Transcranial bypass for spontaneous intracranial carotid artery dissection--a case report.

A case of spontaneous intracranial artery dissection (IAD) of the anterior circulation is reported. A 32-year-old man developed left hemiparesis with headache. Angiographies (AGs) showed progressive occlusion of the distal end of the right internal carotid artery. He underwent a superficial temporal artery-middle cerebral artery anastomosis 20 days after his initial symptoms. He improved gradually after operation. The prognosis and treatment of IAD are discussed. The authors conclude that cases with IADs of the anterior circulation should be followed up by cerebral AG or magnetic resonance angiography and that early bypass surgery should be considered to prevent massive cerebral infarction in some cases.

Migraine with aura-like headache associated with moyamoya disease.

A 49-year-old woman was admitted to our hospital because of severe headache. She had a 10-month history of migraine with aura-like headache that occurred every 7 to 10 days and was preceded by photopsia. Brain CT showed cerebral infarction of the left occipital lobe. Bilateral carotid angiograms showed vascular occlusions in the supraclinoid portion of the bilateral internal carotid arteries with telangiectatic vessels acting as collateral channels to the occluded distal carotid arteries, which were consistent with the diagnosis of moyamoya disease. Headache resolved gradually and has never developed again after the infarct of the left occipital lobe. Pathophysiological mechanisms of migraine-like headache were discussed. We conclude that borderline perfusion of occipital lobe cortex could be a trigger for the development of migraine with aura-like headache in susceptible patients. In the case of atypical attack of migraine detailed investigation should be done to detect underlying vascular diseases such as moyamoya disease.

[Meningioma associated with acute subdural hematoma: a case report].

We present a case of meningioma associated with acute subdural hematoma. This 67-year-old male had a sudden onset of severe headache when he was on the train. He had a CT scan which revealed an acute subdural hematoma at the left parietal convex. Cerebral angiography disclosed a small focus (3 x 4 cm) of vascular stain under the left parietal bone supplied by the left middle meningeal artery. He was diagnosed as having a meningioma with surrounding acute subdural hematoma. The removal of this tumor was carried out without delay. It was fragile and the bleeding point was not detected. Pathological diagnosis was meningothelial meningioma. The literature showed meningioma associated with acute subdural hematoma is rare, but when it is discovered incidentally, surgical resection might be indicated.

[Valproate induced parkinsonism].

Among side effects due to sodium valproate (VPA) administration, parkinsonism is very rare. We report here three cases of VPA induced parkinsonism. Case 1 (75 year-old-woman) developed parkinsonism (rigidity, akinesia, postural instability) 41 month after the VPA administration (800 mg/day). Parkinsonism resolved within 6 months of discontinuing VPA. Case 2 (70 year-old-man) developed parkinsonism (rigidity, akinesia, postural instability, frozen gait) 15 months after the VPA administration (800 mg/day). Parkinsonism resolved within 6 months of discontinuing VPA. Case 3 (74 year-old-woman) developed parkinsonian gait 7 months after VPA administration (800 mg/ day). Parkinsonian gait resolved within a month of reducing VPA. Pathophysiologic mechanisms of this rare toxic reaction remain unknown. VPA induced parkinsonism is not so rare and has been under-reported and under-recognized. When we are confronted with the patients who develop parkinsonism after VPA administration, the possibility of VPA induced parkinsonism should be considered in the differential diagnosis. Old age, long duration of treatment, and VPA dose maintaining serum therapeutic levels might be predisposing factors for this syndrome.

Internal carotid-posterior communicating artery aneurysm manifesting as an unusual ocular motor paresis after minor head trauma--case report.

A 75-year-old female fell down in the road and hit her head. Two days later her right eye abduction was slightly limited on right lateral gaze. Cerebral angiography revealed an ipsilateral internal carotid-posterior communicating artery aneurysm. Craniotomy found the aneurysm had compressed the oculomotor nerve and was successfully clipped. The ocular motor paresis completely resolved after the operation. Therefore, the ocular motor paresis was possibly due to the aneurysmal compression to the oculomotor nerve. The oculomotor nerve stretched over the aneurysm was probably vulnerable to sudden mechanical stress, and aberrant innervation of the oculomotor nerve might be responsible for this unusual ocular motor paresis. Minor head trauma might precipitate ocular motor paresis in patients harboring an otherwise occult mass lesion at the skull base.

