scispace - formally typeset
Search or ask a question

Showing papers in "Journal of Neurology in 1977"


Journal ArticleDOI
TL;DR: In this article, the authors present evidence that thin layer polyacrylamide isoelectric focusing of the CSF gamma globulins is by far superior for this purpose.
Abstract: Despite enormous efforts to find a specific laboratory test for multiple sclerosis, agar gel electrophoresis of the CSF proteins has remained the next to the best one. This study presents evidence that thin layer polyacrylamide isoelectric focusing of the CSF gamma globulins is by far superior for this purpose. In effect, about 91% of the 262 multiple sclerosis patients studied had oligoclonal fractions present in the very alkaline region of the pH gradient. Of the same group of patients, only 65% did show pathological results when studied by agar gel electrophoresis. Of the 272 CSF samples from patients suffering from other neurological diseases, only about 7% showed the presence of oligoclonal bands in the same region of the pH gradient, when submitted to isoelectric focusing.

134 citations


Journal ArticleDOI
TL;DR: Both mortality and morbidity data indicate quite clearly that multiple sclerosis is a geographically-related disease, and thus MS can be thought of as an acquired environmental (exogenous) illness.
Abstract: Both mortality and morbidity data indicate quite clearly that multiple sclerosis is a geographically-related disease, and thus MS can be thought of as an acquired environmental (exogenous) illness. High frequency parts of the world for MS are Europe between 65 degrees and 45 degrees north latitude, northern United States and southern Canada, New Zealand, and southern Australia. These regions are bounded by medium frequency MS regions: in Europe to the north, east, and south; in America for southern U.S.; and the remainder of Australia. Latin America, Asia and Africa are essentially of low frequency from present data. Latitude is not a sufficient criterion: at 40 degrees north latitude, MS is high in America, medium in Europe, and low in Asia. All high and medium risk areas therefore are in Europe or European colonies; thus MS is the white man's burden spread from western Europe. Within the U.S., MS is less common among Negroes, Japanese, and possibly Amerindians than in whites regardless of geography. Migration studies among risk areas indicate that migrants keep much of the risk of their birthplace, but also that overall the risk is decreased by high-to-low migration, and probably increased by low-to-high. For the former, it seems that adolescence is the age critical for retention of birthplace risk. Some preliminary data on a possible epidemic of MS are also presented. All the epidemiologic information would be most easily explained if MS were an infectious (viral) illness with prolonged latency. The proof of this though must come from the laboratory.

133 citations


Journal ArticleDOI
TL;DR: Cerebral amyloid angiopathy may be considered a rare but important cause of cerebral hemorrhage in the aged, and the “vascular” type of presenile dementia, occasionally complicated by focal cerebrovascular lesions or bleeds, is considered a variant of Alzheimer's disease.
Abstract: More than 1400 necropsies performed on patients with either a non-traumatic cerebral hemorrhage (400 cases) or with dementia over the age of 55 (1010 cases), or both, have been reviewed. There were 15 cases in which a cerebral hemorrhage had occurred together with cerebral amyloid angiopathy all of whom had been demented. Eight of the 15 patients were hypertensive. The 7 non-hypertensives showing only the amyloid change included two cases of “atypical” Alzheimer's disease with acute neurological features, and 5 cases of senile dementia (aged 72 to 78 years) coupled with focal neurological disorders. In the hypertensive patients, aged 67 to 86 years, with a progressive dementing syndrome and acute neurological signs, multiple ball-like hemorrhages (7 cases) and/or cerebral hematomas (3 cases) were associated with a combination of amyloid and hyalinar (hypertensive) angiopathy, often affecting segments of the same pial and cortical vessels. From these data and recent reports on lethal cerebral hemorrhage occurring spontaneously or after neurosurgical procedures in demented old people, cerebral amyloid angiopathy, which is not necessarily associated with systemic amyloidosis or severe (pre)senile cerebral degeneration, may be considered a rare but important cause of cerebral hemorrhage in the aged. The “vascular” type of presenile dementia, occasionally complicated by focal cerebrovascular lesions or bleeds, is considered a variant of Alzheimer's disease. The mechanism leading to formation of cerebral amyloid is unknown.

