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PubMedDate not supplied

Aberrant myelinated neurites in the anterior horns of a patient with amyotrophic lateral sclerosis.

A case of amyotrophic lateral sclerosis revealed the classical pathologic features of ALS, i.e. neuronal loss in the anterior horns and pyramidal tract degeneration. In addition to the pathological hallmarks of Alzheimer's disease, senile plaques, neurofibrillary tangles and granulovacuolar changes of neurons were also present. Clinically the 67-year-old patient exhibited ALS of the upper extremity and a mild forgetfulness. Two striking pathological features were noted in the spinal cord: first, at several levels in the spinal cord excessive amounts of myelinated neurites were found; second, the microglial proliferation in the anterior horns was very intense and positive for leukocyte common antigen.

Aged↗
PubMed1989

[A case report of quadriparesis due to compression of the medulla oblongata by the elongated left vertebral artery].

A patient, 30-year-old man, was admitted to Shinshu University Hospital with gait disturbance and numbness of both arms and legs in June 1985. General physical findings were normal except for facial asymmetry, i.e. mild right facial hemiatrophy. Blood pressure was normal and there were no signs of arteriosclerosis. Neurological examination revealed marked bilateral pyramidal tract signs, muscular weakness of all extremities predominant of the right side, spastic gait and sensory disturbance with impairment of pin prick and light touch sensation in the second and third trigeminal nerve regions and extremities of the left side, and mild loss of vibratory sense and numbness in all extremities. X-ray examination of the cervical spine and the myelogram of the spinal canal were normal. CT scan of the brain performed 3 hours after the myelography showed that the left vertebral artery extended from the left side of the medulla oblongata to the midline and distorted it by compression. MRI confirmed the findings of the CT scan. Vertebral angiography disclosed that the elongated left vertebral artery crossed the ventral aspect of the medulla oblongata. Compression of the medulla oblongata by the vertebral artery was diagnosed and neurovascular decompression was performed. About one month after the operation his spastic gait and muscular weakness were slightly improved, but these symptoms progressed thereafter. One year after operation his spastic gait was more marked than preoperatively. In many cases with dolichoectatic intracranial arteries, hypertension and arteriosclerosis have been reported to be usually associated with this condition. In other opinions dolichoectasia has been suggested to be a congenital disease, because such vessels are occasionally present in children.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

[An autopsy case of HTLV-I associated myelopathy (HAM)].

An autopsy case of HTLV-I associated myelopathy (HAM) was reported. The patient was a 55-year-old man from Kagoshima, who had no history of blood transfusion. He was admitted to our hospital because of muscle weakness of legs and dysuria, which having since one month ago. On admission, he was able to walk with assistance, but his legs were severely spastic, and Babinski's sign was positive bilaterally. Superficial sensation was normal, but vibration sense was mildly decreased in his legs. CSF showed mild mononuclear pleocytosis with elevated protein. Myelogram and CT were normal. Serum and CSF antibodies to HTLV-I were positive at titers of X4,096 and X128, respectively by immunofluorescent assay, and specific IgG bands (p19, p24, p28 and p53 in serum and p19, p24, p53 in CSF) were detected by western blot analysis. His paraparesis continued to worsen. He became bed-ridden within 2 months. He was received corticosteroid medication. He regained the ability to walk with assistance, and continued taking corticosteroid. In July 4, 1986, macrohematuria appeared and inoperable transitional cell carcinoma of rt. kidney was found by further examination. Chemotherapy were not effective against the carcinoma and he died on July 21, 1987. Neuropathological findings were summarized as follows: cerebral hemisphere was normal except for mild cellular infiltration in the leptomeninges; lesions consisted in unilateral pyramidal tract of pons & medulla and in partial anterior, posterior and lateral columns of the spinal cord; demyelination with axonal degeneration, marked gliosis, numerous lipid-laden macrophages and mild perivascular infiltration of mononuclear cells in these areas.(ABSTRACT TRUNCATED AT 250 WORDS)

HTLV-I Antibodies↗
PubMed1989

[A case of aseptic meningoencephalitis with recurrent abnormal behavior, status epilepticus and aphthous stomatitis, improved by acyclovir].

