bluuebunny/crossref_metadata_2025 huggingface dataset:
Cerebral and retinal vascular complications of inflammatory bowel disease
Authors: Schneiderman Jacob H. , Sharpe James A. , Sutton David M. C.
Published: January 2005

Recurrent retinal branch artery occlusions, carotid thromboembolism, cerebral venous thrombosis, transient brainstem ischemia, and massive brainstem and cerebral infarction complicated the course of inflammatory bowel disease in 5 patients. Three patients had ulcerative colitis and 2 had regional enteritis. The usual risk factors for stroke were absent. Neuropathological examination in 1 patient showed in situ thrombosis of small cerebral and brainstem arteries and veins. Coagulation studies showed thrombocytosis, short partial thromboplastin times, and elevation of fibrinogen and Factor VIII levels. Platelet counts and coagulation factors returned toward normal after control of intestinal inflammation in each of the 4 surviving patients. Inflammatory bowel disease can be accompanied by a hypercoagulable state that predisposes to stroke.
Suppressor cell function in multiple sclerosis: Correlation with clinical disease activity
Authors: Antel Jack P. , Arnason Barry G. W. , Medof M. Edward
Published: January 2005

Concanavalin A (Con A)–activated suppressor cell activity was determined in multiple sclerosis (MS) patients who had been assigned to one of three subgroups, those with active disease, those recovering from a flare‐up, and those with stable disease. The level of suppression induced by the Con A–activated suppressor cells on the mitogenic response of autologous peripheral blood lymphocytes was reduced in patients with active disease (3 ± 8%) compared with stable patients (30 ± 8%), patients recovering from a flare‐up (62 ± 5%), and controls (40 ± 5%). As a measure of the actual amounts of suppressor factors released, the effect of supernatants from the Con A–activated cells on the proliferative activity of a dividing cell line (L cells) was determined concurrently. The inhibitory effect of supernatants from activated cells was reduced in active and stable MS patients (7 ± 3%) compared to controls (21 ± 4%). Three of 4 with active MS showed mildly elevated immune complex levels as measured by the Raji cell technique; each of these patients had low suppressor activity. Levamisole (1 μg per milliliter) failed to alter suppresor cell activity in our in vitro system.
Prostaglandin effect on lymphokine production in multiple sclerosis
Authors: Willoughby E. W. , Dupont B. , Good R. A.
Published: January 2005

Production of the lymphokine LIF (leukocyte migration inhibitory factor) by normal lymphocytes on stimulation with mitogens is inhibited by E type prostaglandins (PGE). It has been reported that the leukocytes of patients with multiple sclerosis (MS) are resistant to this inhibitory effect. We have studied 10 MS patients using an indirect technique for measuring LIF activity. This allows separation of the effects of PGE on lymphocyte LIF production from the effects on the action of LIF on the target granulocytes. There was no difference between normal subjects and MS patients in the effects of prostaglandins E1 and E2 in concentrations of 2.5 and 0.25 μg per milliliter, on LIF production, or on granulocyte response to LIF. These results do not support the hypothesis that the chronic inflammatory process in MS is the result of resistance to the normal inhibitory effects of PGE on the cellular immune response.
“Arachnoid cysts” of the sylvian fissure versus “temporal lobe agenesis” syndrome
Authors: Shaw Cheng‐Mei
Published: January 2005

Measurements of the two cerebral hemispheres in 2 brains with arachnoid cysts of the sylvian fissure indicate no differences in weight or volume and therefore give no support to the hypothesis that these could be examples of the “temporal lobe agenesis” syndrome.
Progressive myelopathy due to extramedullary hematopoiesis: Case report and review of the literature
Authors: Stahl Stephen M. , Ellinger Gary , Baringer J. Richard
Published: January 2005

Extramedullary hematopoiesis (EMH) in the spinal epidural space is a rare but treatable cause of progressive paraparesis in patients with a variety of hematological and systemic disorders. We report a case of extensive thoracic epidural EMH causing progressive spastic paraparesis in a patient with agnogenic myeloid metaplasia. The literature describes 11 additional cases of myelopathy due to epidural EMH. All patients had EMH in the midthoracic region with elevated cerebrospinal fluid protein and abnormal spine roentgenograms or myelograms. Treatment with decompressive laminectomy, radiation therapy, or both, resulted in marked or complete recovery in 7 of 10 patients. Patients who develop paraparesis and who have a disorder associated with EMH should be evaluted rapidly for this treatable condition.
Development of nystagmus in response to vestibular stimulation in infants
Authors: Eviatar L. , Miranda S. , Eviatar A. , Freeman K. , Borkowski M.
Published: January 2005

Nystagmus in response to perotatory stimulation by torsion swing or ice‐cold caloric (ICC) irrigation of the ear canals was studied in 276 infants from birth to 12 months of age. The percentage of positive responses to perotatory stimulation correlated with gestational age and weight at birth during the first 3 months of life and became comparable among groups by 6 months of age. The quality of perotatory nystagmus did not differ among groups.