129 citations


Journal ArticleDOI
TL;DR: It is concluded from the data n-hexane and MBK have a common toxic mechanism with primary axonal changes and that there is an additional synergistic effect of MEK.
Abstract: In West Berlin in the autumn of 1975 through the following 5 months we observed 18 juvenile patients who had a toxic polyneuropathy and had sniffed a glue thinner. The neurological picture consisted of a symmetrical, progressive, ascending, mainly motor, polyneuropathy with pronounced muscle atrophy and characteristic vegetative alterations. The height of the disease was reached after 1 1/2-2 1/2 months and was characterized by tetraplegia in 7 patients. After 8 months all patients still had a motor deficit. Nerve biopsy showed paranodal axon swelling, dense masses of neurofilaments and secondary myelin retraction. The neurological and morphological data correspond to the "glue sniffer's neuropathy" and the n-hexane and MBK polyneuropathy after industrial exposure, as described in 10 cases to date. However, there was no MBK in the glue thinner. The polyneuropathies occurred in close time relation with the denaturation of the thinner with MEK (2-butanone). It is concluded from the data n-hexane and MBK have a common toxic mechanism with primary axonal changes and that there is an additional synergistic effect of MEK.

117 citations


Journal ArticleDOI
TL;DR: It is concluded that intensive immunosuppression is able to interfere with the pathological processes involved in the pathogenesis of disseminated sclerosis.
Abstract: 140 MS patients were treated with intensive I.V. cyclophosphamide immunotherapy and 110 were followed over 2–4 years. Annual relapse rate incidence was calculated over a period of 2 years before and after treatment and repeated neurological scores were made during this period.

94 citations


Journal ArticleDOI
TL;DR: Changes with temperature of maximum sensory nerve conduction velocity as well as absolute and relative refractory periods were tested in 14 human subjects, with a tendency showing a more pronounced temperature effect at low temperatures.
Abstract: Changes with temperature of maximum sensory nerve conduction velocity as well as absolute and relative refractory periods were tested in 14 human subjects. Corresponding to previously published findings maximum conduction velocity decreased with cooling following a Q10 of +1.4. The absolute and relative refractory periods were increased by cooling, the Q10 being −3.1 and −3.35 respectively. There was a tendency showing a more pronounced temperature effect at low temperatures. The Q10 and the steepness of the regressionline changed at the level of 26.9°C, but were significant for the relative refractory period only.

94 citations


Journal ArticleDOI
TL;DR: The results showed that the injured brain did not tolerate the exposure to an oxygen pressure of 2.0 ATA for 10 to 15 min, but exposure to 1.5 ATA was tolerated and had a favorable effect on the glucose or energy metabolism of the brain as well as on the clinical course.
Abstract: The object of this study was to ascertain the oxygen tolerance limit and the oxygenation state of the injured brain in man. While breathing air, oxygen and hyperbaric oxygen at pressures of 1.5 and 2.0 atmospheres absolute (ATA), the cerebral arteriovenous differences (AVD) for O2, glucose, lactate, pyruvate and blood gas pressures and pH values were measured. The balance of the cerebral glucose metabolism was calculated. The results showed that the injured brain did not tolerate the exposure to an oxygen pressure of 2.0 ATA for 10 to 15 min, but exposure to 1.5 ATA for 35–40 min was tolerated and had a favorable effect on the glucose or energy metabolism of the brain as well as on the clinical course. There was a distinctly increased cerebral glycolysis while breathing air indicating insufficient oxygen delivery to the brain. The change from breathing air to oxygen resulted in a distinct inhibition of cerebral glycolysis, which indicated improved cerebral oxygenation and energy production and gave evidence for a “Pasteur effect” regulating the glucose metabolism of the injured brain in man. At an inspiratory oxygen pressure of 1.5 ATA we had a nearly balanced cerebral glucose metabolism indicating an adequate cerebral oxygenation and energy formation. Further increase in inspiratory oxygen pressure to 2.0 ATA (performed only in group A) increased cerebral glycolysis considerably. This was assumed to be due to cerebral oxygen poisoning resulting in disturbed oxidative energy formation. Following this alteration an extreme reduction of the cerebral glucose uptake appeared, probably due to a disturbance of the specific glucose transport system. These metabolic alterations were not accompanied by seizures or any other clinical neurological manifestation. In group B, exposed to 1.5 ATA, such alterations of the cerebral glucose metabolism did not appear. A nearly balanced cerebral glucose metabolism was found at inspiratory oxygen pressures of 1.0 and particularly of 1.5 ATA, indicating an improved oxygenation and energy production of the affected brain. Finally, a renewed increase of the cerebral glycolysis occurred following the change from breathing oxygen to air. This again indicated an insufficient oxygen delivery to the affected brain.