A case of aseptic meningoencephalitis with recurrent abnormal behavior, status epilepticus and aphthous stomatitis was described. A 39-year-old man, who had a history of low grade fever and abnormal behavior over 2 years without mucocutaneo-ocular symptoms, was admitted to Shinshu University Hospital because of status epilepticus. On admission, neurological examination revealed comatose state with neck stiffness, anisocoria (right greater than left), conjugate deviation to right and bilateral pyramidal tract signs. His cerebrospinal fluid examination revealed a total protein content of 206 mg/dl with 22 lymphocytes and 134 polymorphonuclear leukocytes/mm3. EEG recorded on the 3rd hospital day showed periodic sharp waves and polyspikes, occurring every 1 seconds, predominantly arise from the right hemisphere, which corresponds to periodic lateralized epileptiform discharges (PLEDs). Herpes simplex encephalitis was suspected and acyclovir was administered. Neck stiffness and cerebrospinal fluid findings were improved and PLEDs disappeared. But aphthous stomatitis appeared and meningoencephalitis was relapsed. Laboratory examination revealed erythrocyte sedimentation rate 40 mm/hr, positive C-reactive protein, elevated CH50 and sialic acid, enhanced migration of neutrophils and HLA-B 51. Neuro-Behçet's syndrome was strongly suspected with laboratory data but the clinical course was atypical. His clinical signs were markedly improved after administration of prednisolone. The present case suggests that neuro-Behçet's syndrome with only a part of major symptoms could be diagnosed as viral encephalitis and acyclovir may be effective for the treatment of neuro-Behçet's syndrome.

Acyclovir↗
PubMed1989

[Course and prognosis of multiple sclerosis. Report of 90 cases].

90 cases of multiple scleroses were studied in connexion with the course and prognosis of the disease. The course of this disease may be classified into 4 type: benign, remitting progressive, and chronic progressive types. Relapse took place in 75% of the patients. The frequency of relapse was on the average 0.45 time per year. Severe disability occurred mostly during the first five years. The present study suggested that the factors indicating relatively poor prognosis would be the onset at the age over 40 years, chronic insidious onset, early severe impairment of the pyramidal tract function and multiple involvement of the different systems in the CNS.

Adolescent↗
PubMed1988

[Diagnosis by recombinant DNA techniques and clinical features of familial amyloid polyneuropathy].

Serum prealbumin variant with a substitution of methionine (Met) for valine (Val) at position 30 is known to be related to Andrade type familial amyloid polyneuropathy (FAP). Recently, the diagnosis of FAP by recombinant DNA techniques has been established. The DNA diagnosis is based on the fact that a nucleotide substitution responsible for Val-Met change results in formation of new restriction sites for Nsi 1 and Bal 1. We conducted the DNA diagnosis studies of 30 constituents of FAP pedigrees originated from Nagano and Hiroshima Prefectures, and compared the results with clinical features. Clinical features of the patients originated from Ogawa Village area in Nagano Prefecture and from Hiroshima Prefecture showed Andrade type FAP such as polyneuropathy and autonomic nervous disorders. Those of the patients from Iiyama City in Nagano Prefecture showed central nervous involvement such as cerebellar ataxia and/or pyramidal tract signs in addition to the clinical features of Andrade type FAP (Iiyama type). DNA prepared from white blood cells was digested with restriction endonuclease Nsi 1 or Bal 1 and subjected to Southern blot hybridization. The resulting DNA segments were fractionated by agarose gel electrophoresis. The gel was alkalized to convert the double-strand DNA to a single-strand form, which then was absorbed on a nylon membrane filter. The prealbumin cDNA was labelled with 32P as a probe. The probe was hybridized with DNA segments on a filter. The filter was placed on the X-ray film to obtain the autoradiogram.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1988

[Progressive bulbopontine paralysis in children].

A case of pons-bulbar palsy in a 10 year-old girl is reported. The authors insist on the fact that motor cranial nerve nuclear dysfunction was isolated, without any pyramidal tract disorder. They also emphasize the slow course of the disease, which after one year of quick progression was stable for the next 5 years. Nineteen identical cases were found in the literature. From this nosographic group, one must exclude patients with associated lateral spinal tract dysfunction: these patients must be discussed with the juvenile or infantile amyotrophic lateral sclerosis group. The heterogeneity of the pons-bulbar paralysis group is also underlined: the course may be quickly fatal or, on the contrary, protracted for a long time; genetic transmission is also quite variable.