A direct correlation between the caloric‐induced intensity and duration of nystagmus with gestational age and weight at birth was noted during the first 3 months of life. Premature infants showed the weakest responses, and term‐delivered, large for gestational age children the strongest responses. ICC‐induced nystagmus reached comparable levels for all groups by 6 months except for premature infants, in whom comparable responses were attained by 9 months of age. Vestibular responses mature over time, with patterns that correlate with gestational age and weight at birth.
Inhibitory effects of anticonvulsant drugs on cyclic nucleotide accumulation in brain
Authors: Ferrendelli James A. , Kinscherf Dorothy A.
Published: January 2005

Veratridine causes depolarization of excitable cells and produces marked elevation of adenosine 3′,5′‐monophosphate (cyclic AMP) and guanosine 3′,5′‐monophosphate (cyclic GMP) levels in incubated slices of mouse cerebral cortex. Phenytoin, carbamazepine, phenobarbital, primidone, phensuximide, methsuximide, α‐methyl‐α‐phenylsuccinimide, and high concentrations of clonazepam are anticonvulsant drugs that preferentially prevent maximal electroshock seizures (MES) and generalized tonic‐clonic convulsions; all these agents inhibit veratridine‐induced accumulation of both cyclic AMP and cyclic GMP. In contrast, ethosuximide, trimethadione, valproic acid, and low concentrations of clonazepam are anticonvulsant drugs that act predominantly against Metrazol and absence seizures; these agents are ineffective or inhibit accumulation of only cyclic GMP. The results suggest that inhibition of cyclic AMP and cyclic GMP accumulation in depolarized brain tissue is a molecular neuropharmacological action characteristic of anticonvulsant drugs that have direct effects on cellular membrane function and prevent MES. Anticonvulsant drugs that do not inhibit accumulation of both cyclic AMP and cyclic GMP in depolarized brain tissue preferentially prevent Metrazol and absence seizures and probably exert their effects by altering neurotransmission mechanisms.
Determinants of survival in children with brain tumors
Authors: Gold Ellen B. , Gordis Leon
Published: January 2005

A study was conducted in metropolitan Baltimore to examine time trends in survival and factors which influence survival of children with brain tumors. One hundred eighty‐four cases diagnosed from 1960 to 1975 were identified; 76.5% occurred in white children and 48% were girls. Five‐year survival rates were 35% for children diagnosed in 1960–64, 34% in 1965–69, and 49% in 1970–74. Girls had consistently better survival rates than boys. White children experienced experienced consistently better survival rates than blacks. Of those who survived the first year, children with supratentorial tumors had better five‐year survival rates (46%) than those with infratentorial tumors (27%). Of those who survived the first year, children with astrocytomas had better survival rates than those with other types of tumors.
Myelin basic protein binding cells in active multiple sclerosis
Authors: Vandenbark Arthur A. , Hallum Jules V. , Swank Roy L. , Tong Alex , Burger Denis R.
Published: January 2005

A sensitive, antigen‐specific rosetting technique was used to enumerate blood cells that bind myelin basic protein (BP) in MS patients and controls. Sixteen of 23 MS patients in exacerbation but only 7 of 48 in remission formed elevated numbers of rosettes. Five of the latter 7 patients had recovered from an exacerbation within four months of the laboratory evaluation. Eight of 20 patients with progressive MS, all of whom had had disease for more than four years, had BP rosette‐forming cells. None of the 16 normal volunteers and 1 of 8 neurological controls had BP rosette‐forming cells. These results suggest that BP‐binding cells in MS are confined to patients in acute exacerbation or within four months of activity, and those who have had progressive disease for at least four years.
Oligodendrocyte staining by multiple sclerosis serum is nonspecific
Authors: Traugott Ute , Snyder D. Stephen , Raine Cedric S.
Published: January 2005

The immunofluorescent staining properties of 65 serum samples from 54 patients with multiple sclerosis (MS), 63 samples from 55 patients with other neurological diseases (OND), and sera from 14 healthy normal individuals were examined on frozen sections of bovine and human brain. When tested on bovine brain sections, positive oligoden drocyte staining was present in 63% of MS sera, 43% of OND sera, and 29% of normal sera. The percentages were lower with human brain tissue. Astrocyte and myelin staining was common. F(ab')2 fragments purified from selected positive and negative MS and control sera gave no staining, though IgG fractions from the same sera prior to pepsin digestion gave positive staining. When tested against antihuman IgM and IgA conjugates, the same positive sera and their IgG‐depleted globulin fractions gave minimal or no staining. These results indicate that oligodendrocyte staining is not specific for MS, is not due to specific antibody, and is probably the result of nonspecific binding to Fc receptors.
Controlled trial of EMG feedback in muscle contraction headache
Authors: Bruhn Peter , Olesen Jes , Melgaard Bjørn
Published: January 2005

Twenty‐eight patients suffering from severe, longstanding muscle contraction headache were randomly assigned to two groups, one receiving electromyographic (EMG) feedback therapy and the other, “most suitable alternative therapy.” Headache intensity and severity as well as drug intake were reduced in the feedback group (p ≤ 0.01) as opposed to no improvement in the control group. The positive treatment effect in the feedback group persisted through a three‐month follow‐up peiod. EMG feedback therapy is effective in the treatment of muscle contraction headache even in its chronic, severe form, which is resistant to traditional treatment methods.
Use of a self‐administered questionnaire for detection of transient cerebral ischemic attacks: I. Survey of elderly persons living in retirement facilities
Authors: Wilkinson W. E. , Heyman A. , Burch J. G. , Ostfeld A. , Labarthe D. R. , Leviton A. , O'Fallon W. M. , Whisnant J.
Published: January 2005

A self‐administered questionnaire designed to identify people with transient cerebral ischemia (TIA) in large population groups was distributed to approximately 10,000 elderly persons living in public and private retirement facilities in the United States. The population resided in eight cities, and 74% completed the survey. Transient focal neurological symptoms compatible with a diagnosis of TIA within the previous year were reported by 6.4% of the respondents. Uncertain manifestations of TIA, i.e., dizziness, light‐headedness, or loss of balance, were noted by an additional 15.4% the remaining 78.2% had no manifestations of this disorder.