86 citations


Journal ArticleDOI
TL;DR: The duration of the compound sensory action potentials recorded from the median nerve at the wrist and elbow shows a negative temperature coefficient and the peak-to-peak amplitude of these potentials increases from 22° to approximately 26°C and then decreases progressively again up to 36°C.
Abstract: Sensory conduction velocities of normal subjects are increasing linearly with rising temperature. The duration of the compound sensory action potentials recorded from the median nerve at the wrist and elbow shows a negative temperature coefficient. The peak-to-peak amplitude of these potentials increases from 22° to approximately 26°C and then decreases progressively again up to 36°C. It is believed that this behavior is due to a combination of decreasing temporal dispersion, height and duration of the individual spike potentials.

71 citations


Journal ArticleDOI
TL;DR: In comparison with the prelevodopa era, the reduction of mortality and the increase of life expectancy of patients with Parkinson's disease during levodopa treatment possibly reflect the decrease of the early mortality due to Parkinson's Disease.
Abstract: The effect of levodopa on the mortality of patients with Parkinson's disease was investigated in 349 patients treated with levodopa or levodopa combined with a decarboxylase inhibitor during 1969–1975 inclusive. During the study period, 61 patients died. The expected mortality was 32.99 resulting in a ratio of actual to expected deaths of 1.85. The excess mortality was accounted for by patients with a severe disease at entry and especially, by the less favorable effect of levodopa treatment than in the living patients. In comparison with the prelevodopa era, the reduction of mortality and the increase of life expectancy of patients with Parkinson's disease during levodopa treatment possibly reflect the decrease of the early mortality due to Parkinson's disease.

64 citations


Journal ArticleDOI
TL;DR: It is concluded that basic protein therapy does not influence the course of MS and the annual relapse rate, estimated over a period of 2 years before and after BP therapy, was not modified.
Abstract: Basic protein therapy was applied to 35 MS patients. No deleterious effects were observed but 46% of patients experienced relapses during treatment. The annual relapse rate, estimated over a period of 2 years before and after BP therapy, was not modified. It is concluded that basic protein therapy does not influence the course of MS.

53 citations


Journal ArticleDOI
TL;DR: The similarities in the morphological features and pattern of lesions of the CNS vessels in both isolated and generalized forms of giant cell granulomatous angiitis are in favor of a common basic disorder of hitherto unknown etiology.
Abstract: This is a report of five autopsied cases of giant celled granulomatous angiitis of the central nervous system. There were tree males and two females ranging in age from 42 to 71 years, with an acute course in two, death occurring within one and five weeks, and longer duration in three, with recurrent illness of 16 months to 5 years. The clinical features were mental changes associated with hemiparesis or tetraparesis, extrapyramidal signs, visual disorders with optic atrophy, and brainstem syndromes with final coma. Typical CSF findings were mild lymphocytosis with increased protein and gamma globulin levels. General autopsy disclosed generalized giant cell angiitis with aortitis in two cases, in one of which the diagnosis had been made by superficial temporal artery biopsy, while in three patients the inflammatory lesions were confined to the CNS vasculature, affecting all types and sizes of vessels. Although there were no significant differences between the cases with systemic angiitis with CNS involvement and those with vascular lesions limited to the CNS, the latter group showed predominance of the disorder for the small leptomeningeal and intracerebral or spinal vessels without involvement of the large intra- and extracranial brachiocephalic arteries. The latter are more often involved with cranial or systemic giant cell angiitis. The CNS showed disseminated microinfarctions of the brain and spinal cord (2 cases) or larger recent and old infarcts. The similarities in the morphological features and pattern of lesions of the CNS vessels in both isolated and generalized forms of giant cell granulomatous angiitis are in favor of a common basic disorder of hitherto unknown etiology. Granulomatous angiitis of the CNS is, therefore, regarded as a local variant of giant cell vasculitis rather than a separate nosological entity. From recent data, the disease is perhaps best regarded as an immune complex disorder, although viral infection is also considered.

