Six cases of Cushing's disease, in which perplex diagnostic problem was encountered, were reported. In the endocrinological examination two cases were not suppressed by a high dose of dexamethasone (8 mg), and the other four cases showed complicated clinical findings due to adrenocortical nodular hyperplasia. Among those four cases with adrenocortical nodular hyperplasia two of them were of functionally autonomic and showed different attitude in responding endocrine assay. When the serum cortisol is not suppressed by a high dose of dexamethasone, differential diagnosis from other causes, such as Cushing's syndrome due to adrenocortical macronodular hyperplasia, becomes quite important for surgical treatment. Usually plasma ACTH in the case of Cushing's disease is slightly increased over the normal range and, on the other hand, it is lower than normal value in the case of Cushing's syndrome due to autonomic adrenocortical nodular hyperplasia because of negative feed back phenomenon. However, in some cases of Cushing's disease plasma ACTH is within normal range or even lower than normal level, and, furthermore, it is not extremely unusual to find a case of adrenocortical macronodular hyperplasia with slightly increased plasma ACTH level over normal range. Those cases offer particularly complex problem in making diagnosis and therapeutic indication. According to our clinical experiences with those six cases, selective venous sampling is found quite useful in differentiating Cushing's disease from other causes and ACTH value in selective venous sampling from inferior petrosal vein is remarkably increased in the cases due to pituitary ACTH secreting adenoma. Cushing's disease with adrenocortical nodular hyperplasia reduces the ACTH value in the sampling to the lower normal range as the adrenocortical nodular hyperplasia progresses.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenoma↗

PubMed1984

Dorsal cyst malformations. Part I. Clinical study and critical review on the definition of holoprosencephaly.

Among the cerebral malformations accompanying cystic cavity in the dorsal midline, common radiological findings of galenic dysgenesis are found in 10 cases, which are now reported as dorsal cyst malformations. The authors classified dorsal cyst malformations into prosencephalic, interhemispheric, and porencephalic types according to clinical, radiological, and presumed pathogenetic characteristics. In contrast to recent trends that most of cerebral malformations with dorsal midline dysgenesis are included in the holoprosencephalic category, the authors emphasize that these interhemispheric and porencephalic types of dorsal cyst malformations, though presenting interhemispheric cyst with callosal defect or broad communication between the lateral ventricles, should be separated from holoprosencephaly, because they are completely hemispheric.

Agenesis of Corpus Callosum↗

PubMed1984

[Origin of components P 11 and P 13 in short latency somato-sensory evoked potentials (SSEP): correlative study of SSEP and intraoperative evoked potentials].

In 15 patients with cervical or posterior fossa lesion, SSEPs were recorded between the skull and the non-cephalic reference electrodes during the surgical operation and compared with the evoked potentials directly recorded at the same time from the surface of the cervical spinal cord and the brain stem. The directly recorded evoked potential consisted of three main components appeared within about 25 ms., they were a small negative spike wave, a large positive spike wave and a subsequent slow potential. The positive spike wave of the evoked potentials recorded from the surface of the dorsal column was not only coincided in latency with component P 11 of SSEP, but also showed the greatest amplitude at the lower cervical level. Moreover, the positive spike wave gradually delayed in latency and reduced in amplitude from lower to upper cervical segments. The amplitude of the positive spike wave was greater at the surface of the dorsal column ipsilateral to the stimulated median nerve than that of contra-lateral recording. No polarity change was observed between the anterior and posterior surface of the spinal cord. Similarly, the positive spike wave of the evoked potentials, recorded from the surface of the brain stem, showed fairly same latency with P 13 and a maximal amplitude at the surface of the cuneate tuberculum ipsilateral to the median nerve stimulated, and those positive spike wave traveled to contra-lateral ventral surface of the pons, presumably from ipsilateral cuneate nucleus to the contra-lateral medial lemniscus.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1984

Dorsal cyst malformations. Part II. Galenic dysgenesis and its embryological considerations.

In the preceding part, galenic dysgenesis was stated to be a diagnostic hallmark of dorsal cyst malformations. Failed formation of the galenic drainage system conducts the diencephalic drainage laterally into the transverse sinuses as in the embryonal stage, and it is frequently associated with arrested descent of the torcular portion or other anomalies of the dural sinuses. Highly malformed structures of the deep cerebral veins and the dural sinuses seen in this malformation are correctly interpreted in comparison with findings of venous injection studies performed in human fetuses. Pathogenesis of this malformation is also discussed on the basis of embryological studies of the dural sinuses and falcotentorial structures.

