Blink reflexes were studied in 20 patients with hemifacial spasm. The latencies of R1 on the affected side was not different from that on the unaffected side in 10 patients who had no previous facial nerve block, but increased in 10 patients with experience of facial nerve block. Synkinetic responses (SR 1, SR 2 and SR 2 c) were observed by the simultaneous recording from the orbicularis oris muscle on the affected side. SR 1 were obtained in all patients (100%), but SR 2 in 18 patients (90%) and SR 2 c in 19 patients (95%). The mean latency of SR 1 was slightly longer than the mean latency of R 1. When the site of stimulation was moved along the eyebrow from the supraorbital nerve approaching to the stylomastoid foramen, the latencies of R 1 and SR 1 became shorter, but R 2 and SR 2 were suppressed with increased latency. Following local anesthesia (xylocaine) to the temporal branch of the facial nerve, SR 1 was abolished with preserved R 1, R 2 and SR 2. On the other hand, R 2 and SR 2 were abolished with preserved R 1 and SR 1 following xylocaine block to the supraorbital nerve. The result suggested that the afferent pathway of SR 1 is the facial nerve (antidromic conduction) and that of SR 2 is the trigeminal nerve. Ephaptic transmission of those antidromic and orthodromic impulses forming a reverberating circuit should be one of the etiological factors in hemifacial spasm.

Adult↗

PubMed1987

[Pathogenesis and the treatment of secondary syringomyelia].

Six cases with secondary syringomyelia were evaluated clinically and the pathogenesis was discussed. Three cases had the tumors; an ependymoma arising from the conus medullaris and the filum terminale, a foramen magnum meningioma extending to C2 and a thoracic astrocytoma. Two cases had past history of spinal cord injury with L1 and L2 fracture-dislocation, respectively. One case showed hydromyelic symptoms associated with isolated fourth ventricle after post-meningitic hydrocephalus. Clinical symptoms and signs were complex and various in each case due to the association of the original disease and the syrinx. Syringomyelic symptoms were dominant in three cases of which the syrinx extended from the conus to the cervical cord. Initial symptoms of two cases with post-traumatic syringomyelia were tingling pains which began near the site of injury and extended rostrally. Metrizamide myelography revealed complete or incomplete block at the location of the tumors or the injuries. Delayed CT demonstrated the syrinx in all cases. The syrinx was always present near the sites of primary lesions. The communication between the syrinx and the fourth ventricle was suspected in three cases, and the communication of the syrinx and the spinal subarachnoid space was suspected in two cases. All cases underwent the surgical treatments. Total removal of the tumors were completed in two cases and relieved the majority of symptoms. On the other hand, a case with a thoracic astrocytoma underwent biopsy of the tumor and irradiation, followed by poor outcome. Syringo-peritoneal shunts were performed in two cases with post-traumatic syringomyelia and relieved pain, but neurological signs were unchanged.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1986

The distribution of calcineurin in rat brain by light and electron microscopic immunohistochemistry and enzyme-immunoassay.

Calcineurin is the calcium (divalent cations)-dependent calmodulin-stimulated phosphoprotein phosphatase which is capable of dephosphorylating various substrate proteins. The subcellular and regional distribution of calcineurin in the rat brain has been studied by light and electron microscopic immunohistochemistry using antiserum against calcineurin. Immunoreactivity was observed in many neurons but was not detected in glial cells, such as astrocytes, oligodendrocytes and ependymal cells by the PAP method. Light microscopy demonstrates strong immunoreactivity in neuronal somata and neurites. By electron microscopy, calcineurin immunoreactivity was found to be present in dendrites including postsynaptic densities, somata, spines, axons and terminals. Calcineurin immunoreactivity was present in neurons throughout the brain, but a marked regional variation in strength of the immunoreactivity was observed. The caudatoputamen, hippocampal formation, and substantia nigra were strongly stained. Cerebral and cerebellar neocortex showed moderate immunoreactivity. In substantia nigra and globus pallidus, only neurites were stained, but neuronal somata not. The staining of the substantia nigra was thought to be due to that of the nerve terminals originating from the caudatoputamen, in view of the findings by cerebral hemitransection and electron microscopic immunohistochemistry. We developed an enzyme-immunoassay (EIA) for calcineurin. The sensitivity of the EIA was 1 ng (13 fmol) of calcineurin. We determined the level of calcineurin in various regions of the rat brain. The caudate nucleus, putamen and hippocampal formation showed a high concentration of calcineurin. The results are consistent with those obtained by immunohistochemistry.

Animals↗

PubMed1986

Calcineurin as a neuronal marker of human brain tumors.