Journal ArticleDOI
TL;DR: Two cases with complete and one with partial anterior tarsal tunnel syndrome are presented, etiology, symptomatology, differential diagnosis and therapeutic possibilities are discussed.
Abstract: The anterior tarsal tunnel syndrome, first described in 1968 by Marinacci, is characterized by a compression of the deep peroneal nerve under the inferior extensor retinaculum. The patients complaint of pains on the dorsum of the foot, especially at night. Clinically result sensory deficits in the involved area between the first and second toes as well as paresis and atrophy of the extensor digitorum brevis. The distal latency of the deep peroneal nerve is increased, the EMG shows active and chronic denervation of the extensor digitorum brevis. In cases with partial anterior tarsal tunnel syndrome only the motoric branch to the extensor digitorum brevis or only the sensory branch of the deep peroneal nerve after the division under the inferior extensor retinaculum is compressed. Two cases with complete and one with partial anterior tarsal tunnel syndrome are presented, etiology, symptomatology, differential diagnosis and therapeutic possibilities are discussed.

Journal ArticleDOI
TL;DR: A low prevalence and mortality rate of cancer among multiple sclerosis patients and the possible role of selenium, one of the antioxidants in the pathogenesis of MS and cancer, is discussed because recent data have shown a very high negative correlation between seenium and cancer death rates.
Abstract: The frequency of malignant diseases among 1866 living and 340 deceased multiple sclerosis (MS) patients was investigated in Finland. The study revealed a low prevalence (0.64%) and mortality (0.07%) rate of cancer among MS patients. The difference between MS patients and general population was significant. The highest rate was found in the group from 40 to 49 years while in the general population the rate of cancer among MS patients tended to fall after the age of 50. The possible role of selenium, one of the antioxidants in the pathogenesis of MS and cancer, is discussed because recent data have shown a very high negative correlation between selenium and cancer death rates.

Journal ArticleDOI
TL;DR: The effect of LA and AA on the absolute mobility of RBC may be used as a simple laboratory test involving a long established technique and eliminating the animal and other needs of the macrophage electrophoretic mobility (MEM) test.
Abstract: Erythrocytes from patients with Multiple Sclerosis (MS) show a highly significant reduction in their absolute electrophoretic mobility in the presence of linoleic and arachidonic acids (LA; AA). Patients with other (destructive) neurological disease (OND) and normal subjects show an increased absolute mobility of their erythrocytes in the presence of LA and AA. About 40 per cent of blood relatives of MS patients show an intermediate type of reaction - being slowed by LA and speeded up by AA. Administration of LA (or gamma linolenate) to an MS patient for some months leads to change in the mobilities from the MS to normal type, the AA result altering first. The effect of LA and AA on the absolute mobility of RBC may thus be used as a simple laboratory test involving a long established technique and eliminating the animal and other needs of the macrophage electrophoretic mobility (MEM) test. The implications of these findings for our understanding and handling of MS are briefly discussed.

Journal ArticleDOI
TL;DR: The compound muscle action potential (MAP) and the isometric twitch force of the adductor pollicis of 25 normal subjects were registered after local cooling and the amplitude increased while the twitch force decreased at lower temperatures.
Abstract: The compound muscle action potential (MAP) and the isometric twitch force of the adductor pollicis of 25 normal subjects were registered after local cooling. Intramuscular temperature ranged from 36.6 to 18.0°C. The amplitude of the MAP increased while the twitch force decreased at lower temperatures. The increase of the MAP could be caused by the effect of cooling directly at the muscle cell membrane. The decreased twitch force probably is due to an effect on the contractile apparatus.

