Tanycytic ependymoma in association with neurofibromatosis type 2.

A rare case of tanycytic ependymoma associated with neurofibromatosis type 2 (NF2) is presented for the first time, with emphasis on its clinical course and histopathological features. A 30-year-old man had developed gait disturbance in his childhood, and harbored multiple tumors in spinal nerve roots, in the intradural extramedullary and intramedullary spinal cord. The spinal root tumor and intradural extramedullary tumor were histologically diagnosed as schwannoma and meningioma, respectively. Magnetic resonance imaging showed two intramedullary cystic lesions, one in the cervical and the other in the thoracic spine. Because his sensorimotor dysfunction in the lower extremities continued to worsen gradually, three of the multiple nodular tumors in the thoracic cystic lesion were removed. All three tumors were composed of eosinophilic piloid cells with modest nuclear pleomorphism. No Rosenthal fibers were found. A concentration of slender eosinophilic cellular processes surrounding the vascular wall was seen. Periodic acid Schiff and Masson trichrome-positive balloons were seen in the extracellular space. Detection of ependymal rosettes, although only few in number, led the diagnosis as a tanycytic ependymoma. Recognition of this ependymoma variant should be emphasized to avoid confusion with pilocytic astrocytoma or intramedullary schwannoma.

A case of thrombotic thrombocytopenic purpura refractory to plasma exchange.

We experienced a case of thrombotic thrombocytopenic purpura (TTP) finally relieved after 74 sessions of plasma exchange (PE). The patient was a 56-year-old male. In August 1999, he was examined in emergency because of brown urine and a lowered level of consciousness. As TTP was suspected according to the laboratory findings of abnormally high lactate dehydrogenase and total bilirubin, decreased platelet counts, and numerous fragmented erythrocytes, he was admitted to the ICU of our hospital. Immediately after admission, PE was started consecutively. Upon concomitant use of antiplatelet drugs and prostacyclin, the level of platelet counts recovered to 100,000/microl once, but decreased again. Thus, in addition to the PE, prednisolone and vincristine were administrated, which elevated the level of platelet counts to 200,000 to 300,000/microl. Since the erythrocyte fragmentation was noted frequently, PE was continued twice a week. From the 60th day of admission onward, however, his body temperature rose above 40 degrees C with a rapid increase of C-reactive protein. A blood culture detected methicillin-resistant Staphylococcus aureus (MRSA) which derived from a left lung abscess. During the course of anti-MRSA treatment, he presented acute renal failure and acute hepatic dysfunction, but survived because of the combined therapy. He was discharged on the 180th day of admission. These results suggest that a combined therapy of steroid and vincristine is effective to treat TTP refractory to PE, but careful attention should be paid to the complications caused by immunosuppression.

Double filtration plasmapheresis for the treatment of bullous pemphigoid: a three case report.

Bullous pemphigoid (BP) is an autoimmune disease caused by an antidermal basal lamina antibody. In recent years double filtration plasmapheresis (DFPP) has been reported to be an effective therapy for BP. We experienced 3 cases of BP treated by DFPP. DFPP resulted in an improvement in clinical symptoms and remission allowing a decrease in the required dose of corticosteroid. DFPP was found to be an effective treatment for all 3 patients without noticeable adverse events resulting from DFPP. From these results it is concluded that DFPP is worth considering as an option as treatment for BP patients who were unresponsive to conventional steroid therapy, those in whom corticosteroids should be reduced or discontinued because of complications such as diabetes mellitus and/or osteoporosis.

Plasma exchange for the treatment of human T-cell lymphotropic virus type 1 associated myelopathy.

A 55-year-old man was admitted to our hospital because of myelopathy. He had a history of chronic renal failure due to polycystic kidney disease at the age of 39, being treated by hemodialysis for 9 years with several blood transfusions for the treatment of renal anemia. After cadaver renal transplantation at the age of 48, he discontinued hemodialysis. At 50 years of age, he had pulmonary tuberculosis and tuberculous arthritis of the left elbow joint. He has experienced difficulty in walking since he was 48 years old, with mild dysuria. Gait disturbance gradually aggravated after that, and urinary retention was observed. When he was 55 years old, being human T-cell lymphotropic virus type-1 (HTLV-1)-positive in the serum and cerebrospinal fluid, he was diagnosed as having HTLV-1-associated myelopathy (HAM). As active steroid therapy was unapplicable because of the history of pulmonary tuberculosis and immunosuppression for transplanted kidney, a series of plasma exchanges (PE) was performed with fresh frozen plasma as a replacement fluid. After PE, dyskinesia of the left leg and dysuria subjectively and objectively improved. These results suggest that PE seems to be one of the therapeutic tools for the treatment of HAM.

