MRI in hemiballism due to Sydenham's chorea.
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Adolescent↗
Biomedical subjects
M Konagaya
Publications and source records attributed to M Konagaya.
Quadriceps myositis.
A young woman with slowly progressive muscular weakness and atrophy localized in both thighs is reported. Laboratory, electromyographic and histological findings suggested that the patient suffered from chronic myositis with a background of autoimmune disorder. Quadriceps myositis is a rare condition. The previously reported cases of this disease in the literature are reviewed.
Adult↗
[Central nervous system lesion in myotonic dystrophy--demonstrated by X-ray computed tomography and magnetic resonance imaging].
We made an attempt to detect organic brain lesions in eight patients with myotonic dystrophy (MyD) using X-ray computed tomography (CT) and magnetic resonance imaging (MRI). The patients comprised seven men and one woman with ages ranging from 41 to 58 years (average 47 +/- 5.3 years). Seven patients had intellectual impairment assessed using an intelligence quotient test (WAIS-R < 70). CT scans were obtained in the axial plane with a slice thickness of 10 mm, and MRI scans were performed on a 1.5 tesla system in the axial and coronal sections with a slice thickness of 8 mm. CT scans displayed both marked sulcal and prominent ventricular enlargement in 6 of the 8 patients, indicating the presence of brain atrophy, and 3 cases had a few paraventricular low density area in the cerebral hemispheres. In contrast to CT, MRI study revealed more widespread brain parenchymatous lesions in all the 8 cases, showing the multiple foci of high signal intensity with varying size in the white matter on T2-weighted images and proton density-weighted images. No visible changes were detected on CT or MRI in the brain stem and cerebellum. The present study demonstrated that there exist organic cerebral lesions, predominantly located in the white matter in patients with MyD, regardless of focal neurological symptoms. The parenchymatous lesions in the brain can be detected with MRI, and lesser extent with CT, and are considered to be intimately correlated with intellectual deterioration commonly seen in patients with MyD.
Adult↗
[CSF acetylcholinesterase activity in central neurological diseases involving cholinergic systems].
The enzymatic activity of acetylcholinesterase (AchE) in the cerebrospinal fluid (CSF) is considered to be a marker of central cholinergic neuron integrity. Then, we evaluated CSF AchE activity in 90 cases of neurological diseases involving cholinergic system and their related disease, and 28 control cases without central organic lesions or abnormal findings in routine CSF study. AchE activity was evaluated according to Ellman's method using acetylthiocholine iodide as a substrate and tetraisopropyl-pyrophosphoramide, a specific inhibitor of butyrylocholinesterase. CSF AchE of Alzheimer type dementia (AD/SDAT, N = 12: 21.9 +/- 4.7 nmol/ml/min) showed no significant change from those of both control group (22.1 +/- 3.9) and vascular dementia (9: 21.7 +/- 6.7). In extrapyramidal diseases, reduction of the activity was observed in Huntington's chorea (HC, 4: 16.3 +/- 1.4) and progressive supranuclear palsy (PSP, 4: 17.6 +/- 1.7), whereas normal activity was shown in Parkinson's disease (PD, 19: 22.5 +/- 4.6), dentatorubropallidoluysian atrophy (DRPLA, 4: 22.6 +/- 4.2) and striatonigral degeneration (SND, 4: 20.4 +/- 4.3). In olivopontocerebellar atrophy (OPCA, N = 16), we disclosed reduced CSF AchE activity (15.8 +/- 2.4) which had significant correlations with the atrophy of the pontine base (r = 0.6017, p less than 0.02) and cerebellar vermis (r = 0.5450, p less than 0.05) in MRI. AchE activity in cerebellar cortical atrophy (CCA, 5: 20.6 +/- 2.2) remained within the control values. Normal activity was demonstrated in both amyotrophic lateral sclerosis (6: 24.3 +/- 7.3) and spinal muscular atrophy (4: 22.9 +/- 3.9).(ABSTRACT TRUNCATED AT 250 WORDS)
Acetylcholinesterase↗
[Abnormality of hypothalamic dopaminergic system in neuro-degenerative diseases--evaluation of alpha-melanocyte-stimulating hormone-like immunoreactivity in cerebrospinal fluid].
