bluuebunny/crossref_metadata_2025 huggingface dataset:
Effects of bicuculline‐induced seizures on cerebral metabolism and circulation of rats rendered hypoglycemic by starvation
Authors: Blennow G. , Folbergrová J. , Nilsson B. , Siesjö B. K.
Published: January 2005

To evaluate the effects of substrate deficiency on cerebral function, metabolism, and blood flow during seizures, rats were injected intravenously with bicuculline (1.2 mg · kg−1) following a 24‐hour period of starvation. During the course of seizures, blood glucose concentrations fell, and when they were reduced to below about 3 μmol · gm−1, cerebral function, metablism, and blood flow altered. Changes in function involved the transition of an electroencephalographic pattern of bursts and suppression into one of frequent or sparse single spikes. Oxygen consumption, which initially increased at least twofold, fell toward normal or subnormal values in the single‐spike period. Cortical blood flow was markedly reduced, and there was an attenuated response to carbon dioxide administration. Simultaneously, a small but clear fall was detected in the cerebral phosphorylation potential, and concentrations of glycolytic metabolites (including lactate) and citric acid cycle intermediates were reduced. Changes in amino acids and ammonia were somewhat similar to those observed in insulin‐induced hypoglycemia, but since the amino acid pool did not fall, the experiments failed to give evidence that amino acids serve as oxidative substrates.

The perturbation of cerebral energy state (and of levels of carbohydrate substrates and amino acids) was reversed by glucose administration; but since neither this procedure nor additional bicuculline injections could cause resumption of continuous seizure activity, the results suggest that cellular substrate depletion may have given rise to a sustained disturbance of synaptic transmission.
Position and vibration sensations: Functions of the dorsal spinocerebellar tracts?
Authors: Ross Elliott D. , Kirkpatrick Joel B. , Lastimosa Augusto C. B.
Published: January 2005

Clinical evidence is presented which establishes for the first time that position and vibration sensations may be carried in the dorsal spinocerebellar tracts. We present a patient who incurred a spinal cord infarction eight months prior to death that caused a Brown‐Séquard syndrome with loss of position and vibration senses in the right lower extremity. The infarction spared the right fasciculus gracilus and thus supports the recent observations in human beings (and animals) that conscious proprioception is not a function of the dorsal columns. The vibratory and position losses were best correlated with the portion of the lesion involving the right dorsal spinocerebellar tract. Arguments are furnished based on physiological experiments in animals that conscious proprioception may be carried by Morin's spinocervicothalamic pathway, which forms part of the dorsal spinocerebellar tract in the spinal cord.
Substances which modulate leukocyte migration are present in CSF during meningitis
Authors: Wyler David J. , Wasserman Stephen I. , Karchmer Adolf W.
Published: January 2005

We studied the effects on in vitro leukocyte migration of cerebrospinal fluid from 14 patients with and 7 patients without meningitis. Meningitic CSF, but not control CSF, contained activity that was chemotactic for peripheral blood leukocytes. In an unusual case of staphylococcal meningitis associated with a blunted CSF pleocytosis and a poor clinical response to antibiotics, an inhibitor of leukocyte chemotaxis was identified in the CSF. This inhibitor may have diminished local leukocyte accumulation in vivo and thereby adversely affected host defence mechanisms and clinical outcome. No inhibitors were found in other CSF samples tested. Soluble mediators present in CSF may modulate the local accumulation of leukocytes and therefore affect host defenses in meningitis.
Dermatomyositis and toxoplasmosis
Authors: Hendrickx G. F. M. , Verhage J. , Jennekens F. G. I. , van Knapen F.
Published: January 2005

In a patient with childhood dermatomyositis, high toxoplasma antibodies were found at the time of diagnosis. A direct immunofluorescence technique demonstrated active toxoplasmosis in the muscle biopsy. The response to treatment and follow‐up in this patient suggest that toxoplasmosis could have caused the dermatomyositis.
Activity of 2′,3′‐cyclic nucleotide 3′‐phosphohydrolase in human cerebrospinal fluid
Authors: Banik Naren L. , Mauldin Lawrence B. , Hogan Edward L.
Published: January 2005

Activity of the myelin marker enzyme 2′,3′‐cyclic nucleotide 3′‐phosphohydrolase (CNP) was assayed in cerebrospinal fluid samples obtained from patients with multiple sclerosis (MS) and other neurological diseases. The enzyme activity was found to be elevated in acute cases of MS and reduced during remission. It was present in other demyelinating diseases, and no activity was detected in normal CSF. CNP may be released into CSF from any insult to myelin. The level of activity appears to reflect demyelination and the rate of breakdown of the myelin sheath.
Endoneurial fluid pressure in wallerian degeneration
Authors: Powell H. C. , Myers R. R. , Costello M. L. , Lampert P. W.
Published: January 2005

Endoneurial fluid pressure (EFP) was recorded by an active, servo‐null pressure system after a glass micropipette was inserted into rat sciatic nerve undergoing wallerian degeneration. The lesions were produced by crushing the left sciatic nerve of the anesthetized animal at its point of entry into the thigh. Eighty‐four animals were employed in this experiment, in which EFP was recorded from sham‐operated rats and other controls as well as from rats with wallerian degeneration. The experiment was designed so that EFP could be recorded from 2 or more experimental animals at daily intervals starting at day 0 and concluding on day 28. Pressure progressively increased during the first week, reaching a peak elevation four to five times normal. The subsequent decline in EFP was more gradual, with values approaching normal during the third week after injury. Linear regression analysis showed the progressive increase in EFP to be statistically significant (p ≤ 0.01). To determine the time at which EFP was maximum, we used the Marquardt computer algorithm for least‐squares estimation of nonlinear variables. By this procedure the peak value for EFP occurred at six days. These biophysical observations were correlated with subsequent microscopic examination of 1 μ thick sections of Araldite‐embedded sciatic nerve. Microscopy confirmed the presence of wallerian degeneration associated with edema, which was observed in every instance of elevated EFP.
The CSF‐FTA test and the significance of blood contamination
Authors: Davis Larry E. , Sperry Sally
Published: January 2005