Brain↗

PubMed1984

[Prognostic significance of intraventricular hematoma in the cases of intracranial hemorrhage].

Fifty two patients with intracranial hemorrhage, which was diagnosed with CT scan, were reviewed in relation to the prognostic significance of the intraventricular hematoma. Following items were analyzed in each patient in order to classify the severity of the patients; cause of the hemorrhage, level of the consciousness at the time of evaluation, volume of the intraventricular hematoma, midline shift, cast formation of the intraventricular clots in the third and/or fourth ventricle and acute hydrocephalus. The total mortality rate of the patients analysed was 32.7%, whereas 42.3% of the patients returned to useful life. Level of the consciousness and cause of the hemorrhage were found most contributory factors to the prognosis, and, in general, the patients whose consciousness were better than in semicomatose state, could expect reasonably fair prognosis. When the level of consciousness was worse than semicoma, they were divided in two groups according to the cause of hemorrhage. Cases with ruptured intracranial aneurysm or hypertensive hemorrhage showed as a rule poor prognosis and, on the contrary, patients with AVM or MoyaMoya disease were not always poor in outcome. In the former group the level of consciousness was quite low and the prognosis was poor with higher mortality, if the intraventricular clot occupied over two third of the ventricles. However, cases with intraventricular clot caused by AVM stayed in good level of consciousness even the intraventricular hematoma extended into the all ventricles, and the prognosis was not pessimistic unless the AVM was unoperable. The midline shift on CT scan was well correlated with the high mortality in the cases of ruptured aneurysm.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1984

[A clinico-physiological study on the generators of short latency somatosensory evoked potential].

The purpose of this paper is to locate the generators of each wave component in the short latency somatosensory evoked potential (SSEP), by means of cumulative analysis of the SSEP obtained from various localized lesions in the upper cervical cord through brain stem and cerebral subcortical structures. Since there are considerable inconsistency of naming each component in the literature, SSEP to median nerve stimulation of 10 normal subjects were examined by two different recordings, i.e. recording from an electrode at the parietal scalp with a reference electrode on Erb's point (Par.-Erb), and the other at the frontal scalp with a reference electrode on Cv 7 (Fro.-Cv 7), and the SSEP was carefully studied. In normal subjects, the SSEP by Par.-Erb lead yielded 5 negative components (N 7, N 11, N 16, N 18 & N 26) and 4 positive components (P 9, P 13, P 22 & P 42), while by Fro.-Cv 7 5 negative components (N 7, N 10, N 12, N 16 & N 28) and 5 positive components (P 9, P 11, P 13, P 20 & P 44). Thirty three patients were subjected to analyse the influence of localized lesions to each component of the SSEP and the recording was evaluated in regard to (a) identification of each component, (b) latency of each component and inter-peak latency difference exceeding 2 SD, and (c) over 50% asymmetry and laterality of the amplitude. Cervical spondylotic myelopathy, high cervical cord tumor, tonsillar herniation, pontine infarct and hemorrhage, circumscribed thalamic lesion, and vascular lesion of centrum semiovale were carefully examined with CT scan and the findings were compared with neurological findings periodically. SSEP was taken repeatedly, especially before and after operative intervention, and alteration of the component was referred to the clinical progress of the lesion. In conclusion, results obtained from our present observation indicated that P 9 was the extramedullary projection, P 11 was intramedullary origin of the lower cervical cord, P 13 was medulla oblongata origin and P 13-N 16 was projection from medulla oblongata to thalamus. N 16-N 18 and N 26 were considered projection from thalamus to hand area of the parietal lobe with some association area and N 28 had the generator widely based on frontal projection system. These findings appeared to be quite useful for topographic diagnosis and functional evaluation of the lesions in central nervous system.

Adolescent↗

PubMed1984

[A case of familial type IIa hyperlipoproteinemia with intracranial xanthoma].