Journal ArticleDOI
TL;DR: The neuropathy was diagnosed on the basis of the whole clinical picture and the neurophysiological findings and the pathogenesis of the peripheral nerve disease is discussed in the light of the evidence in the literature.
Abstract: Seven patients with neuropathy associated with vitamin B12 deficiency are reported. Four of them had other signs of malabsorption aside from the abnormal Schilling test. The neuropathy was diagnosed on the basis of the whole clinical picture and the neurophysiological findings. The pathogenesis of the peripheral nerve disease is discussed in the light of the evidence in the literature.

Journal ArticleDOI
TL;DR: Electromyographic and neurographic investigations were performed on 20 persons with AIP and motor nerve conduction velocity was mostly decreased in combination with denervation signs and in a range that indicated a primarily axonal nerve lesion and consequent myelin damage rather than primary demyelinization.
Abstract: Diminished activity of uroporphyrinogen I-synthetase in the liver and other tissues may be regarded to be the primary genetic deficiency of acute intermittent porphyria (AIP). Increased production and renal excretion of delta-aminolevulinic acid (ALA) und porphobilinogen (PBG) are secondary phenomena. The neuropsychiatric symptomatology of AIP consists of neuropathy, vegetative crises and exogenous psychoses. In this study electromyographic and neurographic investigations were performed on 20 persons with AIP. 16 patients had experienced attacks of AIP, 10 of them including neuropathy. 4 persons showed the biochemical findings of AIP but had not yet had symptoms. In cases with persistent pareses following porphyric neuropathy denervation signs or sequelae were still present. In patients without clinical symptoms and in latent cases there were normal or borderline findings. Motor nerve conduction velocity was mostly decreased in combination with denervation signs and in a range that indicated a primarily axonal nerve lesion and consequent myelin damage rather than primary demyelinization. The mean motor conduction velocity of n. tibialis was somewhat lower in patients with porphyric crises without neuropathy than in latent cases without any clinical crises. The differences were not significant in other nerves. The findings are discussed under consideration of the electrodiagnostic results of other investigations and of neuropathological and clinical data.

Journal ArticleDOI
TL;DR: The MEG, as recorded with this particular magnetometer, tending to show diffuse, non-localizable changes associated with localized EEG abnormalities may have its maximum value in helping to determine the dipolar orientation of various EEG waveforms by simultaneously recording the MEG and EEG and also occasionally in its ability to record activity that is not found in the EEG.
Abstract: The EEG and also the MEG were recorded simultaneously from 10 patients with various types of EEG abnormalities; the characteristics of the MEG and its relationship to the EEG were investigated with the use of a digital computer Examples are shown in which the EEG activity is poorly represented in the MEG and include one configuration of a slow wave with a slow descending limb (intermixed with a more sinusoidal waveform), posterior slow waves and the wave of the 3/sec spike and wave complex Magnetic theory would suggest that these patterns are associated with dipolar sources oriented radially or perpendicularly to the outer surface of the cortex and each pattern is discussed in relation to this possibility Examples are shown in which a given pattern is well represented in both MEG and EEG recordings These include eye-blink artifact, slow waves from a tumor, diffuse theta activity, sinusoidal anterior delta rhythms and the spike of the 3/sec spike and wave complex Magnetic theory would suggest that these patterns are likely associated with a dipolar source oriented tangentially or parallel to the cortex's surface and each pattern was appropriately discussed An example is shown of a horizontally oriented delta EEG focus and its corresponding longitudinally oriented magnetic field The few examples of activity recorded better in the MEG than EEG include some rare instances of slow waves associated with a known tumor, alpha activity in a patient with diffuse delta rhythms and the harmonic components of the 3/sec spike and wave complex The MEG, as recorded with this particular magnetometer, tending to show diffuse, non-localizable changes associated with localized EEG abnormalities may have its maximum value in helping to determine the dipolar orientation of various EEG waveforms by simultaneously recording the MEG and EEG and also occasionally in its ability to record activity that is not found in the EEG

