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PubMed1976

Subarachnoid block and enlargement of the spinal canal in hypertrophic neuritis.

A case of Dejerine-Sottas hypertrophic neuritis is reported. The patient, a 45-year-old male, suffered from chronic hypertrophic polyneuropathy, abnormal pupils, fasciculations, tremor, back pain, impotence, sphincter disorders, cramps, and lightning pains in the lower extremities. Besides extensive subarachnoid block, there was X-ray evidence of enlargement of the bony spinal canal with scalloping of the lumbar vertebrae. Surgical exploration showed these abnormalities to be due to extreme hypertrophy of the cauda equina. Histologic findings in peripheral nerve and lumbar root biopsies were typical of hypertrophic neuropathy of the onion bulb type. Vertebral changes secondary to hypertrophied nerve roots appear not to have been described before in hypertrophic neuritis; however, knowledge of their possible occurrence may be of practical importance in the management of similar future cases. A simple way of visualizing enlarged peripheral nerves is briefly described.

Cauda Equina↗
PubMed1979

[Hereditary motor neuron disease: the proximal, adult, sex-linked form (or Kennedy disease). Clinical and neuroendocrinologic observations].

Clinical, electrophysiological, histological and neuroendocrinological findings in a peculiar form of progressive spinal and bulbar muscular atrophy affecting 4 members in two different families are reported. The clinical hallmarks, which characterize this entity among the group of degenerative motor neuron diseases are: sex-linked recessive inheritance; onset in the third decade; slow progression; involvement of facial and bulbar muscles in addition to wasting of the proximal and, in some cases, the distal musculature; asymmetry of clinical signs; consistent and abundant fasciculations predominantly in the peri-oral muscles; intention tremor and a well-developed gynaecomastia, which is the first clinical sign. Electrophysiological and histological findings confirm the neurogenic origin of the muscular atrophy; in addition, muscle biopsy shows "myopathic-like" changes in one case and serum muscle enzyme levels are elevated in all neurologically affected patients. It is believed that the clinical entity corresponds to the rare type III proximal hereditary motor neuropathy (or "Kennedy's disease"), of which 25 published cases are reviewed. Neuroendocrinological data in two patients demonstrate an androgenic insufficiency of hypothalamo-hypophyseal origin and high levels of circulating oestrogens, which probably have induced gynaecomastia. Dynamic neuroendocrinological tests suggest that lesions of certain hypothalamic nuclei may exist, which should be looked for in the forthcoming anatomical studies.

Adult↗
PubMed1984

Neuronal type of Charcot-Marie-Tooth disease with a syndrome of continuous motor unit activity.

The clinical, genetic and electrophysiological study of 3 patients with an association of a neuronal form of Charcot-Marie-Tooth Disease (CMTD) with a syndrome of continuous motor unit activity (CMUA) are reported, with light and electron microscopy of muscle and sural nerve biopsies in 2 patients. The unusual clinical features of CMTD were associated with fasciculation, cramps, myokymia, impaired muscular relaxation and percussion myotonia with their electromyographic (EMG) correspondent, responsive to valproic acid (VPA) therapy. In Case 3, an important muscle hypertrophy which was confirmed by morphometric data, was noted in addition. Nerve biopsy and electrophysiological findings indicated that axonal degeneration with secondary demyelination and remyelination underlie the hereditary motor and sensory neuropathy (HMSN) in our patients. The hyperexcitability and hyperactivity of peripheral motor axons probably induced by the hereditary neuropathy may, in this instance, be the causative condition of the syndrome of CMUA in our patients.

Adolescent↗
PubMed1986

Neurological complications of acute haemorrhagic conjunctivitis.

Forty four cases of the neurological complications of acute haemorrhagic conjunctivitis (AHC) seen in India during 1981 epidemic are reported. The disease predominantly affected adult males. The preceding attack of AHC, a latent period, prodromal symptoms of fever, myalgia and root pains followed by acute onset of lower motor neurone paralysis of limbs and/or cranial nerves formed the classical picture of neurological involvement. The recovery was poor and nearly half of the patients remained severely handicapped. Electrophysiological studies showed early appearance of widespread fibrillations and fasciculations, large polyphasic potentials of increased amplitude and reduced interference pattern. Nerve conduction studies were normal in most of the cases. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis and rise in protein content. Significant antibody titres against enterovirus type 70 (EV 70) were demonstrated in the serum and the CSF. HLA studies showed low occurrence of A2 and B15 HLA antigens. Muscle biopsies revealed neurogenic atrophy and sural nerve biopsies were histologically unremarkable. The similarities of this disease with poliomyelitis and its pathogenesis are discussed.