A clinical history for TIA was taken by a neurologist in a sample of 1,712 respondents to determine the validity of the questionnaire responses. Thirty (7.1%) of 423 persons presumed by the questionnaire to have TIA met the standard clinical criteria for the diagnosis. An additional 8 cases of TIA were found by the neurologist among the 1,289 interviewed persons presumed by the questionnaire to have uncertain TIA or no evidence of the disorder. The twelve‐month period prevalence of TIA was estimated to be 8.2 per 1,000 in the total respondent population and 5.8 per 1,000 among those without a history of prior completed stroke. Despite the high proportion of false‐positive neurological symptoms of TIA elicited by the questionnaire, this survey instrument nevertheless screened out for further study the 6% of the population in whom 55% of the TIAs occurred.
Dynamic properties of partially denervated muscle
Authors: Miller Robert G.
Published: January 2005

The mechanical and electrical properties of the partially denervated first dorsal interosseous muscle were measured in 14 patients and 14 normal control subjects. The following variables were studied during isometric contraction: maximum voluntary contraction; maximum rate of rise of tension in a rapid voluntary contraction; amplitude, rate of rise, time to peak, and duration of peak of the compound muscle action potential; twitch force, maximum rate of rise, contraction time, and half‐relaxation time; and tetanic (50 Hz) force, rate of rise, and tetanus/twitch ratio. The force produced during repetitive stimulation of the ulnar nerve at 10, 20, 50, and 100 impulses per second was also analyzed.

The major findings were: (1) decreased load bearing (voluntary contraction, twitch, and tetanus), (2) prolonged twitch contraction times and half‐relaxation times, (3) decreased tetanus/twitch ratio, and (4) preserved rate of rise of tension.
Tolosa‐hunt syndrome with sellar erosion
Authors: Polsky M. , Janicki Paul C. , Gunderson C. H.
Published: January 2005

A 47‐year‐old patient with Tolosa‐Hunt syndrome was successfully treated with steroids. An unusual clinical feature was erosion of the sella turcica. It is postulated that this was due to the mass effect of the granulomatous changes in the cavernous sinus.
Familial subacute necrotizing encephalomyelopathy of the adult form (adult Leigh syndrome)
Authors: Kalimo H. , Lundberg P. O. , Olsson Y.
Published: January 2005

A family—mother and 2 sons—with a heredodegenerative neurological disease is described. The disease started with bilateral optic atrophy, central scotoma, and color blindness during the second decade. This was followed by a quiescent period until additional neurological symptoms appeared, around the age of 50 years in the case of the mother and 40 and 30 years, respectively, in the sons. The additional symptoms were ataxia, spastic paresis, clonic jerks, grand mal sezures, psychic lability, and slight dementia. The disease was progressive, resulting in permanent hospitalization within a few years. The mother died at the age of 63 years and the sons at 46 and 43 years of age.

Neuropathological examination revealed lesions histopathologically characteristic of subacute necrotizing encephalomyelopathy (SNE, Leigh disease), and their distribution in the brain and brainstem also conformed to this disease. On the basis of the clinical course and neuropathological findings, we consider that these 3 patients represent the first reported familial cases of the adult form of SNE.
Dysarthria–clumsy hand syndrome produced by capsular infarct
Authors: Spertell Robert B. , Ransom Bruce R.
Published: January 2005

A 64‐year‐old hypertensive man presented with the dysarthria–clumsy hand syndrome, manifested by dysarthria, dysphagia, central facial weakness, deviation of the tongue on protrusion, incoordination of the affected hand, and mild imbalance on walking. A computed tomographic scan demonstrated a resolving acute infarction in the vicinity of the genu of the internal capsule.
Agenesis of the corpus callosum: A study of the frequency of associated malformations
Authors: Parrish Margaret L. , Roessmann Uros , Levinsohn Morris W.
Published: January 2005

Review of 11 cases of agenesis of the corpus callosum studied at our institution revealed a high incidence of associated anomalies. Nine patients had associated malformations of the central nervous system, 6 involving the pyramidal system. Eight cases were associated with malformations in the rest of the body. Review of completely examined cases from the literature yielded 47 examples of associated malformations. These were varied and without consistent pattern, with the possible exception of facial abnormalities.
Tomographic measurement of local cerebral glucose metabolic rate in humans with (F‐18)2‐fluoro‐2‐deoxy‐D‐glucose: Validation of method
Authors: Phelps M. E. , Huang S. C. , Hoffman E. J. , Selin C. , Sokoloff L. , Kuhl D. E.
Published: January 2005

Tracer techniques and quantitative autoradiographic and tissue counting models for measuremnt of metabolic rates were combined with positron computed tomography (PCT) and (F‐18)2‐fluoro‐2‐deoxy‐D‐glucose (FDG) for the measurement of local cerebral metabolic rate for glucose (LCMRGlc) in humans. A three‐compartment model, which incorporates hydrolysis of FDG‐6‐PO4 to FDG, was developed for the measure of kinetic constants and calculation of LCMRGlc. Our model is an extension of that developed by Sokoloff et al. Although small, hydrolysis of FDG‐6‐PO4 was found to be significant. A PCT system, the ECAT, was used to determine the rate constants, lumped constant, and stability of the model in human beings. The data indicate that cerebral FDG‐6‐PO4 in humans increases for about 90 minutes, plateaus, and then slowly decreases. After 10 minutes, cerebral blood FDG activity levels were found to be a minor fraction of tissue activity. Precursor pool turnover rate, distribution volumes, and red blood cell‐plasma concentration ratios were determined. Reproducibility (precision) of LCMRGlc measurements (∼2 cm2 regions) was ± 5.5% over a 5‐hour period. The replacement of arterial blood sampling with venous sampling was validated.
Has myelin basic protein received a fair trial in the treatment of multiple sclerosis?
Authors: Alvord Ellsworth C. , Shaw Cheng‐Mei , Hruby Sarka , Kies Marian W.
Published: January 2005