Dopaminergic neuron controls CNS functions such as meso-limbic, striato-nigral and tubero-infundibular systems. The purpose of the present study is the evaluation of the hypothalamic dopaminergic neuron activity in neuro-degenerative disorders. alpha-melanocyte-stimulating hormone (alpha-MSH) is synthesized in the arcuate nucleus and lateral part of the hypothalamus, and its secretion is under the inhibitory control of the dopaminergic neuron both in the hypothalamus and pituitary. alpha-MSH-like-immunoreactivity (alpha-MSH-LI) in CSF is thought to be representative to the dopaminergic neuron activity in the hypothalamus. We therefore evaluated CSF levels of alpha-MSH-LI in spinocerebellar degenerations and extrapyramidal diseases. The subjects are 11 patients with Parkinson's disease, 16 with Shy-Drager syndrome (SDS), 16 with cerebellar cortical atrophy, 3 with Machado-Joseph disease, 3 with dentato-rubro-pallido-luysian atrophy and 2 with Huntington's disease as well as 24 controls. All patients with Parkinson's disease were administered levodopa and carbidopa. CSF was sampled through lumbar puncture in the morning. After the centrifugation, supernatant of CSF was stored at -40 degrees C until used. alpha-MSH in CSF was extracted by Rainero's method and measured by RIA. alpha-MSH-LI levels in control was 23.9 +/- 2.6 pg/ml (mean +/- SD). The significant elevation was observed in Parkinson's disease (40.3 +/- 7.5, p less than 0.001) and SDS (42.3 +/- 9.4, p less than 0.001). The levels showed not significant correlation with age, duration of illness or severity of autonomic disorder. Most of other diseases demonstrated the levels within normal range.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[A case of HTLV-1 associated myelopathy with diffuse white matter lesion of the frontal lobe and continuous lesion of the pyramidal tract on cranial MRI].
In this report, the characteristic findings of cranial MRI of a case with HTLV-1 associated myelopathy (HAM) is described. The patient was a 62-year-old woman with 6 years history of paraplegia. Her main clinical signs were bilateral spastic paraplegia in the lower limb girdle muscles and extremities, paresthesia below the tenth thoracic cord level and urinary disorder. There was bilateral blepharoptosis. Hyperreflexia was observed in the examination of the cranial nerves and upper extremities. She showed no dementia or any other higher cortical dysfunctions. Positive anti-HTLV-1 antibody in the serum and cerebrospinal fluid established a diagnosis of HAM. T2 weighted MRI study revealed the symmetrical diffuse hyperintensity in the subcortical white matter of the frontal lobe and temporal lobe. The hyper-intensity was also observed in the bilateral internal capsule-peduncular base junction and pontine base, which indicated the continuous lesions in the intracranial pyramidal tracts. These MRI findings were different from those of reported cases of scattered lesion in central nervous system. There are some speculations for the nature of the diffuse lesion in the white matter, i.e., fusion of solitary gliosis and/or perivascular cuffing, or diffusely advanced spongy state. The continuous lesion of the pyramidal tract is suspected a systemic demyelination. Although the pathomechanism still remains uncertain, HTLV-1 infection generally affects the spinal pyramidal tract. It seems that this case is selectively affected in the pyramidal tracts in CNS.
Female↗
Abscessed leiomyosarcoma of the ileum.
A-49-year-old man was admitted because of a lower abdominal mass. During the five days prior to admission, he had noted fever and lower abdominal pain. Palpation of the abdomen revealed a tender mass, 10 cm in diameter, in the suprapubic region. An x-ray revealed an irregular collection of gas, 9 cm in diameter, in the pelvic cavity, which appeared as a mirror image when the patient was upright. Based on the physical finding and the results of a barium enema, abdominal CT, MRI, and small intestine imaging, a diagnosis of leiomyosarcoma of the ileum was made. Excision of the tumor and part of the small intestine was performed. A saccular tumor, 11 x 6 x 5 cm, was found 1.0 m from the cecum and growing out of the wall of the ileum. A large amount of pus was found inside the cavity. The pathological diagnosis was leiomyosarcoma.
Abscess↗
Successful transcatheter embolization of pseudoaneurysm associated with pancreatic pseudocyst.
Hemorrhage into a pancreatic pseudocyst is a rare event, but is the most rapidly lethal complication of chronic pancreatitis. Visceral-vessel aneurysms are an unexpectedly common finding in arteriography of patients with chronic pancreatitis. This case report describes bleeding from an anterior superior pancreaticoduodenal artery aneurysm, caused by chronic pancreatitis. The aneurysm was successfully treated by embolization with a steel coil.
Aneurysm↗
Magnetic stimulation study in mirror movements.
A young man with congenital mirror movements was studied by non-invasive magnetic stimulation. Radiological examination showed no craniocervical or pituitary abnormality except for mild atrophy of the right hippocampus. Magnetic stimulation of the motor cortex caused large amplitudes in the ipsilateral hand muscles, indicating the possibility of functional disorder in the motor pathways from the motor cortex to muscles in the upper extremities.