Adolescent↗
PubMed1988

Adult onset motor neuronopathy in the juvenile type of hexosaminidase A and B deficiency.

Two sisters presented with progressive muscle cramps, as well as wasting and weakness of the legs with onset after age 20. They also showed intention tremor of the upper extremities and dysarthria starting during the first decade. The older patient also had fasciculations; the younger, hyperreflexia. Total plasma beta-hexosaminidase (Hex) activity with 4-methylumbelliferyl-acetyl-glucosamine as substrate was reduced to 1.4% and 2.7% of the control in the 2 patients, respectively. Hex A activity measured by 4-methylumbelliferyl-N-acetylglucosamine-6-O-sulphate as substrate was 9.9% and 12.8% of the mean control value in the 2 patients, respectively. Hex B activity was undetectable in both patients. Leukocyte total Hex activity was 7-8% of normal; residual Hex A activity in the 2 patients was 17.8% and 16.3% of normal controls, respectively. Fibroblastic residual Hex A activity in the 2 patients was 9.6% and 22% of normal mean value, respectively. Appendiceal ganglion cells contained membranous cytoplasmic bodies in the younger patient. Thin layer chromatography of the appendiceal extract from one patient (III/2) showed a marked increase of GM2 ganglioside, and some increase of GM3 ganglioside. Northern blots performed on fibroblast cell lines from both patients for the demonstration of alpha and beta locus messenger RNA showed no difference between patients and control. These patients have a rare form of adult-onset progressive motor neuron disease presumably due to abnormal beta subunits, causing severe deficiency of both Hex A and Hex B. The phenotypic expression of this disease is similar to motor neuron disease due to alpha locus mutations, which suggests that the Hex A deficiency, even though only a partial one, may be the important pathogenic factor.

Adult↗
PubMed1989

Adult-onset autosomal recessive neurogenic scapuloperoneal syndrome.

We report a brother and sister who showed weakness, atrophy and fasciculations starting in adulthood, predominantly affecting the muscles of the shoulder girdles and distal lower extremities. Mild ptosis and facial weakness were present in both. Electrophysiological studies and muscle histology supported a neurogenic basis for the autosomal recessive scapuloperoneal syndrome in this sibship.

Adult↗
PubMed1990

Delayed decreases in specific brain mitochondrial electron transfer complex activities and cytochrome concentrations following anoxia/ischemia.

Hyperglycemic, but not normoglycemic cats exposed to anoxia develop neurologic signs following reoxygenation including fasciculations, focal and tonic-clonic seizures and coma after a symptom-free period. These symptomatic hyperglycemic cats may develop brain edema and will show diffuse neuronal injury or brain infarction depending on length of survival. Brain mitochondria isolated from symptomatic but not asymptomatic cats have decreased ADP- and uncoupler-stimulated oxygen consumption rates. Since impaired respiration could result from altered electron transport chain function, we measured cytochrome c, b, and aa3 concentrations and the activities of the five electron transfer complexes in isolated brain mitochondria. In symptomatic cats marked alterations were present in particular in complex IV, cytochrome oxidase, with a 57% reduction in activity and a 45% reduction in prosthetic group (cytochrome aa3) concentrations. Less marked reductions in other segments of the chain included 27% and 41% decreases, respectively, in cytochrome c concentrations and in electron transfer complex II, succinate:ubiquinone oxidoreductase activity. Cytochrome b concentrations and complex I, II and V activities were unchanged. Small but significant decreases in cytochrome aa3 concentrations (18%) and cytochrome oxidase activity (20%) were also present in mitochondria from postanoxic hyperglycemic cats prior to appearance of neurologic signs. These results indicate that delayed decreases in the activities of specific electron transfer complexes are correlated with impaired mitochondrial respiration and neurologic deterioration in postanoxic hyperglycemic cats. However, it is presently unclear if these postanoxic brain mitochondrial alterations are primary or secondary events in the development of brain injury.

Animals↗
PubMed1992

Pathological changes in skeletal muscle in HTLV-I-associated myelopathy.

The main lesion site of HTLV-I-associated myelopathy or tropical spastic paraparesis (HAM/TSP) is the pyramidal tract. In some HAM patients, clinical symptoms and findings indicate neuromuscular involvement, such as muscular atrophy, fasciculation, elevated serum creatine kinase (CK) or significant electrophysiological data. Cases of HAM/TSP complicated with polymyositis or motor neuron disease have been reported. But no investigation has been directed to muscular pathology in many patients of HAM/TSP. We conducted muscle biopsies on 13 HAM patients. Four patients showed neurogenic changes. Six patients showed histological findings indicative of inflammatory myopathy. We investigated surface marker of invading cells in these 6 patients. In all patients, T lymphocytes were more predominant than B lymphocytes and in three of them T helper/inducer cells were more predominant than T suppressor cells. In 2 patients, only slight myopathic change could be seen, such as variation in fiber diameter and increase in the number of internal nuclei. In 1 patient, type 2 fiber atrophy was seen, and was possibly the result of disuse. Disturbance of secondary motor neurons or inflammatory myopathy is thus shown to be possibly associated with HAM/TSP.

