtained in 12 of 13 patients. When depth EEG revealed additional foci of seizure origin, surgical results were fair or poor in all patients. No other localizing criteria predicted depth EEG results or surgical success with such accuracy. Furthermore, some criteria, including neuropsychological tests and radiological abnormalities, were falsely localizing at times. Therefore, of the presently available localizing criteria, depth EEG appears to be the most accurate.
Use of SEPs to localize degeneration in a rare polyneuropathy: Studies on polyneuropathy associated with pigmentation, hypertrichosis, edema, and plasma cell dyscrasia
Authors: Shibasaki Hiroshi , Ohnishi Akio , Kuroiwa Yoshigoro
Published: January 2005

Peripheral and central nerve conduction were studied in five patients who had polyneuropathy associated with pigmentation, hypertrichosis, edema, and plasma cell dycrasia by using the short‐latency somatosensory evoked potential (SEP) to electrical stimulation of the median nerve at the wrist. The clavicular component N9 was markedly reduced inamplitude, and its latency was moderately prolonged. The interpeak latency from N9 to the cervical component N13 was significantly prolonged, indicating a conduction delay in the dorsal root. The interpeak latency from N13 to the cortical component N20 was normal, suggesting normal central conduction. These Physiological findings were consistent with the pathological findings, which showed degeneration of axons and segmental demyelination and remyelination in the sural nerve and extensive segmental demyelination and remyelination in the dorsal root but normal posterior columns of the spina cord. SEP is a noninvasive method of localizing the site of axon or myelin disorder in a peripheral neuropathy.
A placebo‐controlled trial shows no effect of vasopressin on recovery from closed head injury
Authors: Reichert Walter H. , Blass John P.
Published: January 2005

Vasopressin and its analogues have been claimed to benefit patients with posttraumatic amnesia. To test this hypothesis a double‐blind, placebo‐controlled, prospective trial of vasopressin was carried out in the chronic (rehabilitation) phase of recovery from closed head injury. Seven patients who received vasopressin and 6 who received a placbo were comparable in age, sex, and degree of disability upon entry into the study. Both groups inproved at the same rate in mental status, neurological status, and activities of daily living.
Spinocerebellar degeneration secondary to chronic intestinal malabsorption: A vitamin E deficiency syndrome
Authors: Harding A. E. , Muller D. P. R. , Thomas P. K. , Willison H. J.
Published: January 2005

Two adults are described who developed a progressive neurological disorder more than 20 years after the onset of chronic fat malabsorption. The clinical features included dysarthria, cerebellar ataxia, and prominent proprioceptive loss with depressed or absent tendon reflexes. Serum vitamin E was undetectable in both cases. One patient improved clinically and electrophysiologically after oral therapy with vitamin E. The findings in these patients were similar to those in others recently reported with vitamin E deficiency associated with biliary atresia. Electrophysiological observations suggested that the human deficiency state parallels that found neuropathologically in vitamin E‐deficient animals.
Persistent oligemia of rat cerebral cortex in the wake of spreading depression
Authors: Lauritzen Martin , Jøsrgensen Martin Balslev , Diemer Niels Henrik , Gjedde Albert , Hansen Anker Jóan
Published: January 2005

Cerebral blood flow during and after spreading depression (SD) was studied in rat brain by quantitative autoradiography. The rise of cortical blood flow during SD was followed by 20 to 25% reduction belwo normal after SD, lasting at least an hour. Blood flow in the putamen, thalamus, and hippocampus did not change at any time during or after SD. Previous measurements of cerebral blood flow in humans showed that migraine attacks may be accompanied by wavelike spreading oligemia (Olesen et al, 1981). We speculate that the spreading oligemia of migraine may be a phenomenon physiologically related to the present finding of an oligemia after SD.
Neuroimmunology II: Antigenic specificity of the nervous system
Authors: Weiner Howard L. , Hauser Stephen L.
Published: January 2005