Although the fluorescent treponemal antibody absorption (FTA‐ABS) test has found wide application in the diagnosis of syphilis, the cerebrospinal fluid fluorescent treponemal antibody (CSF‐FTA) test is still controversial in the diagnosis of neurosyphilis. We found the CSF‐FTA test extremely sensitive to contamination with FTA‐ABS‐reactive blood at concentrations in CSF that could not be detected by visual inspection. Interpretation of a reactive test should be made with caution if red cells are present in CSF.
Visual evoked potentials in the evaluation of “cortical blindness” in children
Authors: Frank Yitzchak , Torres Fernando
Published: January 2005

Visual evoked responses (VERs) to brief light flashes were recorded from occipital regions in a group of 30 “cortically blind” children aged 4 months to 15 years and were compared with those of 31 children of similar age range who had the same type of central nervous system diseases but without signs or symptoms of blindness. The VERs were analyzed for amplitude, number of peaks, and morphology, follwing a method used previously by other authors. All the responses had some degree of abnormality, but there were no significant differences between the two groups. The findings suggest that recording of VERs is not always an accurate method for diagnosis of cortical blindness in children since the presence of abnormal responses in not incompatible with normal vision. Only 1 patient with cortical blindness showed no response.
Presence of lymphocyte membrane surface markers on “small cells” in a pineal germinoma
Authors: Neuwelt Edward A. , Smith R. Graham
Published: January 2005

A 16‐year‐old boy was operated on via an occipital transtentorial craniotomy for a pineal tumor. Routine histological examination of the tissue revealed it to be a germinoma, as characterized by the presence of two cell populations: large cells and small, lymphoidappearing cells. The cells were evaluated in a singlecell suspension for the peresence of lymphocyte membrane surface markers; small cells exclusively were found to have such markers; A T‐lymphocyte membrane marker was present in 81% of the small cells, whereas 15 to 18% of the small cells had a Blymphocyte membrane marker.
High‐dose barbiturate therapy in humans: A clinical review of 60 patients
Authors: Rockoff Mark A. , Marshall Lawrence F. , Shapiro Harvey M.
Published: January 2005

Over the past three years, high‐dose barbiturate therapy has been used in the treatment of 60 patients with head injury (N = 45), encephalitis (N = 8), acute focal cerebral ischemia (stroke, N = 4), and global anoxia secondary to drowning (N = 3). High‐dose barbiturates appear to be useful adjuncts in the control of intracranial hypertension refractory to other methods of therapy. Administration of barbiturates to patients with this problem will often reduce the requirement for osmotic agents, thereby facilitating medical management by avoiding hyperosmolality and fluid and electrolyte depletion. In a carefully controlled intensive care setting the risk of barbiturate therapy is low, though the costs and demands on personnel are great. Survival appeared to be improved in patients with head injury and encephalitis. Although the ultimate outcome was not altered in patients with stroke or near‐drowning, intracranial hypertension did not occur until barbiturate therapy was withdrawn. This experience provides an ethical basis to justify further randomized studies for determining whether or not barbiturates materially improve the neurological outcome following cerebral ischemic and traumatic insults.
Effects of physostigmine and lecithin on memory in Alzheimer disease
Authors: Peters Bruce H. , Levin Harvey S.
Published: January 2005

Because there is evidence that central cholinergic mechanisms are depleted in dementia, we studied the effects of central cholinergic augmentation on the memory of 5 patients with Alzheimer disease. Patients received placebo, lecithin, physostigmine, or lecithin plus physostigmine in a double‐blind study using titrated doses of the acetylcholinesterase inhibitor physostigmine. Memory was evaluated with alternate forms of the selective reminding procedure. Compared with lecithin alone, the combination of physostigmine and lecithin consistently enhanced memory storage and retrieval; physostigmine without lecithin produced no memory facilitation. The strategy of combining a cholinergic agonist and precursor holds promise, although a larger clinical trial is needed.
Adult type neuronal storage disease with neuraminidase deficiency
Authors: Miyatake Tadashi , Atsumi Tetsushi , Obayashi Taminori , Mizuno Yoshikuni , Ando Susumu , Ariga Toshio , Matsui‐Nakamura Kyoko , Yamada Takamichi
Published: January 2005

We describe a patient with adult‐onset neuronal storage disease characterized by myoclonus, cerebellar ataxia, convulsive seizures, cherry‐red spots, skeletal dysplasia, mild gargoyle features, inguinal hernia, and angiokeratoma. Cytoplasmic inclusions consistent with lysosomal storage disease were demonstrated in neurons of the autonomic nervous system. Accumulation of GM3 and GM2 gangliosides was found in sympathetic ganglia but a catabolic disturbance of these gangliosides was ruled out by normal levels of GM3 ganglioside sialidase and N‐acetyl‐β‐hexosaminidase A activities. β‐Galactosidase activity was decreased in leukocytes and fibroblasts, but not in serum. GM1 gangliosidosis was ruled out by lipid analyses, and mucopolysaccharidosis by normal excretion of mucopolysaccharide in urine.