Adult↗
Neural control of glutamine synthetase activity in rat skeletal muscles.
The mechanism of glutamine synthetase induction in rat skeletal muscle after denervation or limb immobilization was investigated. Adult male rats were subjected to midthigh section of the sciatic nerve. At 1, 2, and 5 h and 1, 2, and 7 days after denervation, rats were killed and denervated, and contralateral control soleus and plantaris muscles were excised, weighted, homogenized, and assayed for glutamine synthetase. Glutamine synthetase activity increased approximately twofold 1 h after denervation in both muscles. By 7 days postdenervation enzyme activity had increased to three times the control level in plantaris muscle and to four times the control level in soleus muscle. Increased enzyme activity after nerve section was associated with increased maximum velocity with no change in apparent Michaelis constant. Immunotitration with an antiglutamine synthetase antibody suggested that denervation caused an increase in the number of glutamine synthetase molecules in muscle. However, Northern-blot analysis revealed no increase in the steady-state level of glutamine synthetase mRNA after denervation. A mixing experiment failed to yield evidence for the presence of a soluble factor involved in regulating the activity of glutamine synthetase in denervated muscle. A combination of denervation and dexamethasone injections resulted in additive increases in glutamine synthetase. Thus the mechanism underlying increased glutamine synthetase after denervation appears to be posttranscriptional and is distinct from that of the glucocorticoid-mediated glutamine synthetase induction previously described by us.
Animals↗
[Pentose phosphate pathway in neuromuscular diseases--evaluation of muscular glucose 6-phosphate dehydrogenase activity and RNA content].
There have been several reports concerning elevated glucose 6 phosphate dehydrogenase (G6PDH), the rate-limiting enzyme of pentose phosphate pathway (PPP), in experimental muscle disturbances. PPP produces ribose, a substrate of RNA, and NADPH which is a cofactor of fatty acid synthesis. PPP also has a role of by-path pathway of glycolysis. Then, we evaluated G6PDH activity and RNA content in biopsied quadriceps muscle. The subjects were muscles from 23 neurogenic amyotrophy, 54 myopathy including 19 progressive muscular dystrophy (PMD), and 10 controls whose muscle was obtained at orthopedic surgery. Neurogenic amyotrophy consisted of 12 amyotrophic lateral sclerosis (ALS), 4 spinal muscular atrophy and 7 peripheral nerve disorders. Myopathy were 3 Duchenne dystrophy, 2 congenital muscular dystrophy, 8 limb-girdle type dystrophy, 6 facio-scapular +-humeral muscular dystrophy, 6 myotonic dystrophy, 6 mitochondrial myopathy, 5 endocrinological myopathy, 3 hypokalemic myopathy, 8 polymyositis and 4 other inflammatory myopathy. The assays of G6PDH and RNA were performed after Glock's and Fleck's methods, respectively. The control values were 3.6 +/- 0.8 nmol formed NADPH/mg protein/min (M +/- SD) in G6PDH and 0.69 +/- 0.17 micrograms/mg non-collagen protein in RNA. Most cases of PMD, as well as some cases of ALS, hyperthyroidism, mitochondria hypokalemic myopathy, inflammatory myopathy showed increased values (beyond M + 2SD of control) both in G6PDH and RNA. There were significant positive correlations between G6PDH activity and RNA content in PMD and motor neuron disease. Myotonic dystrophy showed normal values in both G6PDH and RNA. Half number of cases of mitochondrial myopathy demonstrated increased G6PDH alone.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Studies on enzyme activities relating to amino acid mobilization in biopsied muscles].
We evaluated glutamine synthetase (GS) and alanine aminotransferase (GPT) activities in biopsied muscle from 40 cases of various neuromuscular diseases. GS and GPT catalyze the synthesis of glutamine and alanine, respectively, from amino acids derived in part from the breakdown of muscle proteins. The subjects were 7 cases of muscular dystrophy; 1 Duchenne type (DMD), 3 limb-girdle type, 2 facioscapulohumeral type (FSH), 1 Fukuyama type (FCMD); and 1 myotonic dystrophy (MyD); 5 mitochondrial myopathies; 11 inflammatory myopathies including 6 polymyositis and 3 myopathy associated with collagen disease; 5 endocrinological myopathies including 2 periodic paralysis; and, 11 cases of neurogenic amyotrophies [4 amyotrophic lateral sclerosis (ALS), 4 spinal progressive muscular atrophy (SPMA) and 3 other types]. Control subjects were 8 patients with thigh operations. Biopsied muscle was homogenized and assayed for GS activity by the method of Smith et al.; GPT was assayed by commercial kit. Protein was assayed by the method of Lowry et al. Enzyme activities between mean -2SD and mean +2SD of controls were considered to be the normal range. GS activity in control subjects was 28.22 +/- 7.13 (mean +/- SD) nmol glutamine formed/mg protein/hr. Fifteen of 40 cases showed increased enzyme activity, including DMD and FCMD, the acute phase of polymyositis, and periodic paralysis. GPT activity in controls was 16.56 +/- 4.05 IU/mg protein. Sixteen of 40 patients showed increased enzyme activity: FCMD, FSH, MyD, inflammatory and endocrinological myopathy, and ALS. On the other hand, mitochondrial myopathy showed significantly decreased activity.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[An MRI study of hereditary spinocerebellar degenerations].