Antigenic specificity of the nervous system refers to a property conveyed by unique cell surface that are present on different classes of nervous system tissue. These structures are a major importance for the study of nervous system structure and function, and can play a central role in determining patterns of nervous system injury. Thus, the major classes of nervous system cell are identified by structures that are unique to them: neurons by the presence of tetanus toxin receptors on their surface and oligodendrocytes by the presence of surface galactocerebroside, for example. With the advent of hybridoma technology, a large number of monoclonal antibodies are being identified which have increased by several orders of magnitude the ability to define subclasses of nervous system tissue according to unique antigens. In addition surface nervous system tissue may determine the specificity of nervous system injruy by (1) functioning as receptors for viruses or (2) being the targets of autoimmune responses. Patterns of viral injury to the nervous system are often extraordinarily selective (e.g., poliiviurs tropism for motor neurons), and nervous system viral tropism is due in some instances to the interaction of a virus with a unique surface antigen on neural cells. The specificity of injury in autoimmune disease (such as that against the acetylcholine receptor in myasthenia gravis) likewise must be explained by an immune response against unique system and other tisseus or between nervous system and infectious agents such as bacteria or viruses. The presence of shared antigenic structures between the nervous system and infectious agents creates the possibility that an immune response generated against a virus may concurrently damage normal nervous system tissue.
Interictal cerebral glucose metabolism in partial epilepsy and its relation to EEG changes
Authors: Engel Jerome , Kuhl David E. , Phelps Michael E. , Mazziotta John C.
Published: January 2005

Interictal positron computed tomography (PCT) with 18F‐fluorodeoxyglucose was performed on 50 patients with partial seizure disorders. Electroencephalographic (EEG) monitoring was carried out during the metabolic studies using scalp and sphenoidal electrodes in 33 patients and stereotaxically implanted depth electrodes in 17. Four patients in this series had focal abnormalities on x‐ray computed tomographic scans, but these were at the site of the presumed epileptogenic lesion in only 2. One or more discrete zones of hypometabolism were identified in 35 patients, and only 1 patient appeared to show focal interictal hypermetabolism. No quantitative relationship could be demonstrated between the degree of focal hypometabolism and either the frequency of intericatal EEG spikes or the presence of focal nonepileptifor EEg changes. It was concluded that metabolic and electrophysiological techniques measure different aspects of cerebral dysfunction in seizures diorders. Although interictal PCT in patients with partial epilepsy usually demonstrates zones of hypometabolism, this finding, perŕse, does not reveal the epileptic nature of the abnormality.
Comparative localization of foci in partial epilepsy by PCT and EEG
Authors: Engel Jerome , Kuhl David E. , Phelps Michael E. , Crandall Paul H.
Published: January 2005

One or more intericatal positron computed tomograms of 18F‐fluorodeoxyglucose were obtained on 50 patients with partial seizure disorders. Ictal as well as interictal electroencephalographic (EEG) data were available for all 50 patients, with scalp, sphenoidal, and depth electrode recordings done on 27 and scalp and sphenoidal recordings alone on 23. Thirty‐five patients demonstrated one or more abnormal intericatal zones of hypometabolism, while combined EEG studies were localizing for 36. There were considerable disagreements between the location of metabolic deficits and the epileptic focus revealed by individual scalp and depth EEG recorded ictal and intericatal epileptiform activity; however, there was good correlation between the site of focal hypometabolism and the epileptic focus determined by the combined results of all electrophysiological studies. When focal hypometabolism and focal nonepileptiform EEG abnormalites (i.e., slow waves and attenuation of fast rhythms) were both present in the same patient, their localization agreed completely. Metabolic and combined electrophysiological techniques both occasionally produced false positive as well as false negative results. When used together, the EEG can confirm that a hypometabolic zone is epileptogenic, while FDG scans may indicate whether an epileptic EEG focus represents a lesion or propagation from a distant site.
Diffuse axonal injury and traumatic coma in the primate
Authors: Gennarelli Thomas A. , Thibault Lawrence E. , Adams J. Hume , Graham David I. , Thompson Carson J. , Marcincin Robert P.
Published: January 2005

