Brain autopsy findings in a 14-year-old patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes were compared with those of computed tomography (CT) and magnetic resonance imaging (MRI). Pathologic examinations revealed extensive laminar necrosis bordered by gliotic tissues throughout the cerebral cortices. Moderate losses of myelin and fibrous gliosis were also observed in the subcortical and deep white matter. These lesions were demonstrated as low-density areas on CT and as high-signal areas on T2-weighted MRI. MRI revealed the lesions more distinctively and precisely than CT. Neither CT nor MRI could reveal abnormalities in the basal ganglia, including vascular proliferation and calcium deposits in the blood vessels.

Acidosis, Lactic↗

PubMedDate not supplied

The problem of de novo colorectal carcinoma.

From April 1985 to March 1995, colonoscopy was carried out at our institution in 24,059 patients, 31,800 times in symptomatic and/or asymptomatic average risk persons. 184 submucosal invasive carcinomas were detected. Unlike protruding-type lesion, the depressed-type invades the submucosal layer, even though the size is within 10 mm. The depressed type of invasive carcinoma accounted for 20 lesions, and represented 10.9% (20 of 184) of all the invasive carcinomas. The pit pattern of depressed-type lesions shows a small round pit (type IIIs pit pattern) and that of carcinoma lesions shows the irregular pit and non-structure (type V pit pattern).

Adenoma↗

PubMedDate not supplied

Degeneration of the posterior columns of the spinal cord: postmortem MRI and histopathology.

Postmortem MR images of two cases of posterior column degeneration were correlated with histopathological findings. The abnormal MR findings were volume loss and high signal intensity of the posterior columns associated with mild deformity of the dorsal surface of the spinal cord, corresponding to tract degeneration on histopathological examination. These results strongly suggest that further development of MR technique will permit in vivo detection of posterior column degeneration.

Adult↗

PubMedDate not supplied

The organization of astrocytes in organotypic mouse spinal cord culture: an electron microscope study.

The organization of astrocytes in myelinated culture of mouse spinal cord tissue was analysed ultrastructurally and compared with the pattern in vivo. Astrocyte cell bodies and their processes, connected by punctate adhesions and gap junctions, formed a continuous layer around the entire explant. Throughout the parenchyma, thin astrocytic processes penetrated the neuropil, separated neurons, and invested some synaptic complexes. Others formed flattened sheets and contacted directly with the basal surface of ependymal cells. In the absence of mesenchymal elements, astrocytes in vitro occasionally possessed fragments of basal lamina on the surface of the explant and around minute intercellular spaces. Except for an incomplete basal lamina, these findings indicate that astrocytes in vitro are organized in a manner essentially identical to the situation in vivo.

Animals↗

PubMedDate not supplied

Repetitive monomorphic ventricular tachycardia in a 4-year-old boy with toxic multinodular goiter.

A case of toxic multinodular goiter associated with repetitive monomorphic ventricular tachycardia (VT) is reported. A 4-year-old boy was found to have asymptomatic VT. When treatment with antiarrhythmic agents turned out to be ineffective, thyrotoxicosis was suspected due to the rapid enlargement of the left thyroid gland and associated thyroid function studies. A diagnosis of toxic multinodular goiter was made on the basis of subsequent scintigraphy and ultrasonography. Treatment with antithyroid drugs and inorganic iodine restored the thyroid function to normal, and was accompanied by the disappearance of VT. A left thyroid lobectomy was performed, and the pathological findings were compatible with toxic multinodular goiter. After the operation, the patient was transiently hypothyroid and had no VT without medication. A review of the literature revealed no previously documented cases of VT with toxic multinodular goiter.

Anti-Arrhythmia Agents↗

PubMedDate not supplied

Granulofilamentous profiles in lower motor neurons: a sporadic case of amyotrophic lateral sclerosis with many Lewy body-like inclusions.

In a 62-year-old man with an 8-month course of sporadic classical amyotrophic lateral sclerosis, many Lewy body-like hyaline inclusions (LI) were observed in spinal anterior horn cells, hypoglossal nuclei, nucleus ambiguus, and motor nuclei of the trigeminal nerve. These motor neurons showed a mild degree of neuronal loss, several Bunina bodies, spheroids and chromatolytic neurons. Tract degeneration was limited to pyramidal tracts. In addition to intensely stained LI, immunoreactive skeins or granules were recognized by a polyclonal anti-ubiquitin antibody. Thick filaments of 15 to 20 nm in diameters with granules formed conglomerated masses with varying amounts of neurofilaments in the anterior horns, corresponding to light microscopically observed LI. More commonly, these thick granulofilamentous profiles were dispersed in small bundles or individually in the cytoplasm. Electron microscopically, there was no close association of filaments with Bunina bodies.