Journal ArticleDOI
TL;DR: Continuous activity of motor units with superimposed bursts during muscular spasms was found by electromyography in the two patients reported, suggesting a central genesis, of the pathogenetic mechanisms discussed, is the most probable in these cases.
Abstract: In addition to anamnestic and clinical data electrophysiological and pharmacokinetic investigations may be necessary for the diagnosis of stiff man syndrome. Continuous activity of motor units with superimposed bursts during muscular spasms was found by electromyography in the two patients reported. Rigidity and continuous activity disappears during sleep, after i. v. application of Tubocurarine and Diazepam, during Thiopental anesthesia and after neural block with Procaine. Dipropylacetate and Baclofen improved the condition but did not lead to complete relaxation. Biperidenlactat and Magnesiumlaevulinat have only a temporary effect on rigidity. Neostigmine, Phenytoine, Glycine, Dopa and 5-Hydroxy-Tryptophan had no effect. Passive shortening or stretching of the m. biceps brachii as well as touching the skin increased motor activity which spread to other segments and to the contralateral side. The H/M ratio was increased but the silent period was normal. A combination of Diazepam and Dipropylacetate or Clonazepam was therapeutically effective in the cases reported. A central genesis, of the pathogenetic mechanisms discussed, is the most probable in our cases.

Journal ArticleDOI
TL;DR: The effect of local cooling was studied in 28 patients with myastenia gravis and in many cases a complete failure of the neuromuscular transmission occured, ruling out a practicable application of cooling for the patient.
Abstract: The effect of local cooling was studied in 28 patients with myastenia gravis. We stimulated the ulnar nerve with single stimuli and trains at 3/s for 2s and at 50/s for 1.5 s. The compound muscle action potential (MAP), the muscle twitch and the isometric tetanic force of the adductor pollicis were registered. 1. At 3/s stimulation the pathological decrement of the MAP decreased after slight cooling. 2. The amplitude of the single MAP was higher at lower temperature when compared to normal temperature. The same increase is however to be found in healthy subjects. 3. After slight cooling, the maximum tetanic force was higher. However, the decrement of the force was higher also, therefore ruling out a practicable application of cooling for the patient. 4. After severe cooling (18-22 degrees C) the tetanic force was much lower and in many cases a complete failure of the neuromuscular transmission occured.

Journal ArticleDOI
TL;DR: The greater reduction of the compound action potential of the m.orbicularis oris in recurrent homolateral facial palsy in relation to patients with single manifestation point out the greater denervation and therefore the poorer prognosis of recurrent palsies.
Abstract: The etiology, frequency and prognosis of recurrent Bell's palsy were studied in patients with peripheral paresis of n.facialis of various etiology. Fourteen (11.9%) of 117 patients with Bell's palsy had a recurrent paresis of n.facialis. Nine of these were of homolateral and five contralateral type. Seven recurrent facial paralyses in idiopathic Bell's palsy (i.e. 10.4% of the patients with idiopathic facial palsy), 3 facial palsies of viral origin (i.e. 8.8% of the patients with viral facial palsy), one in association with diabetes mellitus, one during pregnancy, one combined with positive rheumatic serological tests, and one in a case of Melkersson-Rosenthal syndrome were found. The frequency and heterogenity of etiology of recurrent facial palsies suggest a predisposing factor or immune mechanisms. In eight patients there was within 6 to 8 weeks a good, in two patients a moderate and in four patients a poor recovery of function. The greater reduction of the compound action potential of the m.orbicularis oris in recurrent homolateral facial palsy in relation to patients with single manifestation point out the greater denervation and therefore the poorer prognosis of recurrent palsies. A prophylactic decompression to prevent a third attack of recurrent facial paresis is considered.