Autosensitization to some central nervous system antigen still remains one of the best hypotheses for the continuing pathogenesis of multiple sclerosis (MS). Enough is now known about the cause, pathogenesis, and treatment of experimental allergic encephalomyelitis (EAE) to test this hypotesis. Reports of therapeutic failure of the encephalitogen myelin basic protein (BP) in the treatment of MS have their counterparts in similar therapeutic failures in EAE. Only highly inbred strain 13 guinea pigs respond much less well to simple BP therapy, and monkeys hardly at all. In both strains of monkeys so far studied, a nonspecific adjunctive factor–an antibiotic in Macaca mulatta and a steroid in Macaca fascicularis—is also required. Accordingly, human trials of the therapeutic efficacy of BP in MS should include its administration in large concentrations together with an adjunctive agent.
Myelin basic protein treatment of experimental allergic encephalomyelitis in monkeys
Authors: Alvord Ellsworth C. , Shaw Cheng‐Mei , Hruby Sarka
Published: January 2005

Treatment of experimental allergic encephalomyelitis (EAE) in two strains of monkeys with large amounts of myelin basic protein (BP) fails unless an adjunct is also used. In both strains the adjunct by itself is more effective than BP by itself, but in the one strain which could be investigated sufficiently, the combination can be made almost totally effective in reversing EAE. The adjunct varies with the strain of monkey, an antibiotic in Macaca mulatta and a steroid in Macaca fascicularis. Similar adjunctive treatments should be considered in the management of multiple sclerosis, for which EAE remains one of the best studied models.
Degradation of myelin basic protein by cerebrospinal fluid: Preservation of antigenic determinants under physiological conditions
Authors: Alvord Ellsworth C. , Hruby Sarka , Sires Linda R.
Published: January 2005

Cerebrospinal fluid contains several proteolytic enzymes that can degrade myelin basic protein (BP) under physiological conditions into peptide fragments of various sizes which still contain antigenic determinants capable of binding antibodies to BP. These enzymes are optimally active in either acid (pH 4) or neutral (pH 7 to 8) conditions and can be characterized by the nature of the BP peptide fragments produced. Proteinases resembling cathepsin D, thrombin, plasmin (fibrinolysin), or kallikrein are present in variable amounts in CSF. No relatinship to any particular disease has yet been established.
Treatment of reye syndrome
Authors: Trauner Doris A.
Published: January 2005

Although the underlying cause of Reye syndrome is not understood, an effective approach to treatment is based on reversing the known metabolic and pathological abnormalities. A multifaceted therapeutic approach aimed at correcting metabolic derangements and combating intracranial hypertension can result in complete recovery from severe cases of Reye syndrome.
Pituitary adenomas: Clinicopathological and immunohistochemical study
Authors: Martinez A. Julio , Lee Arthur , Moossy John , Maroon Joseph C.
Published: January 2005

The results of conventional histological staining, ultrastructural studies, and immunoperoxidase methods were compared in a study of 50 pituitary adenomas and correlated with clinical features. Final diagnosis was based on immunoperoxidase reactions and ultrastructural characteristics. There were 25 prolactin (lactotrophic) cell adenomas, 4 growth hormone (somatotrophic) cell types, 1 mixed growth hormone‐prolactin cell tumor, 3 adrenocorticotropic cell tumors, and 17 undifferentiated cell adenomas. While serum hormone levels, immunoperoxidase reactions, and electron microscopic features generally correlated well with the clinical features, discrepancies emerged in several cases. Among the responsible factors were: (1) the small sample of tumor removed by transsphenoidal hypophysectomy; (2) uncertainty that the tumor was totally removed or that representative samples were furnished to the pathologist; (3) artifacts produced by electrocoagulation and surgical trauma; (4) inclusion in the biopsy of fragments of altered nonneoplastic adenohypophysis; (5) difficulty in distinguishing hyperplasia from adenoma in small fragments; (6) patchy or scattered immunoreactive cells within an adenoma; (7) the presence of a few cells immunoreactive for hormones other than the predominant one within an adenoma (e.g., cells immunoreactive for growth hormone within a prolactin cell adenoma); and (8) paradoxical serum hormone level abnormalities, especially prolactinemia in patients with undifferentiated cell adenomas and no endocrine symptoms. Biological complexity and technical problems associated with surgery for human adenohypophyseal disease result in continued failure to achieve precise diagnostic correlations with pituitary adenomas.
Gilles de la tourette syndrome: Clinical and family study of 50 cases
Authors: Nee Linda E. , Caine Eric D. , Polinsky Ronald J. , Eldridge Roswll , Ebert Michael H.
Published: January 2005