Traumatic coma was produced in 45 monkeys by accelerating the head without impact in one of three directions. The duration of coma, degree of neurological impariment, and amount of diffuse axonal injury (DAI) in the brain were directly related to the amount of coronal head motion used. Coma of less than 15 minutes (concussion) occurred in 11 of 13 animals subjected to sagittal head motion, in 2 of 6 animals with oblique head motion, and in 2 of 26 animals with full lateral head motion. All 15 concussed animals had good recovery, and none had DAI. Conversely, coma lasting more than 6 hours occurred in none of the sagittal or oblique injury groups but was present in 20 of the laterally injured animals, all of which were severely disabled afterward. All laterally injured animals had a degree of DAI similar to that found in severe human head injury. Coma lasting 16 minutes to 6 hours occurred in 2 of 13 in the sagittal group, 4 of 6 in the oblique group, and 4 of 26 in the lateral group; these animals had less neurological disability and less DAI than when coma lasted longer than 6 hours. These experimental findings duplicate the spectrum of traumatic coma seen in human beings and include axonal damage identical to that seen in severe head injury in humans. Since the amount of DAI was directly proportional to the severity of injury (duration of coma and quality of outcome), we conclude that axonal damage produced by coronal head acceleration is a major cause of prolonged traumatic coma and its sequelae.
Blink rates in parkinsonism
Authors: Karson Craig N. , Lewitt Peter A. , Calne Donald B. , Wyatt Richard Jed
Published: January 2005

Spontaneous eye blink rates were counted in 54 parkinsonian patients, 52 of whom were receiving levodopa. The mean blink rate of the more disabled patients, 7 per minute, was significantly less than the 17 blinks per minute observed in midly disabled (p < 0.02) and 22 blinks per minute (p < 0.001) in moderately disabled patients. Drug‐induced dyskinesia in 21 patients was associated with an elevated blink rate, 32 per minute, as compared with 12 per minute for non‐dyskinetic patients (p < 0.01).
The effects of dexamethasone administration and withdrawal on water permeability across the blood‐brain barrier
Authors: Reid Alison C. , Teasdale G. M. , McCulloch J.
Published: January 2005

We examined the effects of dexamethasone administration and withdrawal on regional cerebral blood flow (CBF) and the permeability surface area (PS) product for water in the central nervous system of conscious rats. There were no significant changes in CBF. Dexamethasone treatment produced a significant decrease in the PS product for water in the cerebral cortex, while withdrawal of dexamethasone resulted in a significant increase. Water content of the cerebral cortex was also increased in rats from which dexamethasone had been withdrawn. These findings may help in understanding the pathogenesis of brain swelling in some patients.
Neuron loss in the nucleus basalis of meynert in parkinsonism‐dementia complex of Guam
Authors: Nakano Imaharu , Hirano Asao
Published: January 2005

The nucleus basalis of Meynert, which supplies diffuse cholinergic fibers to the cerebral neocortex, was investigated in two cases of parkinsonism‐dementia complex of Guam (PDG). The nucleus basalis of the two PDG patients showed extensive neuron loss when compared with age‐matched non‐Guamanian controls, suggesting the necessity of investigating activity of cholinergic enzymes in the brains of patients with PDG.
Immunocytochemical comparison of neurofibrillary tangles in senile dementia of Alzheimer type, progressive supranuclear palsy, and postencephalitic parkinsonism
Authors: Yen Shu‐Hui , Horoupian Dikran S. , Terry Robert D.
Published: January 2005

