Characterization of a phospholipid antigen reacting with serum antibody in patients with peripheral neuropathies and paraproteinemia.

A phospholipid antigen that reacted with the serum antibody from a patient with peripheral neuropathy and paraproteinemia with both impaired sensory and motor functions, but not with sera from patients with only impaired sensory functions and healthy controls, was purified from bovine cauda equina as a minor component with a concentration of about 0.6 microg per gram wet-weight tissue. The structure of the phospholipid was characterized as lysophosphatidylinositol by means of thin-layer chromatography, gas-liquid chromatography, and negative-ion fast-atom-bombardment mass spectrometry. The major fatty acid component of this phospholipid was stearic acid (> 81%). Our data suggest the possible involvement of a lysophospholipid antigen in the immunopathogenesis of peripheral neuropathies with severe motor and sensory dysfunctions. There is an intriguing possibility that the difference in immunoreactivity of serum antibodies may underlie the differential clinical manifestations in patients with peripheral neuropathy and paraproteinemia.

[Persistent primitive olfactory artery: report of 5 cases].

Five cases of a persistent primitive olfactory artery are reported. The anomalous artery coursed anteromedially along the ipsilateral olfactory nerve and made a hairpin turn, supplying the circulation of the anterior cerebral artery. It was proposed that a primitive embryonic olfactory artery had remained as this artery. A cerebral aneurysm was located at the hairpin curve of the artery. Hemodynamic stress is considered to have an important role in aneurysm formation. Any aneurysm occurring at an artery should be considered for surgical treatment.

[Usefulness of galea suturing method for scalp closure].

A method for scalp closure to prevent alopecia along a suture line is described. Only the galea is sutured. By tacking a sufficient width of the galea on both sides with an absorbable suture material, the sutured wound forms a ridge. The outer layer is then closed with skin staples to keep the blood circulation undisturbed. This procedure contrasts with the traditional method in which the galea is sutured with the overlying subcutaneous tissue and consequently the hair follicles are strangled. By adopting the method of suturing the galea, development of alopecia along a suture line has been effectively prevented and scarring has become less conspicuous.

A case of abducens neurinoma mimicking acoustic neurinoma.

Neurinoma arising from the abducens nerve independent of neurofibromatosis has been rarely reported in the existing literature. We present a case of abducens neurinoma which was confirmed during a surgical excision. A 31-year-old female had experienced a hearing disturbance for the past 8 months. Abduction of the right eye ball was almost full. Magnetic resonance images showed a tumor having a size of 44 x 33 X 33 mm at the right cerebellopontine angle. Neuro-otological examination revealed the findings specific to acoustic neurinoma. Surgical excision confirmed that the tumor originated from the abducens nerve. The tumor located at the cavernous sinus or cerebellopontine angle might originate from the abducens nerve, though the abduction of eye ball is not in any way impaired.

Novel modification of ceramide: rat glioma ganglioside GM3 having 3-O-acetylated sphingenine.

A novel O-acetylated GM3 containing 3-O-acetyl 4-sphingenine was isolated with one having a non-acetylated base from transplanted rat glioma tissue. The presence and position of the acetyl group were estimated by one- and two-dimensional proton nuclear magnetic resonance, and fast atom bombardment-mass spectrometries. In addition, the O-acetyl GM3 showed higher immunological activity toward anti-melanoma antibody in the presence of non-acetylated GM3 in complement-dependent liposome lysis than did non-acetylated or acetylated GM3 alone in the liposome, suggesting enhancement of immunological reactivity of the intact tumor cells by a small amount of O-acetyl GM3.

[An adult case of lumbar intradural lipoma].

A 42-year-old female with intradural lipoma at the level of L1 is reported. She was admitted with a history of 32 months of lumbago, 25 months of pain of the left leg, and 12 months of motor weakness in the left leg. Neurologically, sensory impairment below the L1 dermatome of the left leg, and motor weakness of bilateral legs were noted on admission. CT demonstrated a low density mass with no contrast enhanced effect at the level of L1. MRI showed a mass with high signal intensity on the T1-weighted image, and low signal intensity on the T2. L1 laminectomy, and additional Th12 and L2 partial laminectomy were performed and the tumor was partially removed. The tumor was completely in the intradural space. Pathologically, the tumor consisted of mature adipose cells with normal vessels. Postoperatively, the epidural effusion at the operative area caused sensory impairment and motor weakness of the right leg. Finally, the patient came to be neurologically free of defects except for slight sensory diminution of the L4 dermatome of the left leg. In this case, total removal of the tumor was difficult because of adhesion between the tumor, the cauda equina and the conus medullaris. Postoperatively, neurological findings showed a marked improvement. The preoperative neurological deterioration in this case seemed to be associated with simple compression exerted on the nerves.

Photoaffinity labeling of lipoproteins in human cerebrospinal fluid with a heterobifunctional derivative of galactosylsphingosine.