Mutism developing after bilateral thalamo-capsular lesions by neuro-Behçet disease.

We described a 44-year old right-handed man showing mutism, left hemiplegia and pseudobulbar palsy after CT and MRI documented bilateral thalamo-capsular lesions by neuro-Behçet disease. Single photon emission tomography (SPECT) and Xenon CT revealed hypoperfusion of the bilateral frontal lobes. The pathophysiological mechanism of mutism was discussed and we postulate that mutism might occur as the result of frontal lobe dysfunction due to the disconnection of thalamocortical fiber from thalamus to frontal cortex and that it could be interpreted as an incomplete form of akinetic mutism.

Developmental studies of dystrophin and other cytoskeletal proteins in cultured muscle cells.

We studied the developmental changes of localization of dystrophin and other cytoskeletal proteins, especially actin, spectrin and dystrophin related protein (DRP) using immunocytochemistry and quick-freezing and deep-etching (QF-DE) method. In developmental studies of mouse and human muscle cultures, some myoblasts had positive-reactions to spectrin, DRP, and F-actin, but not dystrophin. In aneurally cultured myotubes, dystrophin, DRP, and spectrin were localized diffusely in the cytoplasm and later in discontinuous patterns on the plasma membrane, when myotubes became mature. Spectrin and DRP had more positive reactions in immature myotubes, compared with those of dystrophin. In some areas of myotubes, dystrophin/spectrin and spectrin/actin were localized reciprocally. In innervated cultured human muscle cells, dystrophin and DRP were localized in neuro-muscular junctions, which were co-localized with clusters of acetylcholine receptors. By using the QF-DE method, dystrophin was localized just underneath the plasma membrane, and closely linked to actin-like filaments (8-10 nm in diameter), most of which were decorated with myosin subfragment 1. In actin-poor regions, spectrin was detected as well-organized filamentous structures in highly interconnected networks with various diameters. DRP was distributed irregularly with granular appearance inside the cytoplasm and also under the plasma membrane in immature mouse myotubes. Our present studies show that dystrophin, spectrin, and DRP are localized differently at the developmental stages of myotubes. These results suggest that dystrophin, spectrin, and DRP are organized independently in developing myotubes and these cytoskeletal proteins might play different functions in the preservation of plasma membrane stability in developing myotubes.

Developmental studies of the expression of myosin heavy chain isoforms in cultured human muscle aneurally and innervated with fetal rat spinal cord.

To study the influence of innervation of human muscle fiber type differentiation, we performed immunohistochemical studies using three monoclonal antibodies (McAbs) to myosin heavy chain (MHC) on cultured human muscles at different developmental stages. McAbs QM 355 (McAb-1), E 35-3 (McAb-2) and SM 1-11-2 (McAb-3) bound to fiber types I, IIA, IIB and IIC, types IIA, IIB and IIC, and type I, respectively. At the mononucleated cell stage the majority was immunonegative to the three McAbs; however, a few myoblasts were immunopositive to the McAb-1. They were also weakly stained with McAb-2 but not with McAb-3. In aneurally cultured myotubes (AMs), all myotubes were stained with the McAb-1 and 92.1% of AMs were positive to the McAb-2, whereas only a few (0.9%) AMs were immunopositive to the McAb-3. In contracting muscle fibers in an innervated area (CMis), which were co-cultured with fetal rat spinal cord explants, the percentage of the McAb-3-positive CMis was significantly increased (8.3%; P < 0.01) compared with that of AMs (0.9%). The double staining with the McAbs-2 and -3 clearly showed that slow MHC-positive muscle fibers without fast MHC only appeared in CMis. This is the first report of the neuronal influence on the expression of human adult slow MHC isoform derived from adult human satellite cells in vitro.

Immunocytochemical study of dystrophin in cultured mouse muscle cells by the quick-freezing and deep-etching method.

Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene, is deficient in patients with DMD and in mdx mice. It is immunocytochemically localized in skeletal muscle sarcolemma. However, little is known about the three-dimensional ultrastructural localization of dystrophin and its relationship with other cytoskeletal proteins. We found that dystrophin is localized irregularly, just underneath the plasma membrane in normal cultured mouse myotubes, by using the quick-freezing and deep-etching (QF-DE) method; it was found to be closely linked to actin-like filaments (8-10 nm in diameter), most of which were decorated with myosin subfragment 1, and was attached to the cytoplasmic side of the plasma membrane. These results suggest that dystrophin might play an important role in the preservation of cell membrane stability by connecting actin cytoskeletons with the cytoplasmic side of the plasma membrane.