We evaluated magnetic resonance image (MRI) in 21 cases of hereditary spinocerebellar degenerations (SCD) of autosomal dominant trait. By the discriminant formula based on size of the cerebellar vermis and ventral pons, which was reported in our previous study, the patients were classified into three types. Group 1 included the cases with atrophies in the vermis and pons; OPCA type. Group 2 showed vermian atrophy and less significant atrophy in pons; LCCA type. And Group 3 was no significant atrophies both in vermis and pons. Cases in Group 1 were furthermore divided into two groups according to width of the midbrain tegmentum. Group 1A, with normal midbrain tegmentum, was consisted of five cases. Four cases were diagnosed as Menzel type OPCA. Another case showed various clinical symptoms and relatively mild atrophies for his duration of illness. His family members were classified to Group 3. Seven cases in Group 1B showed reduced midbrain tegmentum. Four cases showed ataxia, spasticity, ocular symptoms, bladder dysfunction and amyotrophy with or without fasciculation, and they seemed to be a special type of SCD mimicking Joseph disease. One case showed bulging eyes, ocular movement palsy and dystonia. However, his sister manifested only ataxia with very mild ocular movement disorder. Their MRI demonstrated severe atrophies in the cerebellum, pons and afferent cerebellar peduncli, and this pedigree was thought to be Menzel type OPCA with various associated disorders. Another case was clinically diagnosed as dentate-rubro-pallido-luysian atrophy. Group 2 was consisted of 6 cases who were clinically diagnosed as Holmes type LCCA. MRI demonstrated medial dominant cerebellar atrophy.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[MRI findings of the tongue in neurodegenerative diseases with bulbar sign].
We examined the magnetic resonance image (MRI) of the tongue in cases of amyotrophic lateral sclerosis (ALS), bulbo-spinal muscular atrophy (SBMA) and Shy-Drager syndrome (SDS) with sleep apnea. ALS case with severe bulbar disorder showed atrophic, irregularly margin tongue with increased signal intensity in T1-weighted MRI. ALS with less severe bulbar disorder demonstrated mixed hyperintensity and normointensity areas in the tongue. ALS without lingual symptoms showed increased intensity of tongue which suggested subclinical involvement of lingual muscle. BSMA case also showed atrophic tongue with diffusely increased signal intensity. SDS case with sleep apnea was revealed to have round shaped tongue without increased signal intensity, but his tongue fell into the posterior oral cavity, indicating hypotonus of genioglossus muscle. We concluded that MRI of the tongue is useful to determine the bulbar symptoms due to lower neuron disorder or not.
Aged↗
[MRI study of three siblings of suspicious Sjögren-Larsson syndrome].
We report the characteristics of high field magnetic resonance imaging (MRI) of three siblings with hereditary spastic paraplegia (HSP), probably of autosomal recessive inheritance. The proband was a 25-year-old woman who manifested slowly progressive spastic paraplegia, mental deficit, decayed teeth since her childhood. The siblings of the proband, 29-year-old woman and 27-year-old man, also showed the same clinical course as hers, and the symptoms are more severe in elder siblings. The proband solely had ichthyosis on her neck and lower thighs, which suggested Sjögren-Larsson syndrome. Sagittal and transverse slices of high field MRI (1.5T), T2-weighted (SE 2800/90) and T1-weighted (500/15) images were obtained. The cerebral atrophies were observed in the frontal and the parietal lobes, especially at the precentral and superior frontal gyri, whereas the occipital lobes were relatively spared. The atrophies of motor cortex seemed to be responsible for the disorder of voluntary movement. T2WI demonstrated a diffuse hyperintensity in the cerebral white matter, which suggested demyelination. T2WI also showed remarkable hypointensities in the globus pallidus, putamen, and thalamus. The degenerative involvement in these regions was also suspected. These abnormal intensities, both hyper- and hypo-intensities, were stronger in elder siblings, which indicated the intensity changes to be of progressive nature. Severe atrophy or hypoplasia of corpus callosum was also observed, however the cingulate gyri could be confirmed. We were not able to detect the callosal disconnection syndrome, because of their severe mental impairment. Spinal cord was slender and its degenerative changes were suggested. The high field MRI of a HSP family showed the extensive abnormal findings of the central nervous system in this HSP family, such as cerebral motor area, extrapyramidal system, corpus callosum, and spinal cord.