Journal ArticleDOI
TL;DR: Analytical methods support the hypothesis of IgG synthesis by cells accumulating within the CNS in MS, which support the correlation of the laboratory results and clinical manifestation of MS.
Abstract: The leucocyte count, total protein, albumin, IgG, IgA and IgM content of the cerebrospinal fluid (CSF) of 103 multiple sclerosis (MS) patients was determined. In 54 cases a simultaneous analysis of serum was also carried out. As a sign of an intact blood brain barrier the albumin concentration was normal in 76.7%. Taking into account the relative IgG quotient in CSF and serum, and the albumin and IgG concentration gradients between CSF and serum, it was possible to reveal an elevation of IgG content in CSF of MS patients in 75 and 83%, respectively. Without a simultaneous analysis of serum this was the case only in 51.5%. In MS cases with an intact blood brain barrier the values for IgA and/or IgM were slightly elevated in 11.7%. This study demonstrates analytic methods, which support the hypothesis of IgG synthesis by cells accumulating within the CNS in MS. A correlation of the laboratory results and clinical manifestation of MS was tried.

Journal ArticleDOI
TL;DR: Muscle biopsy revealed areas with loss of cross striations in the muscle fibers, electronmicroscopically presenting complete disorganization of the myofibrils with streaming of the Z discs and absence of mitochondria.
Abstract: Two patients, a brother and sister, both suffering from congenital generalized muscle weakness with a progressive course are reported. Muscle biopsy revealed areas with loss of cross striations in the muscle fibers, electronmicroscopically presenting complete disorganization of the myofibrils with streaming of the Z discs and absence of mitochondria. Vesicular nuclei with prominent nucleoli were present in these areas. There was a type I fiber predominance in both cases. The mean diameter of the type I muscle fibers in one of the cases was too small. Motor endplate alterations in this patient gave no evidence of denervation but were suggestive of a delayed development of motor nerves.

Journal ArticleDOI
TL;DR: It was found that urinary excretion of histamine was increased on one or more occasions in 7 of 22 patients with cluster headache, and the excretion was significantly higher on attack days than on attack free days.
Abstract: Urinary excretion of histamine, as well as histaminuria following intravenous L-histidine loading, were studied in patients with so-called vascular headache. It was found that urinary excretion of histamine was increased on one or more occasions in 7 of 22 patients with cluster headache. The excretion was significantly higher on attack days than on attack free days. With migraine, increased excretion was found in 5 of 31 patients on days of an attack, whereas the corresponding figure for headache free days was 7 of 24 patients. Three patients showed increased histamine excretion during, as well as between, attacks. The excretion on attack days was not significantly different from that on attack free days. In cluster headache patients, L-histdine administration on attack days did not indicate that an increased histamine formation took place under such circumstances. The underlying mechanism behind the increased histamine output with cluster headache may be increased formation or liberation or altered catabolism. Histamine is more likely to be a consequence than the cause of an attack of cluster headache.

Journal ArticleDOI
TL;DR: It is suggested that neuroaxonal degeneration and incomplete regeneration may represent the major pathogenetic mechanism of the neuropathy in these hypothyroid patients with manifestations of a diffuse peripheral neuropathy.
Abstract: Clinical and sural nerve biopsy findings are described in four hypothyroid patients with manifestations of a diffuse peripheral neuropathy. Clinical examination revealed distal sensory inpairment, complaints of burning and lancinating extremity pains, ataxia and a decrease of deep tendon reflexes with total ankle jerk loss. In one patient, signs of the peripheral neuropathy comprised the leading symptomatology of the underlying metabolic disorder. Light and electron microscopic investigations of the sural nerve biopsies revealed a marked reduction of myelinated fibers, affecting mainly the large myelinated axons. The histograms showed a pathological unimodal spectrum of myelinated fiber diameters. Dystrophic and degenerative changes in axons of all fiber classes and the presence of clusters of thinly myelinated small fibers suggest that neuroaxonal degeneration and incomplete regeneration may represent the major pathogenetic mechanism of the neuropathy in these hypothyroid patients.