Sialyl oligosaccharides were increased in urine and α‐neuraminidase was deficient in fibroblasts. This disorder is considered to be an inherited metabolic disorder of sialyl glycoproteins and oligosaccharides due to deficiency of an α‐neuraminidase.
Disturbances of rapid‐eye‐movement sleep in 3 brothers with Pelizaeus‐Merzbacher disease
Authors: Niakan Enayatolah , Belluomini Joseph , Lemmi Helio , Summitt Robert L. , Ch'ien Lawrence
Published: January 2005

The electrophysiological features of 3 brothers with the classic form of Pelizaeus‐Merzbacher disease (PMD) were studied. Two consecutive overnight polygraphic sleep recordings indicated a gross distortion of rapid‐eye‐movement (REM) sleep for all patients. A lower than normal percentage of REM sleep in these patients was consistent with their retarded intellectual development, which supports current thinking that REM sleep may be a sensitive index of brain function integrity.

Non‐rapid‐eye‐movement (NREM) sleep, in contrast to reported findings in 1 patient with PMD, was uniformly characterized by distinct stages in which the electroencephalograms contained frequent vertex waves and spindles. Tests of peripheral nerve conduction velocity, acoustic brainstem reflexes, and visual and auditory evoked potentials did not indicate any abnormalities, nor did electroencephalograms obtained during wakefulness. One patient did have epileptifrom spikes and spike waves recorded during an all‐night EEG, an unusual finding in a child with cerebral white matter disease.
Brainstem auditory evoked response in adrenoleukodystrophy
Authors: Black James A. , Fariello Ruggero G. , Chun Raymond W.
Published: January 2005

Clinical, laboratory, and electrophysiological data, including brainstem auditory evoked responses, are reported in a case of adrenoleukodystrophy. A striking asymmetry was noted in wave VI of the brainstem auditory evoked potential, followed by absence of any recognizable wave on the abnormal side. The presumed site of origin of wave VI is the medial geniculate body, a structure severely involved in adrenoleukodystrophy. It is suggested that the brainstem auditory evoked response may promise noninvasive diagnostic aid in this disorder and that absence of wave VI may emerge as a clinically useful finding in diseases of the central nervous system.
Dementia in Parkinson disease
Authors: Lieberman Abraham , Dziatolowski Marie , Kupersmith Mark , Serby Michael , Goodgold Albert , Korein Julius , Goldstein Menek
Published: January 2005

In 520 patients with parkinsonism seen over eight years, 168 (32%) had moderate to marked dementia. Although the demented patients were older than the nondemented patients (70.4 versus 65.5 years), the incidence of dementia in Parkinson's disease (PD) was tenfold higher than among controls (similarly aged spouses of PD patients), and dementia is held to be related more to the disease than to age.

Demented patients, in addition to being older, developed PD later, were more severely involved in a shorter time, and responded less well to levodopa. It is suggested that PD with dementia may represent a different disorder from PD without dementia.
Midbrain asterixis
Authors: Bril Vera , Sharpe James A. , Ashby Peter
Published: January 2005

Asterixis is usually a manifestation of metabolic encephalopathy. It was the only skeletal motor sign in a patient with ophthalmoplegia caused by midbrain infarction; no metabolic abnormality was present. The asterixis was accompanied by signs of damage to the mesencephalic reticular formation. We propose that episodic lapses of postural control by the reticular formation are responsible for midbrain asterixis and suggest that this asterixis is a segmental form of drop attack.
Incomplete versus complete cerebral ischemia: Improved outcome with a minimal blood flow
Authors: Steen Petter A. , Michenfelder John D. , Milde James H.
Published: January 2005

It has been reported that incomplete cerebral ischemia with cerebral blood flow less than 10% of control may be more damaging than an equal period of complete ischemia. In this study, the effects of severe, incomplete cerebral ischemia on neurological outcome and cerebral metabolism were studied in dogs anesthetized with nitrous oxide. The results were compared with those of a previous study concerned with the effects of complete ischemia. Dogs could sustain only 8 to 9 minutes of complete ischemia with return of normal neurological function, whereas maintenance of a cerebral blood flow rate less than 10% of control extended this limit to 10 to 12 minutes. Following a 10‐minute exposure, only dogs undergoing incomplete ischemia regained a normal cerebral oxygen consumption within 90 minutes; similarly, animals subjected to incomplete ischemia enjoyed a faster return of EEG activity than dogs exposed to complete ischemia of the same duration. Cerebral metabolite levels did not prove to be a good index of return of neurological function. Within periods of cerebral ischemia in which meaningful neurological recovery might be expected, we conclude that some blood flow is better than no flow.
Chronic progressive myelopathy: Investigation with CSF electrophoresis, evoked potentials, and CT scan
Authors: Paty D. W. , Blume W. T. , Brown W. F. , Jaatoul N. , Kertesz A. , McLnnis W.
Published: January 2005

Chronic progressive myelopathy (CPM) is difficult clinical problem. Many patients who present with CPM turn out to have a spinal form of multiple sclerosis (MS), but until there is clear lesion dissemination, a definite clinical diagnosis cannot be made. We have looked for MS‐related abnormalities in 72 patients with CPM. The mean age of onset was 42 years, mean duration was ten years, and mean Kurtzke disability rating was 4.5. Studies performed were cerebrospinal fluid electrophoresis for oligoclonal banding, pattern‐reversal visual evoked respones, blink reflex latencies, and computerized axial tomography.