Fifty patients with Tourette syndrome were evaluated; data included family history, clinical characteristics, response to haloperidol, and side effects during haloperidol therapy. Sixteen patients had a family history of Tourette syndrome, and another 16 had a family history of tics. Twenty‐four families had more than 2 members with Tourette syndrome or tics. There was no preponderance of families with a Jewish, Eastern European background in this sample. Thirty‐four patients had obsessive‐compulsive behavior. Among the 50 patients there was a high frequency of sleep disturbance, learning disability, self‐destructive behavior, inappropriate sexual activity, and antisocial behavior. Family history was significantly related to the occurrence of sleep distrubance, obsessivecompulsive behavior, haloperidol response, and the frequency of side effects caused by haloperidol. The precise mode of genetic transmission in familial Tourette syndrome remains to be determined.
Startle epilepsy complicating down syndrome during adulthood
Authors: Giménez‐Roldán S. , Martín M.
Published: January 2005

A woman with Down syndrome—a trisomy 21 karyotype—developed severe startle epilepsy and spontaneous seizures at the age of 20 years. Triggering factors, zeizure patterns, and ictal electroencephalograms were the same as in startle epilepsy occurring in children with cerebral palsy but differed in that there was no clinical or radiological evidence of a focal brain lesion in the vicinity of the motor supplementary cortex. A therapeutic trial with clonazepam substantially reduced the frequency of reflex‐induced and spontaneous seizures.
Mycoplasma antibody in guillain‐barré syndrome and other neurological disorders
Authors: Goldschmidt Barbra , Menonna Joseph , Fortunato Julei , Dowling Peter , Cook Stuart
Published: January 2005

Counterimmunoelectrophoresis (CIEP) was used to determine precipitating antibodies to Mycoplasma pneumoniae retrospectively in sera from 100 patients with Guillain‐Barré syndrome (GBS), 125 medical and neurological controls, and 40 normal individuals. Sera from 7 patients produced precipitin lines. These positive cases included 5 patients with GBS, 1 with acute cerebellar ataxia, and 1 with acute disseminated encephalomyelitis. A complement‐fixation test performed with the same antigen showed titers to M. pneumoniae of 1:512 or greater in these 7 sera. In contrast, sera from other patient controls and normal individuals were negative by CIEP and had only low mycoplasma complement‐fixation antibody titers.

No distinguishing clinical features separated the 5 seropositive GBS patients from the whole group except for their young age, which parallels that for human mycoplasma infection in general. Additional laboratory findings consistent with acute mycoplasma infection were demonstrated in 6 of the 7 seropositive patients.
Brainstem auditory evoked responses in 200 patients with multiple sclerosis
Authors: Chiappa Keith H. , Harrison Jane L. , Brooks Elizabeth B. , Young Robert R.
Published: January 2005

Brainstem auditory evoked responses (BAERs) were recorded from 202 patients with definite, probable, or possible multiple sclerosis (MS). Definitions of abnormality were based only on interwave separations and the wave I/wave V amplitude ratio. Thirty‐two percent of the patients had abnormal BAERs, and the presence of clinically unsuspected lesions was revealed by BAER abnormalities in 7.4%. Thirty‐five percent of the patients who had nystagmus and 53% of those who had internuclear ophthalmoplegia at the time of testing had BAER abnormalities. Forty‐five percent of the abnormalities were elicited with stimulation of one ear only, stressing the importance of monaural stimulation. Click rates faster than 10 per second did not reveal abnormalities undetected at slower rates. BAERs were normal by these criteria in patients with labyrinthine diseases and amyotrophic lateral sclerosis. Thus, the BAER in MS can (1) confirm the presence of central lesions in pateints with suspected brainstem involvement, (2) document the presence of clinically unsuspected lesions, and (3) be followed over time to provide possible assistance in evaluating the effectiveness of therapeutic measures. The BAER is a useful tool in the diagnosis and management of MS.
Recovery following brainstem hemorrhage
Authors: Burns James , Lisak Robert , Schut Luis , Silberberg Donald
Published: January 2005

Two patients made virtually complete recoveries following hemorrhage within the brainstem. Progressive clinical deterioration in each case was managed surgically by evacuation of the hemorrhagic lesion in 1 case and treatment of secondary hydrocephalus in both. Computerized tomographic scanning proved essential for nonsurgical diagnosis and later management.
Reduced seizure control due to spoiled phenytoin capsules
Authors: Cloyd James C. , Gumnit Robert J. , Lesar Timothy S.
Published: January 2005

Subtherapeutic phenytoin serum levels and loss of seizure control occurred in a 31‐year‐old man due to decreased bioavailability of oral drug. During storage at extreme temperatures, physical changes of the phenytion capsule resulted in altered dissolution characteristics so that only 50% dissolution occurred at 180 minutes compared with 95% in 120 minutes for control capsules. Similar changes were produced in fresh capsules within seven days at high temperature and humidity. The affected patient has metabolic responses to phenytoin that produce marked fluctuations in serum levels with changes in dose. Altered phenytoin serum concentrations may occur with minor dose changes in such patients despite good compliance.
Radiation‐induced malignant and atypical peripheral nerve sheath tumors
Authors: Foley Kathleen M. , Woodruff James M. , Ellis Frank T. , Posner Jerome B.
Published: January 2005

The reported peripheral nerve complications of therapeutic irradiation in humans include brachial and lumbar plexus fibrosis and cranial and peripheral nerve atrophy. We have encountered 9 patients with malignant (7) and atypical (2) peripheral nerve tumors occurring in an irradiated site suggesting that such tumors represent another delayed effect of radiation treatment on peripheral nerve. The neoplasms appeared 4 to 41 years following radio‐therapy, at a time when the patients (6 females and 3 males) were 16 to 70 years old and cured of their original tumors. Eight patients had received irradiation to treat malignant disease; the ninth had a benign condition.