Antiserum raised against a two‐cycle‐purified human brain microtubule fraction that binds specifically to tangles of senile dementia of the Alzheimer type was tested on the subthalamus, basis pontis, and midbrain sections of two patients with progressive supranuclear palsy. Tangles composed of 15 nm straight fibers in these areas showed positive immunolabeling with the antiserum. The labeling tangles appeared as a fibrous meshwork in neuronal cell bodies and occasionally in short segments of axons. Corresponding sections of brains obtained from patients with idiopathic parkinsonism, striatonigral degeneration, and olivopontocerebellar atrophy treated with the same antiserum did not display peroxidase products. Only in the elderly subjects and patients with postencephalitic parkinsonism did the periaqueductal gray and median raphe show occasional cells that reacted positively with the antiserum. Tangles in the substantia nigra of patients with postencephalitic parkinsonism were also recognized by the antiserum. The results suggest that the two morphologically distinct neurofibrillary tangles share their immunological properties with components present in microtubule fractions of normal brain.
Cerebrospinal fluid monoamine metabolites in narcolepsy and hypersomnia
Authors: Faull Kym F. , Guilleminault Christian , Berger Philip A. , Barchas Jack D.
Published: January 2005

Two groups of patients with the common complaint of excessive daytime sleepiness were examined. One group fulfilled the criteria for narcolepsy. The other had the sole symptom of excessive daytime sleepiness, confirmed by sleep latency tests, which was unaccompanied by systemic illness. Spinal fluid examinations for homovanillic acid, 3,4‐dihydroxyphenylacetic acid, 3‐methoxy‐4‐hydroxyphenylethylene glycol, and 5‐hydroxyindoleacetic acid were carried out before and after administration of probenecid on the 20 patients and 40 controls. Probenecid‐corrected accumulation of the dopamine metabolites was significantly increased in each of the patient groups, suggesting an association between pathological sleepiness and elevated dopamine turnover in the central nervous system.
Axonal atrophy in sensory nerves of the diabetic BB‐Wistar rat: A possible early correlate of human diabetic neuropathy
Authors: Sima Anders A. F. , Bouchier Mark , Christensen Hilary
Published: January 2005

Clinical, morphological, and biochemical findings reported in the spontaneously diabetic BB‐Wistar rat strongly indicate that this animal may be a true model of human insulin‐dependent diabetes mellitus. As such, it may provide a valuable model in which to study the neuropathic conditions of diabetes. We examined somatic peripheral nerves at five levels in a longitudinal fashion using quantitative morphological techniques. Myelinated fiber atrophy occurred earlier in sensory nerves than in motor nerves and showed a distal to proximal progression with duration of diabetes. Axon/myelin ratios revealed disproportionate shrinkage of axons evident already after 4 months of diabetes in the sural nerve and only after 8 months in the peroneal nerve. Endoneurial edema could not be demonstrated by morphometric means in diabetic nerves. We conclude that the distal symmetrical polyneuropathy in diabetes can be characterized as a mainly sensory axonopathy of dying‐back type.
Abnormalities of erythrocytes in duchenne muscular dystrophy
Authors: Yoshida Mizuko , Ando Kazuya , Satoyoshi Eijiro
Published: January 2005

Erythrocytes from patients with Duchenne muscular dystrophy (DMD) were studied in relation to the extracellular calcium ion concentration. The shape of fresh erythrocytes in DMD was normal and not affected by Ca++. When DMD erythrocytes were stored in buffer solution with Ca++, however, the rate of the shape change over time was enhanced compared with that of cells from normal controls. The calcium content of DMD erythrocytes stored in buffer solution with Ca++ and dextrose was also two and one‐half times that of control cells. In contrast, when DMD erythroytytes were stored in buffer stored in buffer solution with dextrose but without Ca++, no differences from controls were observed. The adenosine triphosphate content of DMD erythrocytes stored in the buffer solution was not affected by Ca++ concentration.
Passive joint position sense after total hip replacement surgery
Authors: Karanjia Percy N. , Ferguson John H.
Published: January 2005

Passive joint position sense was tested in 10 subjects after unilateral total hip replacement surgery (which included capsulectomy). Varied initial limb position, amplitude, and velocity of movement were used. The unoperated side was used as a control. Statistically barely significant errors (p < 0.025) on the operated side were detected at 0.6° per second but not at 2° per second. No significant differences were noted among the different hip positions.