Familial hyperlipoproteinemia associated with intracranial xanthoma belonged to a very rare entity and the authors were able to collect only one reported case in our domestic literature. A 57-year-old house wife was admitted with a six months' history of progressive hearing impairment on the left and some cerebellar signs characterized by ataxic gait. Physical examination revealed several hard and painful subcutaneous masses around the joints of four extremities, which had enlarged slowly within the last three years. Also, remarkable deformity of Achilles tendon was seen on both sides. Neurological examination showed bilateral papilledema, left hemifacial hyp- and dysesthesia, left hearing difficulty and left cerebellar signs. Laboratory examination reported markedly elevated serum cholesterol level as high as 575 mg/dl, and determination of the serum lipoprotein disclosed the findings compatible with Type II a hyperlipoproteinemia. Plain skull X-rays showed osteolytic defect of the left occipital bone and CT scan demonstrated a large irregular low density mass extending into the left posterior fossa, which showed ring like enhancement and spotty high density of calcification. Angiogram suggested the mass was of less vascular lesion and situated extramedullary. Suboccipital craniectomy was performed and an epidural solid mass was resected, although the mass was markedly growing into the posterior cranial cavity. Microscopic examination showed the findings typical to the xanthoma, which was totally coincided with that of biopsy specimen obtained from the tumor over the ankle. Electron microscopic examination was performed and various kinds of lipid inclusions in the cytoplasma of xanthoma cells, such as dense bodies, volute or onion-like figures and lucent large vacuoles, were observed.

Adolescent↗

PubMed1984

[Lipids metabolism of the patients with subarachnoid hemorrhage due to ruptured intracranial aneurysm, in comparison with other cerebrovascular diseases].

Lipids metabolism has been extensively studied in the large number of patients with hypertensive cerebral hemorrhage or cerebral infarction, however, the patients with subarachnoid hemorrhage due to ruptured intracranial aneurysm were customarily excluded from the study. Total cholesterol, LDL-cholesterol, HDL-cholesterol, triglyceride and atherogenic index were determined in 40 cases, who had undergone surgical treatment for ruptured intracranial aneurysm in various locations, and the results were compared with the data obtained from 20 operative cases with hypertensive cerebral hemorrhage, 40 cases with non-operative cases with hypertensive cerebral hemorrhage, 60 cases with cerebral infarction and 20 cases of cerebral lesions other than cerebrovascular disease (non-CVA). Serum levels of LDL-cholesterol and atherogenic index were significantly correlated with the preoperative grading of subarachnoid hemorrhage when compared with non-CVA (p less than 0.05). Total cholesterol, LDL-cholesterol and atherogenic index were well correlated with operative morbidity and active daily life (ADL) in the follow-up study of the patients with intracranial aneurysm, where the higher value of cholesterol indicated the poorer risk of the patients. In our present study, none of the data was found significant, when compared with non-CVA, in the other groups of cerebrovascular diseases except for the HDL-cholesterol, which was found significantly in low level in the group of cerebral infarction (p less than 0.001). Triglyceride was found elevated in the group of hypertensive hemorrhage indicating negative correlation to the severity, and rather similar pattern was seen in the subarachnoid hemorrhage, however, the data were not conclusive.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral Hemorrhage↗

PubMed1983

Pseudopsammoma bodies in meningotheliomatous meningioma. A histochemical and ultrastructural study.

A case of meningioma arising from the right sphenoidal ridge is presented. Histologically, the tumour was composed of whorls of meningotheliomatous cells and many tumour cells containing intracellular inclusions were PAS positive, but diastase and neuraminidase resistant. The inclusions were named pseudopsammoma bodies by Kepes in 1961. The meningioma with pseudopsammoma bodies are a very rare entity and have been described in only 7 previous reports. We had an opportunity to examine one case of a meningioma with pseudopsammoma bodies. Electron microscopically, these substances were located in the intracellular spaces lined by microvilli. In earlier reports, the substances were said to be secretory products and a possibility of meningioma cell differentiation to secretory cell was postulated. Using the Concanavalin A staining method, the results of our present study strongly support this opinion.

Adult↗

PubMed1983

Large infratentorial multilocular arachnoid cyst.

Clinical and radiological findings in a 2-year-old boy with a large multilocular infratentorial arachnoid cyst are presented. The cyst was extended in the bilateral paracollicular and retrocerebellar region and was communicating with each other but not with the ventricular system. Associated with those cysts was a significant degree of hydrocephalus due to compression of the fourth ventricle. Delayed metrizamide CT scan showed totally isolated cysts from CSF circulation although the cystic content was determined as the CSF.

Arachnoid↗

PubMed1983