The sites of peripheral nerve tumor were the brachial plexus in 5, cervical plexus in 1, lumbar plexus in 2, and retroperitoneum in 1. Eight patients presented with painful masses, and in 6 of the 8 there was a progressive neurological deficit. Seven patients died with metastases within two years of diagnosis. In all instances the radiotherapy was within an acceptable radiation dosage, yet 3 patients developed local radiation‐induced skin and bony abnormalities. In 3 patients there were either clinical signs or a family history of von Recklinghausen disease, but the malignant peripheral nerve sheath tumors developed only in the radiation port. Animal studies support the clinical observations that malignant peripheral nerve sheath tumors can occur as a delayed effect of irradiation. Other etiological mechanisms, including an increased risk of second primaries in cancer patients and a genetic predisposition to peripheral nerve tumors, may also play a role.
Serum creatine kinase BB and MM concentrations determined by radioimmunoassay in neuromuscular disorders
Authors: Zweig Mark H. , Adornato Bruce , Van Steirteghem Andre C. , Engel W. King
Published: January 2005

We measured the MM and BB isoenzymes of serum creatine kinase (CK) by radioimmunoassay in 89 patients with neuromuscular disorders and 44 definite or possible carriers of Duchenne muscular dystrophy (DMD). The CK‐MM isoenzyme was closely associated with total CK enzymatic activity. CK‐BB was not as closely correlated with total CK but was usually increased in the inflammatory myopathies and DMD and normal in myasthenia gravis, Guillain‐Barré syndrome, and amyotrophic lateral sclerosis as well as in neuropathies in which CK is usually normal. In myopathies, CK‐BB may be useful for assessing the level of disease activity or the regenerative component. More study is necessary to assess the role of CK‐BB in detection of DMD carriers.
Autosomal dominant cerebrovascular amyloidosis: Properties of peripheral blood lymphocytes
Authors: Stefansson Kari , Antei Jack P. , Oger Joel , Burns James , Noronha AvertanoB. C. , Roos Raymond P. , Arnason Barry G. W. , Gudmundsson G.
Published: January 2005

Selected Properties of Peripheral blood lymphocytes (PBLs) from five ambulatory affected individuals of a kindred with autosomal dominant cerebrovascular amyloidosis were studied. The percentage of PBLs bearing surface membrane immunoglobulin (SmIg + cells) was increased in the patient group (30 ± 3% versus 20 ± 2%; p < 0.05). The Percentage of PBLs forming early and total E‐rosettes was comparable in patient and control groups. Mitogenic response to concanavalin A (Con A) was suggestively reduced in the patient group, measured both by total 3Hthymidine incorporation and by comparison of stimulation indices. Mitogenic response to phytohemagglutinin and pokeweed was comparable in the two groups. Capping of Con A by PBLs was significantly reduced in the patient group compared with the controls (13 ± 1% versus 26 ± 2%; p < 0.01). The findings of reduced Con A response and increased SmIg+ cells support the hypothesis that immune dysfunction contributes to the development of amyloidosis. The reduced capping suggests altered membrane properties in this autosomal dominant disorder.
Glycerol kinase deficiency with neuromuscular, skeletal, and adrenal abnormalities
Authors: Guggenheim Mary Anne , McCabe Edward R. B. , Roig Manuel , Goodman Stephen I. , Lum Gary M. , Bullen William W. , Ringel Steven P.
Published: January 2005

Two brothers with a recently described inborn error of metabolism characterized by glyceroluria, hyperglycerolemia, and generalized glycerol kinase deficincy had moderate psychomotor reatradation, spasticity, growth failure, a nonspecific myopathy, osteoporosis, and adrenal insufficiency. Glycerol kinase activity in leukocytes and cultured fibroblasts was less thaan 5% of control values. Heptic and renal tissue obtained at autopsy in one patient had similarly low enzyme activity. Thus the deficiency of glycerol kinase in these patients appears to be generalized and heritable, though the relationship of the clinical phenotype to enzymatic deect is not yet established.
A diagnostic index of active demyelination: Myelin basic protein in cerebrospinal fluid
Authors: Cohen Steven R. , Brooks Benjamin R. , Herndon Robert M. , McKhann Guy M.
Published: January 2005

We have developed a radioimmunoassay to measure a specific neurological component, the basic protein of myelin, and have used this test for assessing this component in spinal fluid. The levels of basic protein in spinal fluid correlate closely with the clinical activity of multiple sclerosis; therefore the test can be used for objective evaluation of disease activity in patients with that disorder. Moreover, it is a useful adjunct in the diagnosis of multiple sclerosis and evaluation of potential therapy. In addition, the test is helpful in diagnosing other diseases in which acute breakdown of myelin occurs, especially leukoencephalopathy resulting from irradiation and chemotherapy for treatment of leukemia in children.
Epilepsia partialis continua associated with nonketotic hyperglycemia: Clinical and biochemical profile of 21 patients
Authors: Singh Brij M. , Strobos Robert J.
Published: January 2005

In 21 patients, epilepsia partialis continua (EPC) was an early symptom of nonketotic hyperglycemia and occurred during an initial phase of hyponatremia and mild hyperosmolality. EPC persisted for an average of 8 days, and its duration correlated predominantly with the degree of hyponatremia. Depression of consciousness and cessation of seizures occurred with increasing severity of hyperglycemia and hyperosmolality. In 9 patients, EPC was the first symptom leading to the diagnosis of diabetes mellitus. Four patients died of serious associated illness.