The binding of a galactosylceramide (GalCer) analog to proteins in normal human cerebrospinal fluid (CSF) was examined by photo-affinity labeling using a radioiodolabeled 2-(p-azido-salicylamido) ethyl-1,3-dithiopropionate (ASD) derivative of galactosylsphingosine (GalSph) as a probe. The affinity-bound peptides appeared at 66, 36 and 28 kDa as radiolabeled bands. The latter two peptides, 36 and 28 kDa, were independently identified by immunostaining of the isolated peptides using biotinylated GalSph, and immobilized Avidin, and by immunoprecipitation of the photolabeled peptide, to apolipoproteins (ALPs) E and A-I, respectively. The direct binding of the GalCer analog to these apo-peptides suggested that the existence in the body fluids or the transfer between the fluids and cells of the glycolipid was related to high density lipoprotein (HDL) constructions.

Human blood group B-active ganglio-glycosphingolipid in rat glioma.

A human blood group B-active glycosphingolipid, belonging to the ganglio-series, was isolated from rat glioma cell line RG2 subcutaneous isografts. The oligosaccharide structure of the glycosphingolipid was completely characterized as Gal alpha 1-3(Fuc alpha 1-2)Gal beta 1-3GalNAc beta 1-4Gal beta 1-4Glc beta 1- 1'ceramide by NMR spectrometry, negative fast atom bombardment-mass spectrometry, sequential degradation by glycosidases and methylation analysis. Human blood group B antigenicity and the activity of this glycosphingolipid were confirmed by immunostaining on thin-layer chromatography and the inhibition of hemagglutination, respectively. Although the lipid has been detected in rat granuloma, bone marrow cells, spleen, thymus, ascites hepatoma cells and gastric mucosa, this is the first report of the occurrence of the B-active lipid in glioma.

[Ganglioneuroma in the cervical ganglion with neurofibromatosis-2: a case report].

The authors report a case of a 20 year old male with ganglioneuroma in the cervical ganglion. The patient was complaining of a mass in the neck on his right-hand side, and neurological examination revealed hypesthesia on the C5 area of the same side. Computed tomography and magnetic resonance imaging scan showed extradural dumbbell-shaped tumor from C3 to C5 levels. A two-staged operation was performed to remove the tumor totally, and a ganglioneuroma was diagnosed. After surgery, he complained of facial weakness and hearing disturbance on the left side. As MRI scan revealed bilateral cerebro-pontine angle tumors, he was diagnosed as having neurofibromatosis-2. This report may represent a rare case of ganglioneuroma with neurofibromatosis-2.

Cerebellar astrocytoma with repeated episodes of fourth ventricle isolation causing peritoneal shunt tube obstruction--case report.

A 9-year-old girl underwent total removal of a cerebellar astrocytoma complicated by hydrocephalus after postoperative meningitis, requiring a ventriculoperitoneal shunt. Five months later, headache, vomiting, and gait disturbance appeared and computed tomography detected enlarged fourth ventricle. A fourth ventriculoperitoneal shunt resulted in immediate relief of all symptoms. After 2 months, obstruction of the peritoneal tube required shunt reconstruction. This recurred three times in 8 months. At the last operation, tumor cells were detected in the cerebrospinal fluid and the substance clogging the tube. This suggested that the tumor had recurred and clogging by tumor cells had caused the repeated episodes of isolated fourth ventricle. Radiation therapy prevented further shunt obstruction and achieved remission of all signs and symptoms.

Cerebral salt wasting syndrome distinct from the syndrome of inappropriate secretion of antidiuretic hormone (SIADH).

Two cases with pituitary tumour developed postoperative hyponatraemia which was not caused by inappropriate secretion of antidiuretic hormone. The one case with non-functioning macro-adenoma showed severe hyponatraemia (116 mEq/l) on day 11 after trans-sphenoidal surgery in association with diabetes insipidus (DI). The patients was treated by aqueous pitressin and saline administration to control urinary output and keep positive salt balance at the same time. The other case with GH-producing macro-adenoma showed progressive negative sodium balance with the total loss of 644 mEq resulting in hyponatraemia of 133 mEq/l. This was corrected by additional salt intake. The plasma atrial natriuretic polypeptide (ANP), antidiuretic hormone (ADH) as well as aldosterone levels were normal in the latter case. These patients were considered to manifest primary salt wasting disorder, which should be clearly differentiated from the syndrome of inappropriate secretion of antidiuretic hormone (SIADH).

[Cauda equina neurinoma associated with intracranial hypertension--case report].

A case of cauda equina neurinoma associated with intracranial hypertension is reported. A 59-year-old female with a history of disturbed orientation was admitted. A neurological examination upon admission revealed the disorientation and gait disturbance. Superficial sensation under L3 was impaired. A computed tomographic(CT) scan presented the enlargement of ventricles and the slightly poor description of cerebral sulci. Since the patient had a high fever up to 40.1 C, meningitis was suspected. Cerebrospinal fluid revealed that cell count was only 2/3, while the protein concentration was markedly elevated (389mg/dl). Froin reaction was extremely positive and fibrin was observed. Based upon these findings, the spinal tumor was considered. Plain lumber film showed the posterior scalloping of the L5 and S1 vertebral bodies. Gd-DTPA enhanced MRI showed a high signal intensity area at the cauda equina. Diagnosed as a cauda equina tumor, the total resection of the tumor was performed via laminectomy of L3-S1. The tumor was involved with nerve filaments at the cauda equina. The pathological diagnosis was neurinoma. After the operation, her symptoms improved and a CT scan revealed the reduction of the ventricular size. However, the protein concentration of cerebrospinal fluid did not normalized.