Adult↗
[MRI study of hemiballism].
MRI findings of four hemiballism cases are described, and pathophysiology, pathogenesis and treatment of hemiballism are discussed. All cases had no family history. The lesions revealed by MRI and the pathogenesis were different each other. Case 1, a 17 years aged girl with a history of hyperthyroidism and repeated tonsillitis, showed right sided hemiballism which was recovered by prednisolone and haloperidol. Although her involuntary movement was ameliorated by administration of sodium valproate and phenytoin, phenytoin caused allergic agranulocytosis which required prednisolone treatment. T2 weighted MRI at the 31st disease day demonstrated hyperintensities in the left caudate nucleus, putamen, lateral pallidum, perirubral area and substantia nigra. Hyperintensity in the prerubral area suggested involvement of the subthalamic nucleus or its connecting pathway. Fourteen months later, she suffered from convulsion and mental confusion. There were theta wave bursts and delta waves in EEG. No abnormal findings in MRI and positive antinuclear antibody (ANA: X320, speckled type) were observed. Case 2, a 78 year aged woman, suffered from right sided hemiballism. MRI findings at the 58th disease day were the left putaminal infarction and lacunar state in the bilateral caudate nuclei and the deep white matter of the centrum semiovale. There were no abnormal findings in the subthalamic nucleus. Case 3, a 51 year aged man with diabetes mellitus, had right sided hemiballism. X-ray CT at the 8th disease day showed hyperdensity in the left subthalamic nucleus region which could not be observed at the 12th day. Hypointensity in the left subthalamic nucleus region was observed in both T2 weighted and proton density MRI at the 52nd day. Case 4, an 82 year aged woman, had right sided hemiballism which remarkably diminished at the third disease day and disappeared by the fifth day. Any pathogenic lesion concerning to hemiballism was detected by X-ray CT or MRI.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Study of the blink reflex in spinocerebellar degeneration].
Blink reflex (BR) was composed of R1 and R2. R1 is considered to indicate the brainstem function. The purpose of this study was to evaluate the brainstem function of spinocerebellar degeneration (SCD) by the R1 latency (R1). BR was evoked by the percutaneous stimulation of supraorbital nerve. And R1 was measured by the action potential of orbucularis oculi muscle. Direct response was recorded by the percutaneous stimulation of facial nerve to determine the terminal latency of facial nerve (D). By these methods R1, D, R1/D and difference between at right and left side of these data (delta R1, delta D, delta R1/D) were measured in 21 normal subjects (57.7 +/- 10.1 ys.) and 28 cases of SCD (56.6 +/- 8.2 ys.), including 12 cases of sporadic type olivopontocerebellar atrophy (SOPCA), 4 of Menzel type olivopontocerebellar atrophy (MOPCA), 6 of late cortical cerebellar atrophy (LCCA) and 6 of Holmes type cortical cerebellar atrophy (HCCA). There was no difference between all types of SCD and normal subject at D, but significant difference of R1 and R1/d was observed between at MOPCA and normal subject. Concerning the difference between right and left side delta R1 and delta R1/D showed the significant difference with MOCA and SOPCA, but delta D showed no difference. Elongation of R1 and R1/D without the delay of facial nerve and the significant difference of R1 and R1/d between at right and left side suggested the synaptic transmission disorder form trigeminal nerve to facial nerve at brainstem of MOCA.
Adult↗
Histamine synthesis after administration of gastrin and blockade of acid secretion in the rat stomach.
Histidine decarboxylase (HDC) activity in the oxyntic gland and gastric volume were measured in rats treated with tetragastrin, cimetidine or omeprazole. HDC activity was dose dependently activated by not only tetragastrin but also cimetidine and omeprazole treatment. Histamine concentration in the oxyntic gland was reduced, but the amount of histamine in the gastric contents was increased by tetragastrin treatment. In rats premedicated with cimetidine or omeprazole, histamine concentration in the oxyntic gland and the amount of histamine in the gastric contents were not changed by administration of tetragastrin. It was concluded that tetragastrin activated HDC which increased histamine release into the gastric contents. Cimetidine and omeprazole induced the secretion of endogenous gastrin, leading to the activation of HDC.
Animals↗