Calcineurin is one of the calmodulin binding proteins and a Ca2+-dependent and calmodulin-stimulated phosphoprotein phosphatase. We used antisera to the calcineurin as a cell-type-specific marker in order to identify neuronal cells in the rat brain and human neoplasms. In normal rat brain slices, basal ganglia were stained macroscopically, and other areas such as cerebral cortex, corpus callosum, cerebellar cortex, granular layer and pyramidal tract of the spinal cord were lightly identified as well. Under the light microscope, it was found that only the neuronal cells were stained, and astrocytes, oligodendrocytes, ependymal cells and vessels were not. Intracellular distribution of the staining showed various patterns and staining intensity of varying degree. Using the PAP method, localization of the calcineurin in formalin-fixed, paraffin-embedded tissues were studied in 65 human intracranial neoplasms, and in 11 human extracranial neoplasms. The neuronal elements of neuroblastoma, ganglioglioma, ganglioneuroma and retinoblastoma were clearly stained. In contrast, glioblastoma, astrocytoma, oligodendroglioma, ependymoma, meningioma, neurinoma, pituitary adenoma, craniopharyngioma, hemangioblastoma, hamartoma, lymphoma and mesenchymal tumor were all negative. Two cases out of 5 medulloblastomas were stained, but others were not. Although positive tumors disclosed various staining patterns and intensities, these results indicated that calcineurin could be a new neuronal marker in human brain tumors.

Animals↗

PubMed1986

Calcineurin in human brain and its relation to extrapyramidal system. Immunohistochemical study on postmortem human brains.

Calcineurin immunoreactivity has been successfully detected in formalin-fixed paraffin-embedded postmortem human brain tissue using the peroxidase-antiperoxidase method. We have examined two autopsy cases with Huntington's disease (HD), three cases with Parkinson's disease, and two senile patients as controls. In the controls, calcineurin immunoreactivity was present in neuronal cells only and highly concentrated in the caudate nucleus, putamen, globus pallidus (striato-pallidal pathway), substantia nigra (striato-nigral pathway) and hippocampal formation. These localizations were similar to those identified in rat brain. There was a marked depletion of neurons containing calcineurin in the caudate nucleus and putamen, and a marked reduction of calcineurin-immunoreactive nerve fibers in the globus pallidus and substantia nigra were found in the cases with HD, but not in those with Parkinson's disease. These findings suggest that calcineurin can be a useful and specific index of neuronal degeneration in the caudato-putamen resulting from extrapyramidal disease, and that the calcineurin-immunostaining method can be a valuable tool for clarifying the anatomy of the human extrapyramidal system.

Aged↗

PubMed1986

Enlargement of a cerebral aneurysm associated with ruptured arteriovenous malformation.

Enlargement of a small aneurysm during an eleven year period was encountered in a 32-year-old man who had experienced intracranial haemorrhage from a distally located AVM. Clipping of the feeding artery together with the aneurysmal neck was performed. It was very rare to observe the aneurysmal growth associated with the AVM. This case was considered worth reporting as supporting evidence that hemodynamic stress plays an important role in the development of an aneurysm.

Adult↗

PubMed1986

Intracerebral venous angioma with arterial blood supply: a mixed angioma.

An 18-year-old man was operated upon with a diagnosis of intracerebral hematoma. Although the angiogram of the right carotid artery demonstrated the characteristic umbrella appearance of the venous angioma in the parietal lobe, fine arterial branches supplying the vascular malformation were noted in the preoperative angiogram. At the operation, no nidus was found, and delicate angiomatous networks with red veins were observed around the hematoma cavity. Histologic examination of the angiomatous network proved it to be an arteriovenous malformation. It was a mixed angioma, which combined venous angioma and arteriovenous malformations. Clinical, angiographic, and pathological features of intracerebral venous angiomas with arterial components were reviewed from the literature.

Adolescent↗

PubMed1986

Acute epidural hematoma complicating Paget's disease of the skull.

Although among Anglo-Saxons Paget's disease is relatively common, it is very rare in Japan. The incidence of Paget's disease increases as age advances, and in elderly people over 90 years of age it can be seen in as many as 10%. On the other hand, acute epidural hematoma seldom occurs in the older generation, because dural adhesion to the internal table of the skull becomes more intense. We have encountered an 86-year old woman with advanced Paget's disease who developed an acute epidural hematoma after trivial head trauma. Acute epidural hematoma complicating Paget's disease has been reported only twice before and this case is the first report in Japan. The literature on the pathogenesis of the acute epidural hematoma complicating Paget's disease is reviewed.

Aged↗

PubMed1986

Neuroradiologic findings of hypothalamic hamartoma with emphasis on computed tomography.

Hypothalamic hamartoma is a relatively rare congenital malformation. Five new cases and 31 cases in the literature were evaluated in regard to neuroradiologic findings with emphasis on computed tomography. Five important computed tomography findings were observed: oval-shaped, isodense suprasellar mass; clear demarcation; no enhancement effect; absence of cystic component or calcification; and no effacement of the third ventricle. Clinical features, mechanism of the precocious puberty, and differential diagnosis are also discussed.

Child↗

PubMed1986

Syringomyelia and syringobulbia associated with an ependymoma of the cauda equina involving the conus medullaris: case report.

A patient with syringomyelia and syringobulbia secondary to an asymptomatic ependymoma of the cauda equina involving the conus medullaris is described. Delayed metrizamide computed tomography myelography was decisive for making the diagnosis of a highly extended syrinx associated with the tumor. Syringomyelic symptoms improved remarkably following the removal of the tumor, and the syrinx was not visualized in a postoperative study. The pathogenesis of syringomyelia associated with a caudally located spinal cord tumor is discussed.

Adult↗