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Biomedical subjects

T Muroga

Publications and source records attributed to T Muroga.

At least 19 recordsLinked to original sources

[A case of Down's syndrome with chronic inflammatory demyelinating polyradiculoneuropathy].

A 19-year-old male patient with Down's syndrome accompanied by relapsing and steroid-dependent chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) was reported. He had been well until the beginning of June 1988, and he rapidly developed a gait disturbance and symmetrical numbness in his arms and legs at the end of that month. He also suffered from difficulty in swallowing and dyspnea soon afterward. A physical examination revealed the specific clinical features of Down's syndrome, but no particular signs. Neurological examination showed the following abnormal findings; fixed nystagmus, diminished gag reflex, flaccid tetraparesis, and distal dominant dysesthesia of upper and lower extremities. Deep tendon reflexes were diminished and Babinski's sign was negative. No autonomic dysfunction was detected. Routine hematology and biochemistry screening test revealed normal level except for increasing GPT and IgG. Protein and gamma globulin values in cerebrospinal fluid (CSF) were elevated without pleocytosis. Nerve conduction velocities were slighty reduced. Pulse therapy and corticosteroid treatment improved on clinical course and CSF results. CIDP probably results from an abnormal immune responsiveness. Down's syndrome has often been taken for autoimmune abnormality. It is of great interest whether there is a close connection of autoimmune abnormality between Down's syndrome and CIDP. This might be the first case report of Down's syndrome associated with CIDP, judging from our survey of the literature in Japan and other countries.

Adult

[A case of idiopathic hypoparathyroidism with extrapyramidal signs and insulin-dependent diabetes mellitus].

A case of a 29-year-old woman with idiopathic hypoparathyroidism was reported. There were neither endocrine nor neurological disorders among her family, except for her mother's hearing loss. She had been suffering from insulin-dependent diabetes mellitus since 21 years of age, and was noticed to be hard of hearing for several years, but never been examined. At the age of 27, choreic movement on her left upper limb and gait disturbance appeared. A year before admission, gait disturbance gradually developed and she could not walk any more. On admission, her height was 137.2 cm and her weight 36.5 kg. She had a round face, uneven teeth and borderline metacarpal sign on her right hand. On neurological examination, Parkinsonism, bucco-lingo-masticatory dyskinesia and bilateral extensor planter reflex were present, but tetany was not observed anywhere. Serum calcium was 3.9 mEq/l, and serum phosphorus 5.3 mEq/l. A CT scan of brain revealed calcifications in the bilateral basal ganglia and thalami, low density area in the left putamen, and atrophy of both caudate nuclei. Serum PTH was less than 100 pg/ml. Ellsworth-Howard's test showed hyperresponsiveness in the secretion of urinary phosphorus and cyclic-AMP. Other endocrinological studies showed no abnormality except for hyporesponsiveness in the secretion of insulin on glucose tolerance test. On the basis of these results, a diagnosis of idiopathic hypoparathyroidism with insulin-dependent diabetes mellitus was made. Administration of alfacalcidol returned serum calcium and phosphorus to normal with considerable clinical benefit. Parkinsonism was gradually improved and she became to be able to walk with a cane after one year of treatment. But buco-lingo-masticatory dyskinesia were not reduced.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult

[A comparative study between amyotrophic lateral sclerosis patients with and without mechanical ventilation].

Fifty-three patients with amyotrophic lateral sclerosis (ALS) were examined as to duration of illness, cause of death and need for respirator assistance. In 47 patients with ALS, respiratory failure is the most common cause of death accounting for 62% of all fatalities. Eleven patients with mechanical ventilation died of pneumonia and respiratory failure in four cases, atelectasis, gastrointestinal bleeding and asphyxia in one. Thirteen of 53 patients with ALS required mechanical ventilation. Six cases were put on a volume-limited respiratory or a pressure-limited respirator with O2 blender, and seven cases were put on a pressure-limited respirator only, which was unable to control oxygen concentration. As to the duration of their illness, patients with mechanical ventilation survived significantly longer than those without mechanical ventilation, so patients using a respirator could expect to live significantly longer than those without one. Patients using a volume limited respirator or a pressure-limited respirator with O2 blender had significantly longer duration of mechanical ventilation than those using a pressure-limited respirator only. It is concluded that a volume-limited respirator or a pressure-limited respirator with O2 blender is useful, if mechanical ventilation is needed, for ALS patients presenting with respiratory failure.