Journal ArticleDOI
TL;DR: No correlation was found between the results of thymectomy and the duration of the disease, sex or histological findings in the thymus, and the Dauer of the Erkrankung, Geschlecht or Histologie des exstirpierten Thymus andererseits.
Abstract: The results of thymectomy of 28 myasthenic children observed from 1 to 9 years after operation are discussed. Improvement was obtained in 19 cases (67.8%), with full remission in 12 cases (42.8%). Six children died. No correlation was found between the results of thymectomy and the duration of the disease, sex or histological findings in the thymus. No increase of incidence or severity of infections were observed in thymectomized children. Caution is recommended regarding vaccination of myasthenic children.

Journal ArticleDOI
TL;DR: In this article, the effect of local cooling has been studied in 27 normal subjects, 8 cases of myotonia congenita, 5 of myOTonic dystrophy and one of paramyotonia.
Abstract: The effect of local cooling has been studied in 27 normal subjects, 8 cases of myotonia congenita, 5 of myotonic dystrophy and one of paramyotonia. Using the adductor pollicis we registered the compound muscle action potential, the isometric twitch force and the time to half relaxation, the maximum tetanic force and the time to 3/4 relaxation. 1. In normal subjects the twitch force and maximum tetanic force decreased after cooling (Fig. 2). The amplitude of the action potential increased. 2. Myotonia congenita and myotonic dystrophy were not aggravated by cooling. Muscle force was reduced only in the same proportion as in normal subjects (Fig. 2). The myotonic after-contraction was made normal by cooling (Figs. 5 and 6). 3. In paramyotonia initial tonic stiffness with a pronouncedly prolonged twitch relaxation occured directly after cooling (Fig. 1 B). Paradoxical myotonia occured only after exercise and was accompanied by increasing paresis (Figs. 3 and 8). The results indicate that exposure to cold has a specific effect on muscle function only in paramyotonia.

Journal ArticleDOI
TL;DR: In this article, the regeneration of nerve fibres in pigs was studied three months after transsection and end-to-end suture of the dorsal and ventral spinal nerve roots L 5 and S 1.
Abstract: The regeneration of nerve fibres in pigs was studied three months after transsection and end-to-end suture of the dorsal and ventral spinal nerve roots L 5 and S 1. Although no detailed quantitative analysis was performed, it was clearly evident that regeneration in motor roots is significantly more efficacious than in sensory roots. Probably as a reaction to Wallerian degeneration, an increase of histiocytes, fibroblasts and collagen fibrils, as well as a thickening of the sheaths was observed in the transsected and sutured roots. Heterotopic glial bundles were found in the regenerated dorsal roots.

Journal ArticleDOI
TL;DR: It is concluded that calcification of the pineal is a more general occurrence than has been realized and within the limits found in other biological apatites.
Abstract: A general scheme of analysis for the investigation of minute calcification is presented. After low temperature ashing, the samples are subjected to chemical, structural and morphological study, using atomic absorption spectrometry, infrared spectrometry, X-ray diffraction and scanning electron microscopy. Details about the calcification of the human pineal gland are given. The content of trace elements is very high, but within the limits found in other biological apatites. It is concluded that calcification of the pineal is a more general occurence than has been realized.

Journal ArticleDOI
TL;DR: In this paper, the role of magnesium depression for seizure precipitation is discussed with respect to the concomitant changes of other electrolytes and acid base disturbances, and a significant decrease of potassium and calcium in serum were revealed.
Abstract: 70 chronic alcoholics in the withdrawal state, 45 with convulsions and 25 controls without convulsive seizures, were tested with respect to electrolyte changes and acid base balance in serum or blood and cerebrospinal fluid (CSF). It was of special interest to note that there was a partial independence between magnesium levels in serum and CSF. Thus the serum level has only a limited liability as to magnesium depletion suggested to be responsible for seizure precipitation. In the seizure group a slightly but significantly lower magnesium, potassium and calcium in CSF and a significant decrease of potassium and calcium in serum were revealed. In the nonzeizure controls a similar decrease of magnesium in serum and potassium in CSF was observed while serum potassium and calcium in CSF and serum remained in low normal range. In both groups there was a prominent respiratory alkalosis. The role of magnesium depression for seizure precipitation is discussed with respect to the concomitant changes of other electrolytes and acid base disturbances.