The majority of the patients had evidence of a localized structural cerebral lesion. Metabolic disturbances including hyperglycemia, mild hyperosmolality, hyponatremia, and lack of ketoacidosis contribute to the development of EPC in areas of focal cerebral damage.
Methanol poisoning: A clinical and pathological study
Authors: McLean Donald R. , Jacobs Harold , Mielke Bruce W.
Published: January 2005

We report 2 survivors of severe methanol poisoning who developed, apart from blindness, a Parkinson‐like extrapyramidal syndrome characterized by reduced initiative, poor voice volume, masked facies, mild tremor, rigidity, and bradykinesia. Both patients were mildly demented and 1 had hyperreflexia and bilateral Babinski responses.

Computed tomographic scans in both patients demonstrated bilateral symmetrical infarction of the frontocentral white matter and putamen. Electromyography in 1 patient showed extensive denervation, mainly involving the legs, but normal motor conduction velocities. L‐Dopa administered to the more severely affected patient had no effect on the parkinsonian features.

Autopsy revealed cystic resorption of the putamen and the frontocentral subcortical white matter in addition to widespread neuronal damage throughout the cerebrum, cerebellum, brainstem, and spinal cord.
Distal neuropathy in experimental diabetes mellitus
Authors: Brown Mark J. , Sumner Austin J. , Greene Douglas A. , Diamond Susan M. , Asbury Arthur K.
Published: January 2005

Although nerve conduction slowing is a well‐accepted abnormality in rats with acute experimental diabetes, reports of neuropathological changes in diabetic rat nerves have been inconsistent. To examine this further, we studied electrophysiological and morphological features of posterior tibial nerves and their distal branches from four‐week streptozotocin‐induced diabetic rats and matched controls.

Diabetic rat posterior tibial motor conduction was slowed (mean ± 1 SD, 34.8 ± 3.1 m/sec; controls, 41.2 ± 2.5 m/sec), and evoked muscle response amplitudes were only half of control values. Using quantitative techniques, we documented a diminution in number of the largest myelinated fibers in otherwise normal mid posterior tibial nerves, with an increase in the smaller sizes, indicating either a degree of axonal atrophy or impaired fiber growth during development. The principal pathological finding was active breakdown of myelinated fibers in the most distal motor twigs of hind foot muscles supplied by posterior tibial branches, with preservation of fibers in more proximal segments of these nerves. This anatomical lesion in diabetic nerves could account for both observed conduction slowing and lowered muscle response amplitudes. A consistent feature in both diabetic and control mid lateral plantar nerves was a zone of demyelination that apparently occurs at this natural site of nerve entrapment in rats. Taken together, the pathological abnormalities of peripheral nerve in acute experimental diabetes are best explained as resulting from a distal axonopathy.
Brainstem tumor presenting with unilateral astereognosis
Authors: Feinsod Moshe , Bentin Shlomo , Moscovitch Morris , Wald Uri
Published: January 2005

A patient with a brainstem tumor presented with astereognosis. The results of CT scan, evoked response studies, and neuropsychological tests all were consistent with a noncortical origin for the sensory defect.
Congenital stiff‐man syndrome
Authors: Sander James E. , Layzer Robert B. , Goldsobel Alan B.
Published: January 2005

A second large family with a dominantly inherited benign disorder resembling the stiff‐man syndrome is reported. The infants are markedly hypertonic at birth, but their tone becomes almost normal by 3 years of age. Stiffness reappears at adolescence, often precipitated by sudden movement or cold, but remains mild when compared with the sporadic disorder.
Neonatal intracranial abscess: Two cases caused by citrobacter and a literature review
Authors: Curless Richard G.
Published: January 2005

Intracranial abscesses are uncommon in the newborn. Observation of 2 infants and a review of the literature reveal that the offending organism was a Citrobacter in 12 of the 16 documented cases. In the Neonatal Meningitis Cooperative Study this organism was responsible for meningitis in only 4%. However, an analysis of all published cases of neonatal Citrobacter meningitis reveals a 42% incidence of intracranial abscess. Accordingly, early identification by computerized tomography is important in facilitating prompt surgical therapy.
Complex partial status epilepticus following myelography with metrizamide
Authors: Russell D. , Anke I. M. , Nyberg‐Hansen R. , Sletcnes O. , Sortland O. , Veger T.
Published: January 2005

Complex partial status epilepticus developed in two patients following myelography with metrizamide. The status epilepticus was manifested by confusion and complex motor symptoms, and electroencephalograms (EEGs) showed ictal activity alternating independently over both hemispheres. Immediate clinical improvement occurred with antiepileptic treatment, and to date no sequelae have been observed. Neither patient had a previous history of epileptic seizures, but both had preexisting EEG abnormalities.
Compressive myelopathy in maroteaux‐lamy syndrome: Clinical and pathological findings
Authors: Young Richard , Kleinman George , Ojemann Robert G. , Kolodny Edwin , Davis Kenneth , Halperin John , Zalneraitis Edwin , Robert DeLong G.
Published: January 2005

A 41‐year‐old woman with Maroteaux‐Lamy syndrome developed spastic tetraparesis due to a compressive cervical myelopathy. There was substantial improvement following lammectomy and excision of the markedly thickened dura. Light and electron microscopic studies showed extensive accumulations of balloon cells in the dura.
Thrombotic stroke associated with elevated plasma factor VIII
Authors: Kosik Kenneth S. , Furie Bruce
Published: January 2005