Bulbar Palsy, Progressive↗
PubMed1988

Recovery of motor function after severe traumatic coma.

This paper describes the resumption of motor activity during the successive stages of recovery following severe traumatic coma and discusses the likely mechanisms of such resumption. Even in severe brain injury a considerable amount of motor recovery is possible and leads to a normal level of function in half of the cases. The impairment of voluntary movements is often transitory and recovers spontaneously but in cases of persistent deficits probably from direct lesion of the pyramidal tract re-education efforts are relatively inefficient. The disorders of reflex and semi-automatic motor activity subserving tonic adjustments, gait and locomotion are nearly constant and often severe. The recovery of this basal function is the most important to attain and to secure and this can be achieved through extensive and prolonged retraining of reflex activity. Presumably these disorders are not caused by direct lesions of brainstem structures but rather to a functional denervation akin to spinal shock. A resumption of function in the denervated groups of neurons should necessitate the reopening of silent synapses and/or a structural reorganisation of synaptic connections, this dynamic process being highly influenced by retraining.

Coma↗
PubMedDate not supplied

[Effect of strychnine on the neuronal reactivity of the sensorimotor cortex and the temporary connection in rabbits].

On awake nonimmobilized rabbits, evoked activity was studied of the sensorimotor cortex neurons in response to stimulation of the pyramidal tract, medial lemniscus and reticular nucleus of the midbrain tegmentum by stimuli of different frequencies, and driving reaction of cortical neurons to stimulation of these brain structures by series of stimuli of increasing frequency. Conditioned reflexes were also studied, established on the basis of combination of direct stimulation of the sensorimotor cortex and electrocutaneous stimulation. Application of the cortex of low concentration of strychnine solutions (less than 1%) heightened neurons reactivity and provides for the formation of temporary connection. Application of strychnine solutions of higher concentration (greater than 1%) led to opposite effects. Interconnection of electrical and behavioural effects is discussed.

Animals↗
PubMed1988

[Peripheral neuropathies simulating amyotrophic lateral sclerosis in gammopathies].

Particular clinical pictures of ALS may occur during the course of some "benign gammapathies". We observed 12 patients (age range 57 to 74 years; 9 men-3 women) with initially benign gammapathy (9 IgG, 1 IgA, 2 IgM) associated to a clinical picture of progressive anterior horn and pyramidal tract involvement. These cases led us to recognize some particularities of gammapathy-associated ALS: Relative frequency of asymmetrical clinical manifestations, rarity of bulbar signs; Decrease of sensory nerve conduction velocities without evidence of sensory clinical symptoms (8 out 9); Increase CSF protein content including the monoclonal component, axonal degeneration and immunostaining evidence of the paraprotein fixation observed on nerve biopsies (5 out 7 cases). Association of ALS and gammapathy is not fortuitous as shown by epidemiology, experiments, pathology and effects of different immunological treatments as related in this study. Demonstration of infra-clinical neuropathy face to a clinical syndrome of ALS should prompt too careful screening for a gammapathy.

Aged↗
PubMed1987

[Primary intracranial epidermoid carcinoma accompanied with epidermoid cyst in the cerebellopontine angle--a case report].

A case of epidermoid carcinoma arising in an epidermoid cyst in the cerebellopontine angle is presented. Clinical features and CT appearance are discussed in comparison with those of benign epidermoid cyst. A 43-year-old man was admitted to the Department of Neurosurgery, University of Tokyo Hospital on April 14, 1983, with complaints of right facial numbness and weakness of six months' history. On neurological examination, sensation of the right half of the face was decreased in all modalities. Marked atrophy of the ipsilateral temporal muscle was also noted. Right facial paresis of peripheral type was evident. Gag reflex was decreased on the same side. Except for a slightly increased left deep tendon reflexes, there were no pyramidal tract signs. A CT without contrast material failed to show any abnormalities. A postcontrast CT demonstrated an irregular enhancement in the right cerebellopontine angle. The finding of asymmetry of the ambient cistern indicated minimum mass effect on the metrizamide CT cisternography. Suboccipital exploration of the right cerebellopontine angle was carried out on April 28, 1983. Leaving a part of the capsule indenting the pons between the roots of the fifth and the seventh nerve, we removed a white pearly tumor. Histological diagnosis was typical epidermoid cyst. He left the hospital one month later with signs of the right seventh and the eighth nerve. His postoperative course, however, was beyond our expectation. Over a few months following his discharge, left hemiparesis as well as horizontal and vertical nystagmus gradually developed. He was readmitted on November 10, 1983. A postcontrast CT revealed enlargement of the enhanced lesion filling the right ambient cistern.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1988

Neurologic complications of thyrotoxicosis: case report.