The influence of muscle contractile activity versus neural factors on morphologic properties of innervated cultured human muscle.

In contrast to aneurally cultured human muscle, which is immature in regard to its morphologic phenotype and only rarely and weakly contracts spontaneously, innervated cultured human muscle fibres have: (1) nearly continuous, d-tubocurarine-inhibitable contractions; (2) well-developed cross-striations, basal lamina, t-tubules, and postsynaptic folds of the neuromuscular junctions; (3) the majority of their nuclei peripheralized; and (4) acetylcholinesterase-positive sites present only at the neuromuscular junctions. To see whether the expression of the muscle morphologic phenotype is induced only by neural factors generated from the spinal cord explants or also by their frequent contractile activity, we paralyzed innervated cultured human muscle fibres with 2 microM tetrodotoxin for four weeks, either from the first day of muscle contractions or following four weeks of muscle contractions. In both experimental designs, by light microscopy tetrodotoxin paralysis abolished cross-striations and caused prominent internalization of muscle nuclei; however, it did not influence the intensity of acetylcholinesterase staining at the neuromuscular junctions. By electron microscopy, there was no difference between paralyzed and contracting muscle fibres in development of t-tubules, basal lamina and postsynaptic folds. Our study demonstrates that in human muscle contractile activity: (1) regulates peripheral migration of nuclei and development of cross-striations; and (2) does not influence development of the neuromuscular junction, basal lamina, and t-tubules, which are mainly regulated by neural influences. This culture model may be useful for studying detailed mechanisms of human muscle fibre development and structural abnormalities in human neuromuscular diseases.

Glucocorticoid increases acetylcholinesterase and organization of the postsynaptic membrane in innervated cultured human muscle.

We have studied the influence of hydrocortisone (HC) on the neuromuscular junctions (NMJs) established on cultured human muscle fibers that had been innervated by fetal rat spinal cord neurons. Treatment with HC was begun 4 weeks after innervation and continued for 1-28 days. Four weeks of treatment significantly increased (a) size of acetylcholinesterase (AChE)-positive sites, indicative of NMJs; (b) intensity of AChE staining; (c) A12-AChE (junctional) molecular fraction; and (d) organization of junctional postsynaptic folds. The effect of HC depended on the dose and duration of treatment. These effects on the molecular properties of the postsynaptic component of the human neuromuscular junction could be through an action of HC directly on the muscle fiber or indirectly by affecting the motor neuron. Because the increased organization of the postsynaptic folds and the increased AChE seem to be salutory effects on the NMJ of prolonged HC treatment, these changes of the NMJ itself might contribute to the long-term beneficial effect of prednisone, another glucocorticoid, in myasthenia gravis patients.

Developmental study of the expression of dystrophin in cultured human muscle aneurally and innervated with fetal rat spinal cord.

So far there have been no developmental studies including the influences of innervation and contractile activity on the expression of dystrophin in cultured human muscle. We performed immunocytochemical studies of the localization of dystrophin on aneurally cultured non-contracting (AMs) and innervated continuously contracting cross-striated human muscle fibers (ICMs) with fetal rat spinal cord from normal and Duchenne muscular dystrophy (DMD) biopsied muscles. In normal AMs, myoblasts and some immature AMs showed negative staining of dystrophin, but many AMs had a patchy (discontinuous) distribution of dystrophin in the subplasmalemmal region and with some granularity near the sarcolemma and in the deeper cytoplasm. In normal ICMs, dystrophin was localized continuously at the inner aspect of the sarcolemmal membrane and some periodic dense patterns were detected in some areas. Both AMs and ICMs from DMD had negative staining of dystrophin. To investigate the muscle contractile activity on the distribution of dystrophin, we paralyzed ICMs with tetrodotoxin (TTX) for two weeks from the first appearance of muscle contractions. In paralyzed innervated muscles (PIMs), dystrophin remained in a patchy (discontinuous) pattern at the inner aspect of the plasmalemma similar to that in AMs. It is strongly suggested that muscle contractile activity plays an important role in the continuous and even distribution of dystrophin at the sarcolemma during development.