Adult↗
PubMed1993

Dominant inherited distal spinal muscular atrophy with atrophic and hypertrophic calves.

The clinical, electrophysiological, radiological and morphological data of 3 members of a family with autosomal dominant distal spinal muscular atrophy (DSMA) are reported. One patient has the clinical picture of peroneal muscular atrophy with atrophic calves. His father and sister suffer from cramps and fasciculations of the calves with true neurogenic muscular hypertrophy of the calves. The electromyogram and the biopsy specimen are conclusive for motor neuron disease in this family. These findings suggest that the DSMA variant as described by D'Alessandro et al. (Arch. Neurol. (1982) 39: 657-660), concerning benign spinal muscular atrophy with hypertrophy of the calves, has to be considered as a mild manifestation of DSMA.

Adult↗
PubMed1996

Pathological findings in a patient with amyotrophic lateral sclerosis and multifocal motor neuropathy with conduction block.

We studied a 53-year-old woman with progressive weakness of the left arm, gradually spreading to the other limbs. Neurological examination revealed a motor neuron syndrome with paresis, fasciculations and atrophy. Electrophysiological studies showed multiple motor conduction blocks. The anti-GM1 IgM titer was elevated. The patient was thought to have a multifocal motor neuropathy. Despite intravenous cyclophosphamide treatment, however, she died with respiratory insufficiency. On postmortem examination, the brachial plexus showed patches of demyelination underlying different areas of motor conduction block. The spinal cord, however, revealed severe neuronal loss in the ventral horn and axonal loss in the corticospinal tract, indicative of amyotrophic lateral sclerosis. Demyelination of peripheral nerves could have been responsible for the other conduction blocks in this patient. The prominent degeneration of motor neurons, however, must also have played a role in the clinical picture. Some patients with the syndrome of a multifocal motor neuropathy may have MND rather than, or in addition to, a demyelinating peripheral motor neuropathy.

Action Potentials↗
PubMed1983

Convulsant activity of pyridoxal sulphate and phosphonoethyl pyridoxal: antagonism by GABA and its synthetic analogues.

Pyridoxal phosphate and its synthetic analogues--pyridoxal 5'-sulphate and the 5-phosphonoethyl analogue of pyridoxal (phosphonoethyl pyridoxal) in doses of 0.125-0.250 (mumol/10 microliters/i.c.v./rat), caused epileptic seizures characterized by running fits, vocalization, muscular fasciculation and tonic-clonic convulsions. These effects were specific and could not be demonstrated with 5'-deoxypyridoxal, N-methylpyridoxal phosphate or the 5-trans-carboxyethenyl analogue of pyridoxal phosphate (carboxyethenyl pyridoxal). Structure-activity relationships of these analogues indicated that the presence of a CHO in position 4 of the pyridine ring was essential, since its conversion to CH2NH2 or CH2OH abolished activity. The presence of an unsubstituted N was essential, since convulsions did not occur with N-methylpyridoxal phosphate. The presence of the hydroxyl group in position 5' was essential since 5'-deoxypyridoxal was inactive. The convulsive activity was potentiated in the presence of both CHO and PO4, CHO and CH2--CH2PO2-4 but especially CHO and --OSO23-- groups. This seizure activity was prevented, attenuated or reversed by intracerebroventricular administration of 20 microliter of GABA (1 mumol), muscimol (0.025 mumol), trans-4-aminocrotonic acid (0.25 mumol), isoguvacine (0.25 mumol) or THIP (0.25 mumol), but not by biogenic amines. An understanding of the mechanism of pyridoxal phosphate-related seizures may provide additional insights not only about GABA receptor sites but also about the biochemical manifestation and expression of epilepsy.

4-Aminobutyrate Transaminase↗
PubMed1980

Spindle cell carcinoma of the oral cavity. A clinicopathologic assessment of fifty-nine cases.