Weakness accompanying hyperthyroidism may be due to a variety of causes. A case is presented of a patient who, during management of thyrotoxicosis, became quadriparetic due to a unique combination of axonal neuropathy, myopathy, and pyramidal tract dysfunction. Electrodiagnostic, muscle biopsy, and nerve biopsy results are presented.

Adult↗
PubMed1988

[Midbrain arteriovenous malformation causing bilateral total ophthalmoplegia as an initial ocular symptom--a case report].

A 61-year-old woman is presented with a bilateral total ophthalmoplegia as an initial ocular symptom, caused by a midbrain hematoma. She complained of acute headache, nausea, vomiting and bilateral closure of her eyelids. Examination on admission showed meningeal irritation; mild consciousness disturbance; bilateral total ophthalmoplegia; left hemiparesis; ataxia in all extremities, more marked to the left. Computed tomography demonstrated a small hematoma in the midbrain tegmentum. Angiography demonstrated midbrain arteriovenous malformation, and she was treated conservatively. Abduction of both eyes and adduction of the left eye appeared on the next day of the ictus, and after that, improved gradually. Left ptosis had improved since one week after the ictus. Light reflex of the left pupil had seen 5 days after the ictus. At the same time, the left pupil revealed an oval-shape. Right internal ophthalmoplegia continued to exist. One year later, the right eye deviated externally, and the left deviated inferio-medially. Abduction of both eyes was normal. Infraduction of both eyes was seen, but limited on the left. Upgaze paresis remained unchanged, and adduction of the right eye was absent. Adduction of the left eye showed almost full recovery. There was right complete ptosis, but left ptosis became indefinite. These ocular findings indicated typical right oculomotor paresis plus superior rectus paresis of the left eye, which suggested a destructive lesion in the right oculomotor nucleus. Also, her left extremities showed a hemiparesis as a pyramidal tract sign (Weber's syndrome) and an ataxia as a cerebellar sign (Claude's syndrome).(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebellar Ataxia↗
PubMed1977

[New stereotactic treatment of spasmodic torticollis with a brain stimulation system (author's transl)].

The author reports here on a new method for treating spasmodic torticollis with the permanent implantation of a brain stimulation system (Medtronic) in the extrapyramidal-motor thalamic nuclei (V.o.a./V.o.i.) and the subthalamic zona incerta including the pyramidal tracts H1 and H2 (according to Forel). Thanks to stimulation which lasts 30--40 minutes and is determined and undertaken by the seven patients themselves, control over the position and the morbility of the head is attained while simultaneously suppressing the symptoms of spasmodic torticollis. This new stereotactic and functional method of neurosurgical treatment for functional disturbances of the motor systems is not destructive and entails neither operative complications nor side-effects.

Aged↗
PubMed1988

Monitoring of motor action potentials after stimulation of the spinal cord.

We recorded motor action potentials in cats, using surface electrodes placed over the soleus muscle. The action potentials were generated by stimulating the spinal cord with electrodes in the epidural space at the level of the fifth or sixth thoracic vertebra. This also was done in humans, using the same methods of stimulating and recording, but the intensity of the stimulus was adjusted to produce little or no twitch of the paraspinal muscles. In the animal experiment, the motor action potential was abolished after transection of the pyramidal tract and was progressively attenuated with effective doses of a curare-like agent. We also tested the effect of distraction, using the same technique as is used in Harrington instrumentation, and found that the amount of distraction that caused reduction of the amplitude of the motor action potential of more than 50 per cent, when sustained for longer than seven minutes, caused permanent paraplegia in two cats. The evaluation of spinal evoked potentials that were obtained from epidural electrodes placed caudad to the level of distraction, and of motor action potentials that were recorded over the soleus muscle, following the same stimulus, showed a similar pattern of reduction after distraction in five of seven cats. The other two cats had irreversible reduction of motor action potential associated with unchanged spinal evoked potential, and both cats became paraplegic.(ABSTRACT TRUNCATED AT 250 WORDS)

Action Potentials↗
PubMed1987

[Chondromyxoid fibroma of the skull base extending from the middle fossa to the posterior fossa--case report].