Oligoclonal banding was found in 32 patients (44%), patterned visual evoked responses were abnormal in 32 (44%), and blink reflex latencies were abnormal in 40 (56%). At least one of these studies was abnormal in 61 patients (85%) and at least two in 48 (66%). The CT scan was abnormal in 38 (53%), 36 with atrophy and 3 with low‐density or enhancing lesions. These results suggest that at least 44% of patients with CPM may have MS that could be diagnosed by oligoclonal bands. Other physiological tests suggesting diffuse or disseminated disease bring the total to 85%. Only autopsy follow‐up will tell us the exact diagnostic accuracy of these studies in this complex syndrome.
Penile prosthesis for impotence in multiple sclerosis
Authors: Massey E. Wayne , Bernard Pleet A.
Published: January 2005

Loss of libido and impotence are common problems for patients with multiple sclerosis. A penile implant may correct one aspect of sexual dysfunction in selected males.
Cerebellar degeneration due to chronic phenytoin therapy
Authors: William McLain L. , Martin J. True , Allen Joseph H.
Published: January 2005

Five patients developed cerebellar degeneration while being treated with phenytoin. All had high plasma levels of the drug, and none was having seizures of a type that could have caused systemic hypoxia at the time the cerebellar syndrome appeared. Cerebellar degeneration was confirmed by the finding of atrophy on CT scan and by persistence of cerebellar signs when plasma phenytoin levels were decreased. We suggest that chronic phenytoin therapy can cause cerebellar degeneration.

The question of whether phenytoin or the cumulative effect of hypoxia from repeated convulsions causes cerebellar degeneration should not be posed as one of exclusive alternatives, since hypoxia is a well‐known cause of cerebellar atrophy. Instead, the question should be whether or not phenytoin can also be responsible. The cases reported here suggest that it can.
Molecular features of immunoreactive myelin basic protein in cerebrospinal fluid of persons with multiple sclerosis
Authors: Bashir Rifaat M. , Whitaker John N.
Published: January 2005

The molecular weight of the myelin basic protein (BP)‐like material appearing in cerebrospinal fluid (CSF) of 2 persons with active‐phase multiple sclerosis (MS) and 3 with recent cerebral infarctions was determined by gel filtration chromatography under acidic conditions. Column fractions were examined by radioimmunoassay for BP peptide 43‐88 antigen as well as for binding activity to BP. Material cross‐reactive with BP peptide 43‐88 eluted over a wide spectrum of molecular weights. Greater representation of lower molecular weight species occurred in MS. Fractionated CSF from 2 patients with MS and 2 with cerebral infarction contained binding activity, presumably antibody, to BP which was greater than that in unfractionated CSF. Binding to BP was also examined in unfractionated CSF from 70 persons, including 16 with MS and 36 with other neurological diseases. Although CSF from MS patients showed higher binding, the presence of BP binding activity was not specific for MS. Our findings suggest that the antigen cross‐reacting with BP and its fragments in CSF appears in a spectrum of molecular weight species, with lower forms predominating in MS. The detection of binding activity to BP as well as its enhancement by treatment at low pH suggest that the BP‐like antigen in CSF may exist in free as well as in complexed forms.
Mechanisms underlying epileptiform burst discharge
Authors: Schwartzkroin P. A. , Wyler A. R.
Published: January 2005

Studies of hyperexcitable cells in alumina‐gel and penicillin epileptogenic foci suggest that epileptic neurons have intrinsic burst‐generating properties. The burst mechanism may be released or triggered by a number of extrinsic factors which disrupt the normal depolarizing‐hyperpolarizing balance. This paper presents the hypothesis that the most important influence for burst discharge is influx of calcium ions. This excitatory effect is modulated by synaptic inputs, hyperpolarazing conductances, cell morphology and membrane characteristics, and the extracellular milieu. The hypothesis incorporates findings from both penicillin and alumina models of epilepsy and provides experimentally testable concepts for further investigation.
Intraventricular hemorrhage in the high‐risk preterm infant: Incidence and outcome
Authors: Ahmann Peter A. , Lazzara Anthony , Dykes Francine D. , Brann Alfred W. , Schwartz James F.
Published: January 2005

To determine the incidence of subependymal (SEH) or intraventricular hemorrhage (IVH) and its short‐term outcome, infants of less than 35 weeks' gestation who required intensive care were evaluated and computerised tomographic scans obtained. If the scans showed blood, serial scans were followed until the hemorrhage had resolved and ventricle size was stable. Hemorrhage was quantitated.

Seventy‐seven of 191 (40.3%) infants were shown to have SEH, IVH, or both; 22 of them (28%) died, and hemorrhage was thought to be the primary cause of death in 17. Fifty‐five survivors (71%) with SEH, IVH, or a combination of the two had serial follow‐up scans. Six had SEH alone; 49 had IVH. Severe progressive hydrocephalus developed in 12 (22%) infants. Thirty‐seven (75.5%) did not show progressive hydrocephalus. The degree of hemorrhage in these 37 was mild in 14, moderate in 13, and marked in 10. Of those with progressive hydrocephalus, hemorrhage was marked in 8 and moderate in 4. Hydrocephalus resolved spontaneously in 4 of the 12. Medical treatment (repeated lumbar punctures) was successful in 3, but failed in 4. Hydrocephalus was managed by shunt surgery in 5.