The clinical features, histomorphologic characteristics, therapeutic results, and follow-up data for fifty-nine cases of spindle cell carcinoma of the oral cavity were analyzed. Circumstantial evidence favors a pathogenesis from metaplastic malignant epithelial cells for the spindle cell proliferation. These neoplasms occurred most frequently on the lower lip, tongue, and alveolar ridge in either polypoid, exophytic, or endophytic configurations. A male predominance was attributed to a military and veteran bias. Mean age at occurrence was 51 years for men and 67 years for women. Symptoms were infrequently reported. Histomorphologically, fasciculated, myxomatous, and streaming patterns of the spindle cells were observed, with variable mitotic activity, pleomorphism, benign and atypical giant cells, inflammation, vascularity, and infiltration. Follow-up data on forty-five patients revealed that only fourteen were alive and well, whereas twenty-five were dead of their disease. Mean survival time for those dead of the disease was under 2 years. Five-year survival was a significant indicator of cure. No clinical or histomorphologic characteristic other than distant metastasis was found to be a reliable prognostic indicator. Wide surgical excision, alone or with radical neck dissection, was the most successful therapeutic modality. Radiation was ineffective.

Adult↗
PubMed1990

Progressive bulbar palsy: a case report of a type of motor neuron disease presenting with oral symptoms.

A case is presented of a patient with progressive bulbar palsy, a form of motor neuron disease, which is a progressive degenerative disorder of the motor nuclei in the medulla producing atrophy and fasciculations of the musculature of the tongue, dysarthria, dysphagia, and excessive accumulation of secretions. The patient may initially seek dental consultation. Clinicians should be aware of the signs and symptoms of this inexorably fatal disease so that an early neurologic referral is made and the appropriate symptomatic therapies instituted.

Aged↗
PubMed1995

Prophylactic efficacy of phenytoin, acetazolamide and hydrochlorothiazide in horses with hyperkalaemic periodic paralysis.

Horses with hyperkalaemic periodic paralysis were challenged with an oral dose of potassium chloride, and the prophylactic efficacy of phenytoin, acetazolamide and hydrochlorothiazide was evaluated, with at least three weeks separating the trials of each drug. After the administration of potassium chloride without prophylactic medication the horses' clinical signs ranged from generalised fine muscle fasciculations to gross tremors, and weakness with occassional prolapse of the nictitating membrane; plasma potassium concentration increased significantly (P < 0.01) from 4.0 +/- 0.2 to 6.0 +/- 1.01 mmol litre-1. After treatment with acetazolamide the administration of potassium chloride resulted in a significant (P < 0.02) increase in plasma potassium from 3.7 +/- 0.3 to 4.5 +/- 0.4 mmol litre-1 and two of five horses showed clinical signs unless the dosage was increased from 2.2 to 4.4 mg kg-1 twice daily. Three of the four horses treated with hydrochlorothiazide showed clinical signs but their plasma potassium did not rise significantly (3.6 +/- 0.3 to 4.6 +/- 1.0 mmol litre-1). None of the five horses treated with phenytoin showed clinical signs despite a significant increase in plasma potassium from 3.8 +/- 0.6 to 5.3 +/- 1.1 mmol litre-1 (P < 0.05). In general the clinical signs were not correlated consistently with the plasma levels of potassium, and phenytoin appeared to prevent the clinical signs in spite of the hyperkalaemia.

Acetazolamide↗
PubMed1981

Fine structural study of the abdominal muscle receptor organs of the crayfish (Procambarus clarkii). Fast and slow receptor muscles.

Receptor muscles of the abdominal muscle receptor organs of the crayfish, Procambarus clarkii, were examined by electron microscopy. Both the fast and the slow receptor strand comprises a single muscle fibre which is divided by invagination of the cell membrane into numerous cytoplasmic processes in its intermediate region (the so-called intercalated tendon). Most of these myofibrillar processes insert in this region, but some of them pass through the intermediate region without interruption and join the other portion of the fibre. Thus the receptor muscles, whilst maintaining cytoplasmic continuity throughout their whole length, are modified in their intermediate regions, becoming fasciculated and providing spaces which are occupied by the connective tissue and the dendrites of the sensory neurone. Clear-cut differences in fine structure are shown between the muscle of the two types of receptor unit. The fast receptor muscle shows the typical features of arthropod fast muscles, including short sarcomere length (on average 3.3 micron), cylindrical myofibrils, well-developed sarcoplasmic reticulum, and regular hexagonal array of the myofilaments. By contrast, the slow receptor muscle fibre is characterized by long sarcomeres (average 6.5 micron) and unique organization of the myofilaments, with very thick 'thick' filaments having diameters in the range of 25-36 nm surrounded by about 12 thin filaments.

Animals↗
PubMed1988

The role of endogenous heparan sulfate proteoglycan in adhesion and neurite outgrowth from dorsal root ganglia.