Intracerebral hematoma due to ruptured nontraumatic middle meningeal artery aneurysm--case report.

A 77-year-old woman suddenly lost consciousness and presented with right hemiparesis. Computed tomography showed a large subcortical hematoma in her left frontal lobe associated with subarachnoid hemorrhage. The first impression was hemorrhage due to a ruptured aneurysm of the middle cerebral or the internal carotid artery on the left. Left internal carotid angiography showed no aneurysm or vascular anomaly, but back flow of contrast medium into the external carotid artery disclosed two saccular aneurysms arising from the anterior branch of the left middle meningeal artery (MMA). Emergent surgical intervention confirmed that the hematoma was due to ruptured MMA aneurysm. Nontraumatic MMA aneurysm should be recognized as a possible causative lesion of intracranial hemorrhage despite the extremely low incidence. External carotid angiography should be performed in patients with intracranial hemorrhage in whom no vascular cause can be detected in the internal carotid system.

A case of factor VIII inhibitor-positive acquired hemophilia treated by plasmapheresis.

We report on a case of factor VIII inhibitor-positive acquired hemophilia in which combined therapy of plasma exchange (PE) and steroids was effective. The patient, a 68-year-old man, had undergone hemodialysis since April 1998, due to chronic renal failure caused by diabetic nephropathy. The hemostasis of blood access sites gradually became difficult after the initiation of dialysis and the prolongation of activated partial thromboplastin time (APTT) (74.5 s), and a decrease in factor VIII (0.02%) and an abnormally high concentration of factor VIII inhibitor (111 U/ml) were found. Under the diagnosis of factor VIII inhibitor-positive acquired hemophilia, 3 consecutive PE were performed, followed by a large dose administration of gamma globulin. However, the effect of this therapy disappeared within 20 days. Then the PE therapy was performed again accompanied by pulse methylprednisolone therapy. After that, factor VIII inhibitor was suppressed and the patient's hemostatic defect continued to improve even after the reduction of the steroid dose. These results suggest that PE is very effective in treating factor VIII inhibitor-positive acquired hemophilia.

[Trigeminal neuralgia with the offending artery transfixing the trigeminal nerve: a case report].

We report a rare case of a patient manifesting trigeminal neuralgia with the offending artery transfixing the trigeminal nerve. A 67-year-old woman was admitted with typical episodes of trigeminal neuralgia on the right side. Magnetic resonance angiograms revealed the right superior cerebellar artery (SCA) compressing the nerve. Through a retromastoid suboccipital craniotomy on the right side, the right SCA was found to compress the root entry zone (REZ) and then penetrate the portio major of the trigeminal nerve. The nerve was dissected parallel to the axis, 7 mm distally from the transfixing point to facilitate transposition of the SCA. In addition to anchoring the distal portion of the SCA to the tentrium, a prosthesis was interposed between the proximal portion and the pons to secure the decompression. The pain was completely relieved immediately after operation with no neurological deficit. Procedures, such as the longitudinal rhizotomy employed in this case, must be carried out for microcompression, in response to individual anatomical variations in neurovascular structures.

Massive subependymal hemorrhage caused by an occult vascular malformation--two case reports.

Two patients presented with massive subependymal hemorrhage caused by vascular anomalies occult to angiography, computed tomography (CT) scanning, and magnetic resonance (MR) imaging. A 47-year-old male initially presented with a headache and became comatose 3 weeks later. CT and MR imaging demonstrated a massive hematoma in the right caudate head projecting into the lateral ventricle. A 60-year-old male sustained heaviness of his left extremities 8 days prior to admission and his condition gradually deteriorated. Neuroimaging revealed a right thalamic hematoma adjacent to the lateral ventricle. Cerebral angiography, CT, and MR imaging failed to detect any vascular anomaly. The hematomas increased in size gradually and were surgically explored. The histological diagnosis was arteriovenous malformation for the first case and cavernous angioma for the second case that had ruptured repeatedly. These cases were unique in the unusual location in the subependyma, and diagnostic neuroimaging modalities could not disclose the vascular anomalies, and the hematomas enlarged progressively to become critically symptomatic. Surgical intervention is mandatory for mass reduction and correct diagnosis of such lesions, with favorable outcome as long as the surgery is not delayed until too late.