This study revealed that the quantity of blood is prognostically important with regard to both survival (p < 0.001) and development of progressive hydrocephalus (p < 0.05). Furthermore, hydrocephalus, even if progressive, may not necessitate surgical management.
Cerebellar involvement in multifocal eosinophilic granuloma: Demonstration by computerized tomographic scanning
Authors: Adornato Bruce T. , Eil Charles , Head Gordon L. , Loriaux D. Lynn
Published: January 2005

Central nervous system involvement outside the hypothalamus or pituitary in multifocal eosinophilic granuloma (MEG) is unusual. Eleven patients with MEG have been examined with cranial computerized axial tomograms (CT). Four patients with moderate to severe cerebellar dysfunction, 3 of whom had no detectable lesions by other neuroradiological techniques, were found to have cerebellar abnormalities. All of the remaining 7 patients with normal neurological examinations had normal CT scans. Computerized axial tomography is a useful technique in the evaluation of patients with MEG and neurological impairment.
Sarcoid polyneuropathy: A histologically proved case
Authors: Oh Shin Joong
Published: January 2005

A 58‐year‐old patient had subacute polyneuropathy Electrophysiological studies showed inexcitability of the distal portion of the peroneal and posterior tibial nerves and minimal reduction in amplitude of the muscle potential with minimal slowing in motor nerve conduction velocity. The sural nerve biopsy showed multiple sarcoid granulomas in the epineurial and perineurial spaces, periangiitis and panangiitis, and axonal degeneration. Remarkable clinical improvement followed corticosteroid treatment.
Absence of REM and altered NREM sleep in patients with spinocerebellar degeneration and slow saccades
Authors: Osorio Ivan , Daroff Robert B.
Published: January 2005

The pontine tegmentum contains the neurons responsible for generation of saccadic eye movements and certain phases of sleep. We studied two genetically unrelated patients with spinocerebellar degeneration and slow saccadic eye movements. Multiple all‐night sleep studies in both patients disclosed absence of REM and stage 4 sleep with an extremly short stage 3 and long stage 2. Both patients had a sleep stage (X) not previously reported. These are the first awake and ambulatory humans in whom consistent abscence of REM sleep has been demonstrated. Both behaved appropriately during wakefulness and showed no overt psychological abnormalities.
Peripheral nerve conduction in fabry disease
Authors: Sheth Kumudchandra J. , Swick Herbert M.
Published: January 2005

Peripheral nerve conduction was studied in 34 members, aged 3 to 54 years, from three separate kindreds with Fabry disease. Nerve conduction velocity was prolonged in one‐third (11 of 34) of the study group. Of the 9 control family members with normal plasma α‐galactosidase levels, 8 had normal velocity. A 6‐year‐old girl with normal plasma and leukocyte α‐galactosidase levels and absent corneal deposits had slightly lower than normal nerve conduction velocity. One of the 3 obligate female carriers and 1 of 10 suspected carriers of Fabry disease had prolonged conduction velocity but normal distal latency. On the other hand, 8 of 12 males affected with Fabry disease demonstrated slow nerve conduction velocity while 4 of the 8 had prolonged distal latency. Frequently, only one of the two nerves studied showed abnormalities. These abnormalities were not related to patient age. Interfamilial variations in the abnormalities were present.
Prevention of experimental allergic encephalomyelitis in lewis rats with peptide 68‐88 of guinea pig myelin basic protein
Authors: Chou Frank C.‐H. , Chou C.‐H. Jen , Fritz Robert B. , Kibler Robert F.
Published: January 2005

The highly encephalitogenic guinea pig peptide 68‐88 has been used to develop an effective and reproducible model of protection in the Lewis rat. Doses as low as 0.1 nmol of peptide protected 70% of rats when injected intraperitoneally six and four weeks prior to challenge with 0.05 nmol of the peptide in complete Freund's adjuvant. Fragments derived from guinea pig peptide 68‐88 by selective enzyme cleavage were then tested for their capacity to provide protection in this model system. These fragments had previously been well characterized both biochemically and immunologically.

The protection provided by each fragment closely paralleled its capacity to induce disease. This suggests that the region of peptide 68‐88 required for protection is similar to that needed for induction of experimental allergic encephalomyelitis and the other T‐cell functions of the peptide. B‐cells did not appear to participate; peptide 68‐85, which has no capacity to produce antibody against peptide 68‐88, gave full protection, while peptide 79‐88, which contains the major B‐cell determinant of the peptide, afforded no protection. Rat peptide 68‐88 did not protect against challenge with the guinea pig peptide, demonstrating a critical role for serine 79. These studies support the concept that nonencephlaitogenic agents do not protect against experimental allergic encephalomyelitis at doses comparable to those of encephalitogenic agents.
Surgically correctable lesions solely detected by CT scan in adult‐onset chronic epilepsy
Authors: Jabbari Bahman , Huott Archer D. , Chiro Giovani Di , Martins Albert N. , Youngblood Lloyd A. , Harper Michael G.
Published: January 2005

A prospective study of 162 patients with adult‐onset chronic epilepsy revealed 4 in whom computed tomographic scanning was the only diagnostic test that detected the presence of a surgically correctable cerebral lesion. These patients had suffered from seizures for 1 to 14 years and at the time of operation demonstrated no clinical neurological deficits except organic mental changes.
Fetal methylmercury poisoning: Clinical and toxicological data on 29 cases
Authors: Marsh David O. , Myers Gary J. , Clarkson Thomas W. , Amin‐Zaki Laman , Tikriti Sadoun , Majeed Muhammed A.
Published: January 2005