Adult

[A nosological study of a patient showing ataxia & lower motor neuron involvement].

A sporadic case of spinocerebellar degeneration with prominent involvement of the motor system has recently been encountered. A 54-year-old man without family history noticed speech disturbance at the age of 46 and weakness in his right hand the following year. The muscle weakness and atrophy were slowly progressive and made walk impossible at the age of 50, when his dysphagia increased. At the age of 54, he was admitted to our hospital when neurological findings revealed marked amyotrophy of general skeletal muscle and tongue with fasciculation. Deep tendon reflexes were decreased. Cerebellar ataxia was impossible to evaluate because of profound muscle weakness. And sensory disturbance was suspected in the distal portion of the lower extremities. CT scan revealed progressive atrophy of the brain stem and cerebellum. The patient died at the age of 54 due to CO2 narcosis. The clinical course was 8 years. A summary of the pathological findings was as follows: 1) Marked neuronal loss of the anterior horn of the spinal cord and motor cranial nerve nuclei except for oculomotor nuclei, with mild degeneration of pyramidal tract below lumbosacral level. 2) Degeneration of cerebellipetal system, spinocerebellar tract, Clarke's column and the middle root zone and cerebellifugal system, dentate nucleus, superior cerebellar peduncles, and red nucleus. 3) Mild degeneration of pontine nuclei, inferior olivary nuclei, pontine transverse fibers, the middle and inferior cerebellar peduncles, cerebellar white matter and Purkinje cells as in OPCA.(ABSTRACT TRUNCATED AT 250 WORDS)

Amyotrophic Lateral Sclerosis

Degenerating compartment and functioning compartment of motor neurons in ALS: possible process of motor neuron loss.

Using a morphometric method, we studied ventral spinal roots and anterior horn neurons of the fourth lumbar segment in 17 patients with ALS. Both populations of large myelinated fibers and anterior horn cells had significantly high correlations to muscle strength in the legs and duration of symptoms. However, active axonal degeneration was consistently present in terms of either large myelinated fibers or anterior horn cells.

Aged

Effects of thyrotropin releasing hormone on cerebellar mutant mice--a kinesiological comparison between rolling mouse Nagoya, weaver and reeler.

Rolling Mouse Nagoya (RMN), weaver and reeler mice were examined for the clinical effectiveness of drugs (thyrotropin releasing hormone (TRH), reserpine, L-dopa) that are reported to facilitate or alter the metabolic system of noradrenaline (NA). Behavioral changes and ataxic gait were recorded by 16 mm cinefilms and EMG of the thigh extensor muscles. In addition, latencies in the reflex discharge of the muscle by tilting load were measured before and after the intraperitoneal injection of each drug and compared to those of normal littermates. TRH injection increased spontaneous behavioral activity in RMN and weaver, and shortened the latency of the reflex discharge by tilting load in RMN. Reserpine injection did not increase behavioral activity, but was accompanied by a slight shortening of latencies in the reflex discharge by tilting load only in RMN. L-dopa did not affect behavioral and EMG activities of these cerebellar mutant mice. No remarkable deficit has yet been in any selective intracortical neurons in RMN and no other characteristic abnormality in central and peripheral nervous, skeleton and muscular systems. It was, therefore, suspected that there was a possibility of metabolic disorders of NA in the cerebellum not only in RMN, but also in patients with certain spinocerebellar degenerations.

Animals

Tourniquet-induced ischemia and somatosensory evoked potentials.

We studied the effect of tourniquet-induced ischemia on the somatosensory evoked potential (SEP). During 24 minutes of ischemia, short-latency components (P9, P14, and N18) and Erb's potential were abolished earlier than the long-latency components (P22, N30, P40, and N60). The latency increase was the same for Erb's potential and for P9, P14, and N18, but it was greater for P22, N30, and P40 and greatest for N60. These dissociated effects suggest that SEP components are not transmitted through a single pathway but are mediated through independent routes, possibly involving different first-order afferent fibers. Selective abnormalities of the early SEP peaks in association with relative preservation of late peaks may occur in peripheral nerve disorders.

Adult