Combined hyperactive dysfunction syndrome of the cranial nerves: trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia: 11-year experience and review.

OBJECTIVE: A pathological condition caused by vascular compression at the root entry/exit zone of the cranial nerves is designated hyperactive dysfunction syndrome (HDS) of the cranial nerves. Patients with HDS who exhibited a combination of trigeminal neuralgia (TN), hemifacial spasm (HFS), and/or glossopharyngeal neuralgia were retrospectively reviewed, to study the incidence, etiological factors, and demographic characteristics for this combined HDS group. METHODS: Medical and surgical records were analyzed for 41 patients with combined HDS, of 1472 consecutive patients with HDS who were treated between 1984 and 1994. RESULTS: The combined HDS group accounted for 2.8% of all patients with HDS; 19 patients (1.3%) exhibited bilateral symptoms, i.e., 14 cases of TN, 3 of combined TN and HFS, and 2 of HFS. Twenty-two patients (1.5%) exhibited ipsilateral symptoms, i.e., 19 cases of TN and HFS and 3 of TN and glossopharyngeal neuralgia. Excluding three patients whose symptoms were associated with brain tumors or arteriovenous malformations, this patient group was older (63.2 versus 55.3 yr, P = 0.0009) and exhibited an increased percentage of associated hypertension (47.4 versus 17.5%, P = 0.000008), with a female predominance (86.8 versus 71.3%, P = 0.07), compared with the single HDS group. Thirty-six of these patients underwent a total of 61 microvascular decompression procedures, with favorable outcomes. The offending vessels were similar to those in single HDS, which were usually conventional and multiple. CONCLUSION: The associated etiological factors for vascular compression syndromes were more evident in the combined HDS group than in the single HDS group. Progressive arteriosclerotic vasculoarchitectural changes of the vertebrobasilar system, accelerated by aging and hypertension, bring about the development of combined HDS, with a remarkable female predominance.

Mechanism of intraocular pressure elevation during hemodialysis.

PURPOSE: To detect the mechanism of intraocular pressure elevation during hemodialysis. METHODS: We measured intraocular pressure, as well as serum osmolality and plasma CO2 pressure, every 30 min during hemodialysis, in 5 eyes with severely compromised aqueous outflow facility (Group A) from 4 renal failure patients. The same measurements were repeated on the same eyes using intravenous hyperosmotic Glyceol to prevent a rapid change in serum osmolality. We also measured the same parameters on 8 eyes with normal aqueous outflow facility (Group B) from 5 patients. The mean +/- SE of percent changes in each parameter was used for the statistical analysis. RESULTS: In Group A, the mean percent change of intraocular pressure increased significantly after 90 min, with the exception of the change at 180 min. The mean percent change of serum osmolality decreased significantly after starting dialysis. A negative correlation in the mean percent change of intraocular pressure with serum osmolality was detected (r = -0.759, r < 0.0001). The administration of intravenous hyperosmotic agent prevented significant changes in not only serum osmolality but also intraocular pressure. In Group B, the mean percent change in intraocular pressure showed no significant difference at any time, although the change in serum osmolality decreased significantly. CONCLUSION: A remarkable rise in intraocular pressure occurs during hemodialysis in eyes with an impaired aqueous outflow, when serum osmolality decreases rapidly.

Chordoid meningioma in a child. Case report.

A case of chordoid meningioma occurring in a 15-year-old girl is presented. The patient manifested seizures as the initial symptom and subsequently exhibited subclinical microcytic hypochromic anemia. The tumor, located in the falcotentorial region and associated with diffuse edema, was totally resected. On histological examination of the surgical specimen, the clustering pattern of partly vacuolated cells in the mucoid stroma mimicked chordoma; however, positive staining of individual cells for vimentin and epithelial membrane antigen led to a diagnosis of meningioma. Interestingly, the tumor cells were surrounded by a periodic acid-Schiff- and type IV collagen-positive substance. Electron microscopy demonstrated a strikingly dense and thick basal lamina. The patient's microcytic hypochromic anemia disappeared after the tumor was removed. Both the clinical and pathological features of this case resemble those of chordoid meningioma, a rare meningioma variant.

A large thrombosed superior cerebellar artery aneurysm: a case report.