This report describes psychomotor retardation in infants caused by prenatal exposure to methylmercury. A study of 29 mother‐infant pairs established a relationship between maximum maternal hair mercury concentration during pregnancy and the frequency of neurological effects in the infants. The latter included delayed achievement of developmental milestones with or without neurological signs. The infants were 4 1/2 to 5 years of age on last examination. Ten infants of mothers who had maximum hair concentrations in the range of 99 to 384 parts per million (ppm) had a significantly higher frequency of abnormal findings than those in two groups having lower maternal hair mercury concentrations (12 to 85 and 0 to 11 ppm). The group sizes were too small to allow identification of a specific ‘threshold’ maternal hair concentration above which such effects can be expected. Transient paresthesias during pregnancy occurred in 80% of the mothers in the higher concentration group (99 to 384 ppm) as compared with 30% and 22% in the lower groups. Neurological abnormalities were found in some children whose mothers had been asymptomatic during pregnancy.
Diagnosis of infantile neuroaxonal dystrophy by skin biopsy
Authors: Wisniewski Krystyna , Wisniewski Henry M.
Published: January 2005

Skin biopsy with ultrastructural examination of cutaneous nerves showed dystrophic axons (spheroids), confirming a diagnosis of infantile neuroaxonal dystrophy. Independent of axonal changes, a few endoneurial and Schwann cells showed cytoplasmic inclusion bodies composed of structures similar to those seen in the spheroids.
Peripheral neuropathy in the cherry‐red spot—myoclonus syndrome (Sialidosis type I)
Authors: Steinman L. , Tharp B. R. , Dorfman L. J. , Forno L. S. , Sogg R. L. , Kelts K. A. , O'Brien J. S.
Published: January 2005

A patient with the cherry‐red spot‐myoclonus syndrome associated with autosomal recessive deficiency of lysosomal neuraminidase was found to have clinical, electrophysiological, and morphological evidence of a sensorimotor peripheral polyneuropathy with distinctive inclusions in the cytoplasm of Schwann cells.
Pathological verification of ischemic score in differentiation of dementias
Authors: Rosen Wilma G. , Terry Robert D. , Fuld Paula A. , Katzman Robert , Peck Arthur
Published: January 2005

Fourteen case histories of persons who had a histological diagnosis of either senile dementia of the Alzheimer type, multiinfarct dementia, or a mixed dementia composed of these two types and who showed evidence of a moderate to severe dementia on psychological testing were rated for the presence of thirteen clinical features comprising Hachinski's Ischemic Score. These features are frequently considered primarily characteristic of vascular dementia. Persons with senile dementia of the Alzheimer type were clearly differentiable from persons with multiinfarct dementia and mixed dementia, while the latter two groups were indistinguishable from one another. In our sample, eight features were found to characterize those persons with vascular dementia. These data verify the usefulness of the Ischemic Score in differentiating between senile dementia of the Alzheimer type and vascular dementia.
Slowed nerve conduction with wrist flexion in carpal tunnel syndrome
Authors: Schwartz M. S. , Gordon J. A. , Swash M.
Published: January 2005

Phalen's wrist flexion test may reproduce the sensory symptoms of carpal tunnel syndrome (CTS). We have used this test in conjunction with motor and sensory latency studies in 20 patients with symptomatic CTS in one or both hands. The test caused an increase in the motor or sensory latency in half the patients. In 2 patients the latencies were abnormal only after wrist flexion. The test may thus be useful in diagnosis of borderline cases.
Lobar cerebral hemorrhages: Acute clinical syndromes in 26 cases
Authors: Ropper Allan H. , Davis Kenneth R.
Published: January 2005

The acute syndromes and CT findings are described in 26 cases of spontaneous cerebral hemorrhage. Occipital hemorrhage (11 cases) caused severe pain around the ipsilateral eye and dense hemianopia. Left temporal hemorrhage (7 cases) began with mild pain in or just anterior to the ear, fluent dysphasia with poor auditory comprehension but relatively good repetition, and a visual deficit subtending less than a hemianopia. Frontal hemorrhage (4 cases) caused a distinctive syndrome beginning with severe contralateral arm weakness, minimal leg and face weakness, and frontal headache. Parietal hemorrhage (3 cases) began with anterior temporal (“temple”) headache and hemisensory deficit, sometimes involving the trunk to the midline. One patient had a right temporal hemorrhage.

Spontaneous lobar hemorrhage and branch artery embolism in the same region produce similar clinical syndromes. Headache is a first and prominent symptom. A rapid but not instantaneous onset over several minutes, when combined with one of the typical syndromes, suggests lobar hemorrhage rather than other types of stroke.

Ancillary investigations (including CT scanning, angiography in 11 patients, and autopsy in 4) disclosed 2 patients with bleeding diatheses due to warfarin, 2 with arteriovenous malformations, and 1 with metastatic tumor. Only 8 of the 26 patients had chronic hypertension (blood pressure greater than 130/85 mm Hg), suggesting that hypertension is not an etiological factor in most lobar hemorrhages.
Oligoclonal IgG and free light chains in multiple sclerosis demonstrated by thin‐layer polyacrylamide gel isoelectric focusing and immunofixation
Authors: Laurenzi Maria Assunta , Mavra Milica , Kam‐Hansen Slavenka , Link Hans
Published: January 2005

A modified technique of isoelectric focusing on thin‐layer polyacrylamide gel followed by immunofixation with monospecific antisera was used to identify individual cerebrospinal fluid (CSF) and serum proteins and to define the oligoclonal reaction observed in multiple sclerosis (MS). “Normal” IgG gave about 20 to 30 bands at pH 3.5 to 9.5, IgA about 10 bands at pH 3.5 to 6.4, β‐trace protein a smear at pH 3.5 to 8.5, and γ‐trace protein 1 or 2 bands at pH 8.0, 9.5, or both.