An extremely rare case of chondromyxoid fibroma of the skull base extending from middle fossa to posterior fossa is reported. The patient is a forty-seven year old woman who became paranoiac four months before admission and her gait was unsteady for one month. On April 27th, 1985, she fainted and was admitted to Kanto Rosai Hospital on April 30th. She was drowsy and disoriented. Neurologic examination revealed left pyramidal tract signs and impaired functions of right Vth (first and second branches), VIth and VIIIth cranial nerves. She was anemic and bleeding tendency due to thrombocytopenia was also recognized. Hematologist's diagnoses were myelodysplastic syndrome plus iron deficiency anemia. Neuroradiologic studies including CT scan, cerebral angiographies and MRI revealed a huge extra-axial skull base tumor extending from middle fossa to posterior fossa completely destroying the skull base. Stenosis of cavernous portion of internal carotid artery due to tumoral involvement was also noted. Surgical excision of the tumor was performed three weeks after the admission with the use of platelet transfusion. Transpetrosal transtentorial approach was performed removing the intracranial part of the tumor which was a semi-translucent jelly-like soft mass. The patient had a good post-operative course and improvement in the neurologic condition was recognized. Microscopically, the tumor showed marked lobular formation with small vessels and fibroblasts in the periphery of the lobule. The fibroblasts took stellate figures in the myxoid matrix at the center of the lobule. Chondroid pattern was also observed. Mitosis and nuclear atypism were not observed. Histological diagnosis was chondromyxoid fibroma.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral Angiography↗
PubMedDate not supplied

[Effect of GABA on a temporary connection in a neuronal population of the sensorimotor cortex in rabbits].

Evoked activity of sensorimotor cortical neurones in response to stimulation of the pyramidal tract, medial lemniscus and reticular nucleus of the midbrain tegmentum; driving reaction of cortical neurones at stimulation of these brain structures of growing frequency, and conditioned reflexes elaborated by combination of direct stimulation of the sensorimotor cortex and electrocutaneous stimulation were studied in awake nonimmobilized rabbits. Application to the cortex of GABA solutions of low concentration (less than or equal to 1%) emphasizes the evoked neuronal responses, facilitates the appearance of driving reaction and contributes to the manifestation of the temporary connection. Application of GABA solutions of higher concentration (greater than 2%) leads to opposite effects. Positive correlation is found between electrical and behavioural phenomena. The described experimental approach may be used for analysis of various types of influences on temporary connection formation.

Action Potentials↗
PubMed1987

Phorbol esters that activate protein kinase C induce long-term changes of membrane excitability and postsynaptic currents in neocortical neurons.

Intracellularly injected tumor promoter phorbol esters (PhEs) that activate protein kinase C (PKC) increased the excitability and altered the postsynaptic responses of neurons of the motor cortex of awake cats. PhEs increased the amplitude and duration of EPSPs and decreased the amplitude and durations of IPSPs. No consistent changes in resting membrane parameters that would account for these modifications were found. Corresponding changes in peak excitatory and inhibitory postsynaptic currents (EPSCs, IPSCs) were measured directly with the single electrode voltage clamp technique. The changes lasted for 50 min or longer. Quantitative analysis of EPSCs in response to ventrolateral thalamic stimulation and IPSCs in response to pyramidal tract stimulation made in a subgroup of fast PT cells suggested that PhE acted within the injected neuron rather than presynaptically to alter the synaptic currents. PhE also reduced a voltage-dependent, 3-aminopyridine sensitive fast outward current (IA) and an apamin and EGTA sensitive slow outward current (IK(Ca]. Control injections of a phorbol ester that did not activate PKC failed to induce changes in synaptic responses or resting membrane properties. These observations provide the first evidence that activation of PKC, in vivo, can induce long-lasting changes in synaptic responses of neocortical neurons by direct modification of postsynaptic ion channel conductivities.

Action Potentials↗

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