UNLABELLED: MATERIAL AND RESULT: A large thrombosed aneurysm arising from the distal superior cerebellar artery was successfully resected. DISCUSSION AND CONCLUSION: An aneurysm in this location is very rare. Accumulation of intraoperative hemodynamic data may be useful in evaluating the capacity of collateral circulation.

Hemifacial spasm in childhood and adolescence.

Hemifacial spasm (HFS), a hyperactive dysfunction of the facial nerve, is rarely seen in young people. Between 1984 and 1994, we treated 924 patients with HFS by microvascular decompression at our institution. Of these, 8 (0.9%) were younger than 30 years. In most of the older patients with HFS, the offending artery which compresses the root exit zone was elongated, redundant, and focally arteriosclerotic as a result of hemodynamic effects due to aging or hypertension. On the other hand, the offending artery did not exhibit such characteristic changes of the vasculature in children and adolescents with HFS. In all of the young patients who underwent initial microvascular decompression at our clinic, the arachnoid membrane around the facial nerve was thickened and encased the artery, resulting in compression of the root exit zone of the facial nerve. Such thickening of the arachnoid surrounding the offending vessel may play an important role in the pathogenesis of HFS by trapping and encasing the artery to compress the root exit zone, particularly in the young patients.

[Treatment of intractable postherpetic neuralgia and blepharospasm: intraneural injection of adriamycin].

Adriamycin, an anthracycline antineoplastic agent, can swiftly be transported to the sensory or somatic motor neurons by way of axoplasmic transport when injected into the subepineurium of the trigeminal nerve or sciatic nerve in experimental animals, and is consequently able to induce degeneration of the neurons without any systemic side effects. Intraneural injection of this agent was carried out for the treatment of a total of 22 patients presenting with intractable neural dysfunction (12 with neuralgia, including 7 with post-herpetic neuralgia and 10 with facial dystonia). The nerve which innervated the affected site was exposed under local anesthesia and approximately 10-60 microliters of 1-20% adriamycin was injected into the subepineurium. Results of the treatment after average follow-up periods of 21.5 months were as follows: Out of 12 patients with neuralgia, good results were obtained in 2 cases (16.7%), fair results in 6 cases (50.0%) (overall effective rate 67.7%). There were no changes in symptoms in 4 cases (33.3%). Out of 10 patients with facial dystonia, good results were obtained in 2 cases (20.0%), fair in 2 cases (20.0%) (overall effective rate 40.0%), and no changes in symptoms in 6 cases (60.0%). No major complications were encountered during these procedures and, once symptoms had disappeared after the treatment, no recurrence of symptoms was experienced. This method clearly differs from other various kinds of simple peripheral neurotomy, since transection of the peripheral nerve does not cause any, destructive changes in the sensory ganglion or motor nucleus and, hence, symptoms may recur.(ABSTRACT TRUNCATED AT 250 WORDS)

Coexistence of intracranial meningioma and primary malignant lymphoma--case report.

The authors present a case of multiple primary intracranial tumors of different cell types. A 62-year-old female presented with symptoms suggestive of an intracranial mass lesion. Computer tomographic scans revealed two lesions, one in the right temporal lobe and one in the posterior fossa. Both tumors were approached in one operation. The right temporal tumor was diagnosed as primary malignant lymphoma, and the right posterior fossa tumor as meningioma. Phacomatosis was ruled out as a possible etiology. Multiple primary intracranial tumors of different cell types are rare, and this is the first report of coexistent intracranial meningioma and malignant lymphoma.

Infantile myofibromatosis with a solitary lesion in the skull--case report.

The term "infantile myofibromatosis" was coined in 1981 to describe a rare type of soft-tissue tumor in infants. Solitary lesions are usual, but extremely rare in the skull. An infantile case involving a lesion in the left parietal bone is described. The patient was a 6-month-old girl admitted with a mass measuring 2 x 2 cm in the left parietal region. Skull x-rays showed an osteolytic lesion with a sclerotic margin in the parietal bone. Computed tomographic (CT) scans revealed a low-density mass with homogeneous enhancement. The tumor adhered to the dura and had destroyed the left parietal bone. Histological examination disclosed spindle-shaped cells arranged in short bundles and abundant vasculature. Phosphotungstic acid hematoxylin staining revealed longitudinal fibrils resembling myofibroblasts. This is the first report in which CT findings are described in a case of infantile myofibromatosis with a solitary lesion occurring in the skull.