Up to 11 oligoclonal IgG bands migrating between pH 6.5 and 9.5 were found in CSF from 26 of 27 consecutive patients with MS and also in 20 of the corresponding sera, although at lower numbers and concentrations. In 26 patients, 1 or more of the bands corresponding to normal polyclonal IgG were stronger in CSF than in serum. These data support the hypothesis that two colonies of lymphocytes are activated intrathecally, one of them synthesizing oligoclonal and the other polyclonal IgG. Up to 11 mostly faint bands of free light chains, predominantly of λ type and migrating between pH 3.5 and 9.5, were found in 8 of 9 CSF specimens from patients with MS.
Skin and conjunctival nerve biopsies in adrenoleukodystrophy and its variants
Authors: Martin J. J. , Ceuterick C. , Libert J.
Published: January 2005

This study demonstrates that skin or conjunctival biopsies are capable of diagnosing adrenoleukodystrophy and its variants. Electron microscopy of cutaneous nerve twigs in eleven patients showed characteristic curved clefts and leaflets in Schwann cells surrounding myelinated axons. In addition, three conjunctival biopsies were done, two of which were positive. The two peripheral nerve trunk biopsies performed also showed characteristic Schwann cell changes.
Infantile neuronal degeneration masquerading as werdnig‐hoffmann disease
Authors: Steiman Gerald S. , Rorke Lucy Balian , Brown Mark J.
Published: January 2005

We investigated two infants whose clinical illness resembled Werdnig‐Hoffmann disease (WHD). Motor nerve conduction velocities in one infant were markedly decreased, suggesting a demyelinative neuropathy. Pathological examination of the central nervous system in both patients revealed the expected loss of cell bodies in the anterior horn and motor cranial nerves. In addition, there was widespread neuron loss and gliosis in both gray and white matter of the thalamus, cerebellum, pons, and spinal cord. Motor, sensory, and mixed peripheral nerves of one infant showed evidence of segmental demyelination with only mild fiber loss. These and similar patients previously described have a disorder that should be separated from WHD and can be termed infantile neuronal degeneration.
Locked‐in syndrome with recovery
Authors: Khurana Ramesh K. , Genut A. A. , Yannakakis George D.
Published: January 2005

We report three patients who recovered from “locked‐in” syndrome of presumed vascular origin. One patient narrated her experience of the “locked‐in” phase and its termination. Another demonstrated palatal myoclonus and mirror movements upon recovery. These patients deserve considerate handling and vigorous medical support.
Treatment of intractable temporal lobe‐limbic epilepsy (complex partial seizures) by temporal lobectomy
Authors: Glaser Gilbert H.
Published: January 2005

The results of lobectomy in carefully selected patients with intractable epilepsy now are realized as worth the risk, for complete seizure relief can be achieved in up to 50% of such cases, and moderate to good seizure control in at least an additional 25%, with reduction in the drug requirement. Along with seizure relief often comes improvement in behavior and in social and personality development and life adjustments. Temporal lobectomy for treatment of temporal lobe‐limbic epilepsy therefore also has preventive value, especially if performed as early as the criteria indicate in appropriately selected patients. The resected tissue allows careful morphological studies that can enhance our knowledge of the pathogenesis of this disorder.
Sustained limbic seizures induced by intraamygdaloid kainic acid in the baboon: Symptomatology and neuropathological consequences
Authors: Menini Christian , Meldrum Brian S. , Riche Danielle , Silva‐Comte Carmen , Stutzmann Jean Marie
Published: January 2005

In Papio papio baboons chronically prepared for cortical and deep electroencephalographic recording, injection of kainic acid into the amygdala (7 animals) or temporal pole (2 animals) gave rise to focal epileptic discharges lasting 15 to 150 hours. Electrographically, the seizure activity spread ipsilaterally and contralaterally within the limbic system but did not become generalized. The principal associated motor signs were arrest of movement and oral automatisms. Histological examination after two to ten days demonstrated lesions (neuron loss and gliosis) at the injection site that varied according to the amount of kainic acid injected (2 to 68 μg). “Remote” lesions occurred in the ipsilateral hippocampus (end‐folium and Sommer sector) and neocortex (occipital and frontal regions). The hippocampal lesions were comparable to those previously described as consequent to status epilepticus.
Tolosa‐hunt syndrome: A case with associated facial nerve palsy
Authors: Swerdlow Barry
Published: January 2005

A 54‐year‐old man developed a painful right third nerve palsy with signs of involvement of the first two divisions of the trigeminal nerve and, later, ipsilateral peripheral facial nerve weakness. A cavernous sinus venogram demonstrated narrowing of the third portion of the right ophthalmic vein and poor filling of the adjacent cavernous sinus. Resolution occurred with steroid therapy. The Tolosa‐Hunt syndrome may be a variant of a larger syndrome of recurrent multiple cranial polyneuropathies without identifiable cause.
Kernicterus and central pontine myelinolysis in a 14‐year‐old boy with fulminating viral hepatitis
Authors: Ho Khang‐ChengHo , Hodach Richard , Varma Rajiv , Thorsteinson Vigfus , Hess Timothy , Dale David
Published: January 2005

An adolescent boy with severe viral hepatitis and elevated unconjugated bilirubin was diagnosed postmortem to have kernicterus and central pontine myelinolysis. This association has not been reported previously.
Comparison of agar gel electrophoresis and isoelectric focusing in multiple sclerosis and subacute sclerosing panencephalitis
Authors: Mattson David H. , Roos Raymond P. , Arnason Barry G. W.
Published: January 2005

Isoelectric focusing (IEF) and agar gel electrophoresis (AGE) were used to examine cerebrospinal fluid (CSF) and sera from patients with multiple sclerosis (MS) and subacute sclerosing panencephalitis (SSPE). All 15 SSPE samples and 29 of the 33 MS CSF samples showed oligoclonal IgG bands by AGE and IEF. Serum bands were more frequent in SSPE than in MS and were more commonly detected by IEF than by AGE. In MS CSF the number of bands on IEF correlated with: (1) disease duration, (2) CSF IgG, (3) CSF IgG/albumin ratio, and (4) central nervous system IgG synthesis. Serial studies revealed increases in IEF band number in 4 of 10 MS and 3 of 5 SSPE CSF specimens; fluctuations in band intensity were also noted.