A previously healthy 43‐year‐old woman developed bilateral middle cerebral artery occlusion, thrombocytopenia, and multiple thrombi of arteries in the spleen. No underlying cause of her thrombotic disease was detected except for a factor VIII level that was elevated fivefold. An etiological relationship between the cerebral occlusive disease and elevated factor VIII level is suggested. We recommend that factor VIII levels be assayed in patients with stroke when an underlying disorder is not identified.
Generalized chorea associated with chronic subdural hematomas
Authors: Bae Sung H. , Vates Thomas S. , Kenton Edgar J.
Published: January 2005

Two patients with chronic bilateral subdural hematomas developed symmetrical generalized choreiform movements. In one patient the chorea disappeared after evacuation of the hematomas, and in the other the movements improved. The latter patient has a family history of Huntington's disease.
Correlation of intracellular redox states and pH with blood flow in primary and secondary seizure foci
Authors: Tenny Robert T. , Sharbrough Frank W. , Anderson Bas Robert E. , Sundt Thoralf M.
Published: January 2005

Epileptogenic foci were created by topical application of penicillin to the cerebral cortex in 40 paralyzed and artificially ventilated cats receiving halothane anesthesia. The animals were divided into two equal groups to compare primary and secondary foci. The following variables were recorded at normocapnia, hypocapnia, and hypercapnia prior to and during seizure activity: cerebral blood flow (CBF), determined by clearance of xenon 133; cortical redox states, measured by the fluorescence of reduced pyridine nucleotides (PN); brain pH, measured using a lipid‐soluble, pH‐sensitive fluorescent indicator; and electroencephalograms (EEG). Mean arterial blood pressure, arterial pH, arterial carbon dioxide tension (Pa), and arterial oxygen tension (Pa) were monitored in each animal. All animals had a normal Pa‐CBF response prior to the creation of a seizure focus, assuring the presence of autoregulation and normal metabolic function.

CBF increased equally with seizures in the primary and secondary hemispheres. The relative increase was related to the Paco2 but approximated 68% at normocapnia. There was an alteration in the Paco2‐CBF response with seizures, but the ability of the cerebral vasculature to constrict and dilate with hypocapnia and hypercapnia was retained. There was no significant difference in the reduced PN signal with variations in Paco2 prior to seizures, but there was an apparent 10 to 15% fall with seizures. The “equivalent” intracellular pH fell to 6.94 at normocapnia in the primary focus but remained essentially unchanged from the control value of 7.10 in the secondary focus. These differences in pH were consistent with the greater degree of seizure activity observed in the primary focus. We conclude that a nonhypoxic acidosis existed in the primary focus and that changes in CBF were not related to it because the CBF changed equally in both hemispheres.
Human diabetic endoneurial sorbitol, fructose, and myo‐inositol related to sural nerve morphometry
Authors: Dyck Peter James , Sherman William R. , Hallcher Loretta M. , John Service F. , O'Brien Peter C. , Grina Lucy A. , Palumbo Pasquale J. , Swanson Carol J.
Published: January 2005

Fascicles of the sural nerve from each of 20 diabetic patients, mostly with maturity‐onset diabetes, were studied by biochemical and pathological techniques, and results were compared to values found in nerve specimens from 15 healthy persons. The sorbitol and fructose content was much more variable in diabetic than in healthy nerves. More than one‐third of the diabetic nerves had sorbitol and fructose values above the highest levels for controls. myo‐Inositol and scyllo‐inositol content was not reduced in diabetic nerves. The sorbitol, fructose, and inositol concentrations could not be related to clinical, neurophysiological, or pathological severity of neuropathy. A comparison of scored symptoms and signs and clinical neurophysiological studies against morphometric and teased fiber studies of sural nerve demonstrated that the former three provide sensitive and reliable measures of severity of neuropathy that can be used for controlled clinical trials of diabetic neuropathy. The presence and type of teased fiber abnormalities could be related to the duration of diabetes and to symptoms of neuropathy. In untreated diabetics without symptoms of neuropathy, a higher than normal frequency of teased fibers showing segmental demyelination and remyelination was found. Untreated diabetics with symptomatic neuropathy showed two kinds of abnormalities: fibers with segmental demyelination and remyelination and fibers undergoing axonal degeneration. In treated diabetics, who often had longstanding neuropathy, the most common abnormalities were fibers undergoing axonal degeneration.
Encephalitis after inhalation of measles virus: A pathogenetic study in hamsters
Authors: Parhad Irma M. , Johnson Kenneth P. , Wolinsky Jerry S. , Swoveland Peggy
Published: January 2005

A neuroadapted strain of measles virus (HNT) was administered by inhalation to newborn hamsters. Primary replication of virus in the lung was followed by the transient appearance of virus in spleen within 7 to 9 days of inoculation. A terminal encephalitis occurred between 6 and 60 days in 31% of infecfted hamsters, and virus was recovered by explant culture of these brains. Virus could not be cultured directly from brain or tissue homogenates. At least 7% of hamsters that had survived the infection for two months had antibody to measles virus. The histopathological change in morbid animals was limited to the central nervous system (CNS) and consisted of small foci of necrosis, perivascular mononuclear cell infiltrates, intracytoplasmic and intranuclear inclusions, and vacuolated pyknotic neurons. Immunofluorescent studies disclosed measles antigen in the lungs and brain. This hamster moENl of measles encephalitis following a “natural” route of inoculation appears to represent a faithful reproduction of certain CNS complications of natural measles infection in humans, i.e., measles encephalitis and subacute sclerosing panencephalitis.