Some phases of dorsal root ganglion (DRG) substratum attachment and growth cone morphology are mediated through endogenous cell surface heparan sulfate proteoglycan. The adhesive behavior of intact embryonic chicken DRG (spinal sensory ganglia) is examined on substrata coated with fibronectin, fibronectin treated with antibody to the cell-binding site (anti-CBS), and the heparan sulfate-binding protein platelet factor four. DRG attach to fibronectin, anti-CBS-treated fibronectin, and platelet factor four. The ganglia extend an extensive halo of unfasciculated neurites on fibronectin and produce fasciculated neurite outgrowth on platelet factor four and anti-CBS antibody-treated FN. Treatment with heparinase, but not chondroitinase, abolishes adhesion to fibronectin and platelet factor four. Growth cones of DRG on fibronectin have well-spread lamellae and microspikes. On platelet factor four, and anti-CBS-treated FN, growth cones exhibit microspikes only. Isolated Schwann cells adhere equally well to fibronectin and platelet factor four, spreading more rapidly on fibronectin. Isolated DRG neurons adhere equally well on both substrata, but only 10% of the neurons extend long neurites on platelet factor four. The majority of the isolated neurons on platelet factor four exhibit persistent microspike production resembling that of the early stages of normal neurite extension. Endogenous heparan sulfate proteoglycan supports the adhesion of whole DRG, isolated DRG neurons, and Schwann cells, as well as extensive microspike activity by DRG neurons, one important part of growth cone activity.

Animals↗
PubMed1987

Differences in central and peripheral neural actions between soman and diisopropyl fluorophosphate, organophosphorus inhibitors of acetylcholinesterase.

Toxic doses of acetylcholinesterase (AChE) inhibitors produce prominent motor symptoms (fasciculations, fibrillations, and body tremors) and muscle fiber necrosis. The severity and quality of motor symptoms and fiber necrosis depend upon the specific AChE inhibitor. To examine the importance of nerve and muscle activity in producing muscle necrosis, we recorded electromyographic activity from normal and acutely denervated rat gastrocnemius muscle following administration of the organophosphorus AChE inhibitors soman and diisopropyl fluorophosphate (DFP). The motor symptoms induced on the denervated side represented activity originating at the nerve terminal while those of the contralateral nondenervated muscle represented the sum of peripheral plus central descending activity. The results indicate that soman and DFP produce different responses. At nonlethal toxic doses, the majority of motor symptoms induced by soman is due to impulses descending from the central nervous system, and a proportion of these symptoms are epileptiform activity. This activity is not generated at spinal levels. In contrast, DFP produces motor symptoms mainly by peripheral action that is dependent on a functioning nerve terminal. At lethal doses, both agents have central and peripheral effects. Different patterns of electrical activity are associated with each of the motor symptoms. We found that muscle fiber necrosis correlates best with peripherally generated high-frequency repetitive discharges.

Acetylcholinesterase↗
PubMed1987

Interaction of cycloheximide and diisopropylphosphorofluoridate (DFP) during subchronic administration in rat.

Male Sprague-Dawley rats daily treated with DFP (0.5 mg/kg/day, sc) exhibited signs of cholinergic toxicity such as tremors and muscle fasciculations between Days 3 and 5 comparable to those observed 15 min after a single acute signs-producing dose (1.5 mg/kg, sc). Further administration of DFP (0.5 mg/kg/day, sc) for 6-14 days led to tolerance development as evidenced by disappearance of the described toxicity signs. The protein synthesis inhibitor cycloheximide, when given in a nontoxic dose (0.5 mg/kg/day, sc) 1 hr before DFP (0.5 mg/kg/day, sc) administration, potentiated the DFP toxicity and rats died after the fifth injection. DFP-tolerant rats developed toxicity signs when subsequently treated with cycloheximide (0.5 mg/kg/day, sc) and DFP (0.5 mg/kg/day, sc). Each drug when given alone for 4 days caused 30-50% reduction of [14C]valine uptake in vivo into the free amino acids pool as well as its incorporation into proteins of brain and skeletal muscles. A combination of these drugs caused a significantly greater inhibitory effect on [14C]valine incorporation into proteins. Cycloheximide (0.5 mg/kg/day, sc) administered for 4 days did not significantly alter the levels of acetylcholinesterase (AChE), butyrylcholinesterase (BuChE), or carboxylesterase (CarbE) activities but potentiated the DFP-induced inhibition of the activities of these enzymes. It is concluded that the cycloheximide pretreatment potentiates DFP toxicity by a mechanism that is related to inhibition of the synthesis of proteins such as AChE, BuChE, and CarbE.

Acetylcholinesterase↗