Densitometric scans of CSF IEF gels showed high, sharply angled IgG band peaks in SSPE; the CSF band peaks in MS were flatter and had higher background IgG. The SSPE pattern could be maEN to resemble the MS pattern through addition of normal polyclonal IgG to SSPE CSF. These findings suggest that in addition to oligoclonal IgG, polyclonal IgG is synthesized locally within the central nervous system in MS.
Intracranial infection in cardiac transplant recipients
Authors: Britt Richard H. , Enzmann Dieter R. , Remington Jack S.
Published: January 2005

Infections have produced most of the deaths in the Stanford cardiac transplant program. Of the first 182 transplant recipients, 27 developed nonviral intracranial infections: meningoencephalitis/abscess in 16 patients, meningitis in 9, and rhinocerebral phycomycoses in 2. The responsible organisms included aspergillus, toxoplasma, candida, klebsiella, cryptococcus, coccidioides, listeria, mucor, and rhizopus. Characteristically, the areas of meningoencephalitis and abscesses were multiple and deep seated. Intracranial infections were invariably associated with pulmonary or disseminated infection with the same organism.

Computed tomographic (CT) brain scans in patients with meningoencephalitis often showed minimal, nonspecific, low‐density lesions which usually did not exhibit contrast enhancement. At surgery the lesions were found to differ from typical pyogenic abscesses in that capsules were not well developed, and the aspirate consisted of necrotic fragments of edematous white matter and inflammatory cells rather than liquefied pus.

Aspergillus infections of the central nervous system usually developed within the first three months after transplantation. Cases of meningitis occurred at variable times after transplantation, but approximately half appeared within 30 days after immunosuppressive therapy for treatment of rejection was increased. The prognosis for brain abscess depended on the causative organism. All patients with aspergillus infection died despite treatment with amphotericin B. The toxoplasma abscess responded to a combination of sulfadiazine and pyrimethamine. Meningitis was successfully suppressed or cured with appropriate treatment except for 1 patient with disseminated Cryptococcosis.
Demyelination of the lateral geniculate nucleus in central pontine myelinolysis
Authors: Goldman James E. , Horoupian Dikran S.
Published: January 2005

Postmortem examination of the central nervous system in 6 of 9 patients with central pontine myelinolysis (CPM) revealed demyelination in the lateral geniculate nucleus (LGN). Histologically, the geniculate lesions consisted of myelin swelling and disintegration, largely in the central part of the nucleus, with preservation of axons and neuronal perikarya. The pathological changes resembled those found in the basis pontis in CPM.
Postischemic hypervascularity of infancy: A stage in the evolution of ischemic brain damage with characteristic CT scan
Authors: Shewmon D. Alan , Fine Michael , Masdeu Joseph C. , Palacios Enrique
Published: January 2005

One to 2 weeks after severe brain ischemia, four infants developed an unusual vascular lesion with a characteristic appearance on computed tomography that has not been reported previously. Restricted areas (most frequently the basal ganglia and thalamus) displayed increased attenuation, which enhanced further upon infusion of contrast medium. Autopsy of one infant revealed that the CT abnormality corresponded to an extremely dense neovascular network which had almost completely replaced the parenchyma in that region. We postulate that this pathological change is a stage in the organization of ischemic brain damage reflecting the infant's vascular plasticity. Thus, hypoxia induces marked capillary proliferation in regions of normally high metabolism and capillary density. The CT pattern may prove useful in predicting the location and extent of sequelae to perinatal asphyxia.
Multiple sclerosis serum and cerebrospinal fluid immunoglobulin binding to fc receptors of oligodendrocytes
Authors: Ma Booe I. , Joseph Barry S. , Walsh Michael J. , Potvi Alfred R. , Tourtellotte Wallace W.
Published: January 2005

Suspensions of bovine oligodendrocytes were used to study the immunofluorescent staining properties of sera from multiple sclerosis (MS) patients and normal individuals. All sera (14 MS patients, 8 patients with other neurological diseases, and 11 normal individuals) showed positive oligodendrocyte staining by indirect immunofluorescence. Staining persisted after extensive absorption of sera with bovine liver to remove nonspecific binding. Similar findings were obtained for cerebrospinal fluid from all 5 MS patients as well as all 5 patients with other neurological diseases. In additional studies to determine if binding is mediated by the Fc fragment of IgG, results were: (1) ultracentrifuged normal and MS sera failed to react with oligodendrocytes, (2) positive staining with oligodendrocytes was observed after heat aggregation of IgG, (3) ox red blood cells, complexed with antibody, reacted with oligodendrocytes to produce strong rosette formation, and (4) the rosette formation could be blocked by prior treatment of oligodendrocytes with heat‐aggregated IgG. The studies fail to support a previous claim for specific binding of immunoglobulins to oligodendrocytes in MS. However, they confirm and extend previous findings that the binding of IgG to oligodendrocytes may not necessarily be an antigen‐antibody reaction. Therefore, adherence reactions with a putative antibody must exclude Fc‐mediated binding.