The results indicate that the ability to detect passive hip joint position sense remains largely, but not completely, intact after hip joint replacement. The joint capsule, ligaments, and joint surfaces, although not essential for kinesthesia, may provide some limb movement and position information, the absence of which is not now clinically apparent.
Na+ ‐ and K+‐dependent adenosine triphosphatase changes in multiple sclerosis plaques
Authors: Hirsch Hilde E. , Parks Mary Ellen
Published: January 2005

Na+ and K+ ‐dependent adenosine triphosphatase [(Na+ + K+)‐ATPase] was studied in microdissected samples from sections of histologically active and inactive plaques of multiple sclerosis. In active plaques the enzyme was found to be markedly reduced in activity, whereas in old, chronic plaques ATPase activities were higher than those of unaffected white matter. White matter from control patients and normal‐appearing white matter from MS patients did not differ with regard to ATPase activities. The results suggest that reversible changes in the sodium pump mechanism may be involved in the pathophysiology of multiple sclerosis.
Specific messenger RNA changes in joseph disease cerebella
Authors: Morrison Marcelle R. , Rosenberg Roger N.
Published: January 2005

Joseph disease is an autosomal‐dominant, spinocerebellar degeneration characterized at the biochemical level by elevations in the steady‐state levels of several abundant proteins (H, J, and L) in affected brain areas such as the cerebellar cortex. The increased levels of these proteins could either be a consequence of a relative increase in their de novo synthesis or result from altered rates of proteolysis in degenerating brain cells. These alternatives can be distinguished by comparing the in vitro protein‐synthetic capacities of the messenger ribonucleic acid populations isolated from cerebellar cortex of control subjects and patients with Joseph disease. Protein H (glial fibrillary acidic protein) is synthesized at detectable levels by all messenger ribonucleic acid isolates, and the levels of its translatable messenger ribonucleic acid are reproducibly increased in ribonucleic acids isolated from cerebellar cortex of patients with Joseph disease as compared with those isolated from cerebellar cortex of control subjects. Thus, the increased level of protein H in Joseph disease is a consequence of an increase in its de novo synthesis and is correlated with the increased number of cerebellar glial cells. In contrast to these results, there is no detectable synthesis of proteins J and L by messenger ribonucleic acid populations isolated from cerebellar cortex of either Joseph disease patients or control subjects, suggesting that the increased levels of these proteins in affected cerebellar cortex are a consequence of posttranslational protein modifications.
Regional blood‐to‐tissue transport in ethylnitrosourea‐induced brain tumors
Authors: Blasberg Ronald G. , Kobayashi Tomowo , Horowitz Marc , M Jerry M. , Groothuis Dennis , Molnar Peter , D Joseph D.
Published: January 2005

Regional blood‐to‐tissue transport, expressed as a unidirectional transfer rate constant (K), was measureed in experimental brain tumors using alpha‐aminoisobutyric acid (AIB) labeled with carbon 14 and quantitative autoradiography. A total of sixteen oligodendrogliomas, four mixed gliomas, three astrocytomas, two diffuse gliomatoses, one anaplastic astrocytoma, one ependymoma and four malignant schwannomas were studied in 9 rats. The mean Ks for all glioma classifications were similar, averaging 2.6 ± 0.4 (standard error of the mean) ml · kg−1 min−1, and were only slightly higher than those for nontumorous parietal cortex (2.1 ml · kg−1 · min−1), corpus callosum (0.9 ml · kg−1 min−1), and a comparable region of brain tissue in the contralateral hemisphere (1.3 ml · kg−1 · min−1). Values of K varied minimally in the intracerebral gliomas and were marginally correlated with tumor cell morphology in only two tumors. In some (but not all) of the larger gliomas, increased vascularity, with or without endothelial proliferation, was associated with a 3‐to 15‐fold increase in K. Regional K values in malignant schwannomas were highly variable (4 to 207 ml · kg−1 · min−1) and generally were not correlated with specific histological features of the tumor, except in some regions with increased vascularity. Estimates of the average fractional extraction of AIB by the intracranial gliomas and malignant schwannomas were 0.01 and 0.2, respectively; average fractional extractions for nontumorous brain were approximately 0.003.