Actin Cytoskeleton↗

PubMedDate not supplied

Pathology of motor neurons in amyotrophic lateral sclerosis with dementia.

One type of motor neuron disease (MND) associated with dementia is well known for the fact that it displays atrophy of the frontal and temporal lobes, neuronal loss and sponginess of the superficial layers of the cortex, and subcortical gliosis (so-called Mitsuyama type). In an attempt to determine characteristics of the pathology of motor neurons, three cases of MND with dementia were examined and compared with 16 cases of sporadic classical amyotrophic lateral sclerosis (ALS) with light and electron microscopes. All these cases with dementia showed loss of motor neurons, chromatolysis, spheroids, and Bunina bodies. Quantitative analyses of the 5th lumbar anterior horns showed no significant differences in incidences of chromatolysis and Bunina bodies between cases with and without dementia. Ultrastructures of the anterior horns were essentially identical in both groups. Therefore, the pathology of motor neurons at least in one type of MND with dementia seems to share common features with that in sporadic classical ALS without dementia.

Aged↗

PubMedDate not supplied

Adult-onset motor neuron disease with basophilic intraneuronal inclusion bodies.

A 53-year-old man initially displayed muscle atrophy and weakness and a slight degree of pyramidal signs, which became more prominent with time. Later, gaze palsy, autonomic dysfunction, and bedsore developed. The total clinical course was 63 months. Pathologically, there was loss of upper and lower motor neurons and other regions, as well as degeneration of the anterior and lateral funiculi of the spinal cord. Round or irregularly-shaped basophilic inclusions were found not only in the motor neurons, but also in other regions such as the putamen, globus pallidus, thalamus, subthalamic nucleus, red nucleus, midbrain tegmentum, pontine nucleus, dentate nucleus, inferior olivary nucleus and others. The inclusions consisted of randomly arranged 12-25 nm thick filaments studded with granules. This patient with adult onset of the disease exhibited features resembling those of "juvenile amyotrophic lateral sclerosis" with basophilic inclusions. Clinically as well as pathologically, the disease manifestations involved many systems other than the motor neurons.

Age Factors↗

PubMedDate not supplied

Subcortical neurofibrillary tangles, neuropil threads, and argentophilic glial inclusions in corticobasal degeneration.

This report concerns an autopsy case of corticobasal degeneration (CBD). The patient was a 64-year-old woman with slowly progressing clumsiness and gait disturbance who died approximately 7 years after the onset of symptoms. The salient neuropathological findings were focal loss of neurons of the cerebral cortex, ballooned neurons and gliosis of the subcortical white matter, thalamus, and basal ganglia. In addition, there were marked neuronal loss and gliosis in the substantia nigra. The ballooned neurons were positively immunostained for phosphorylated neurofilament, alpha B-crystallin and synaptophysin; staining for the latter was intraneuronal. The examination of silver impregnated and Tau immunostained preparations revealed numerous basal neurofibrillary tangles, neuropil threads, and glial inclusions; their presence was most pronounced in the brainstem tegmentum, basal ganglia, and thalamus. Neurofibrillary tangles consisting of straight tubules with a diameter of approximately 13-15 nm and constricted tubules were identified in the substantia nigra. The results of the present study indicate that the cytopathology of the subcortical gray matter and brainstem in CBD patients resembles that of progressive supranuclear palsy.

Axons↗

PubMedDate not supplied

Sporadic amyotrophic lateral sclerosis with dementia and Cu/Zn superoxide dismutase-positive Lewy body-like inclusions.

This report concerns a case of sporadic amyotrophic lateral sclerosis (ALS) with dementia and Lewy body-like hyaline inclusions (LBHIs). The patient was a 70-year-old woman who initially showed memory disturbance and later developed bulbar palsy, muscle atrophy and weakness. The total clinical course was 51 months. The postmortem examination revealed superficial sponginess and subcortical gliosis in the frontotemporal cortices. Ubiquitin-positive intraneuronal inclusions were found in small cortical neurons of the frontotemporal lobe. Neuronal loss was marked in the spinal anterior horn with degeneration of the pyramidal tracts. The anterior horn cells had ubiquitin-immunoreactive skein-like inclusions and Bunina bodies. LBHIs were present in the lumbar horn; ultrastructurally they were composed of randomly arranged thick filamentous structures studded with granules. The LBHIs were intensely stained with anti-ubiquitin antibody. As in familial ALS and in certain cases of sporadic ALS, some of these inclusions reacted with an antibody against Cu/Zn superoxide dismutase, the enzyme whose gene was recently found to be mutated in some forms of familial ALS.

Aged↗