[A case of possible immunoglobulin G4-related disease (IgG4-RD) with retroperitoneal fibrosis and central diabetes insipidus due to infundibulohypophysitis].

We report a case of possible immunoglobulin G4-related disease(IgG4-RD)that resulted in complications such as retroperitoneal fibrosis and infundibulohypophysitis. The patient was a 72-year-old male who presented with polyuria and polydipsia. Magnetic resonance imaging(MRI)revealed a thickened pituitary stalk and contrast enhancement with gadolinium. T1-weighted imaging revealed that the posterior pituitary high-signal zone had disappeared. Central diabetes insipidus was diagnosed on the basis of results of the hypertonic saline test. In addition, pressure due to retroperitoneal fibrosis resulted in hydronephrosis and elevated serum IgG4 levels. Because it was determined that the patient could have IgG4-RD, he was administered prednisolone, following which a decrease in the size of the pituitary stalk and retroperitoneal fibrosis was observed. IgG4-RD is characterized by elevated serum IgG4 levels and the infiltration of IgG4-positive plasma cells into various organs, including the central nervous system. Recently, IgG4-RD research teams organized by the Ministry of Health, Labour and Welfare established guidelines for the diagnosis of IgG4-RD. According to these guidelines, this case would fall under the category of "possible IgG4-RD." This case suggested that when infundibulohypophysitis is detected by neuroradiology, further investigation into the possibility of IgG4-RD should be recommended.

Xanthogranuloma of the sellar region--case report.

A 55-year-old woman presented with a rare xanthogranuloma of the sellar region after complaining of severe headache and visual disturbance 3 months previously. Clinical examination showed she was alert with early signs of bitemporal hemianopsia. Endocrinological examination revealed hypopituitarism. Magnetic resonance imaging showed an intrasellar mass extending into the suprasellar region and compressing the optic chiasma, which appeared homogeneously hyperintense on T(1)-weighted images. Endonasal transsphenoidal resection of the tumor was performed. Histological examination disclosed granulomatous tissue with cholesterol clefts, hemosiderin deposits, fibrous tissues, and macrophages containing rich fat. The histological diagnosis was xanthogranuloma of the sellar region. Her visual symptoms recovered postoperatively although the hypopituitarism remained. Xanthogranuloma of the sellar region is rare, but must be considered in the differential diagnosis of tumors of the sellar region.

Controversy of surgical treatment for severe cerebellar infarction.

Treatment for severe cerebellar infarction has been controversial. Clinical outcomes of patients with external ventricular drainage (EVD) and decompression as the first treatment for the infarction were compared. A total of 25 patients with severe cerebellar infarction were subdivided into two groups to compare outcome of the group (group A) with EVD with that of the group (group B) with decompressive surgery as the first surgery. There was no statistically significant difference in age between group A with 71 +/- 6 years and group B with 61 +/- 15 years. The preoperative status was Glasgow Coma Scale (GCS) score 6 in all the patients in group A. It was GCS score 4 in one patient, GCS score 6 in 9 patients, GCS score 7 in 8 patients, and GCS score 9 in two patients in group B. The preoperative neurologic background was almost the same for both groups. In group A, one patient had a good recovery. However, 3 patients became severely disabled and one patient died. In group B, 10 patients had a good recovery and 6 patients became moderately disabled, although two patients were disabled and two patients died. The outcome was good in one patient of group A and in 16 patients of group B, although it was poor in 4 patients each in both groups. Patients in group B had a significantly better prognosis than those in group A. No clear evidence of surgical indications for EVD or suboccipital decompression from neurologic signs or symptoms and from neuroimaging has been reported. Our results seem to suggest that pre-emptive suboccipital decompression with or without resection of necrosis is warranted in the patients with severe cerebellar infarction.

Delayed onset massive oedema and deterioration in traumatic brain injury.

A 52-year-old man fell from standing and a computed tomography (CT) scan revealed traumatic intracerebral haematoma and subarachnoid haemorrhage in the temporal cortex. He was treated without surgery and discharged. On day 30 after the accident, he had no neurological deficit. On day 37 he complained of headache and urinary incontinence, and on day 39 he was hospitalized due to progressive neurological deterioration (reduced conciousness, dilated pupils, and left hemiplegia). A CT scan revealed a diffuse low-density in the right cerebral hemisphere with marked midline shift. Emergency decompressive craniectomy and right temporal lobectomy were performed. Angiography after surgery revealed moderate vasospasm in the right middle and anterior cerebral arteries. The patient remained severely disabled. Delayed onset neurological deterioration can be caused by brain oedema and vasospasm after traumatic brain injury, despite an intervening period of improvement.

Treatment of cerebral vasospasm with intra-arterial fasudil hydrochloride.

OBJECTIVE: Symptomatic cerebral vasospasm is a major cause of disability and death in patients with aneurysmal subarachnoid hemorrhage. The purpose of this study was to determine the efficacy of intra-arterial infusion (IAF) of fasudil hydrochloride for symptomatic vasospasm in terms of neurological improvement and the angiographic features of cerebral vessels. METHODS: A consecutive series of 23 patients underwent IAF therapy for the treatment of symptomatic vasospasm after subarachnoid hemorrhage. Angiographic vasospasm was assessed before and after the IAF treatment. Immediate clinical effect was assessed within 24 hours after the IAF treatment, and the Glasgow Outcome Scale was used to evaluate late clinical outcome at 3 months after the onset. RESULTS: IAF treatment was performed on 34 occasions for 23 patients (16 women, 7 men). Angiographic improvement was observed on all occasions (100%), and immediate clinical improvement was observed on 15 occasions (44.1%). At 3-month follow-up, 15 patients (65.2%) showed good recovery or moderate disability on the Glasgow Outcome Scale. CONCLUSION: IAF therapy was effective in dilating spastic arteries, and clinical improvement was observed in approximately half of the fasudil hydrochloride infusions. Our findings suggested that the IAF therapy was safe and effective for patients with symptomatic vasospasm after subarachnoid hemorrhage.

Cerebral infarction in a 24-year-old pilot.

Ischemic stroke is a rare event in young adults. We report on a 24-yr-old pilot with cerebral infarction of undetermined etiology, temporally associated with chain smoking. The patient exhibited dysphasia, stupor (confused consciousness), and right facial-nerve palsy. Computed-tomography revealed a low-density area in the left insular cortex. Cerebroangiography showed severe stenosis in a branch of the left middle cerebral artery. After admission, the patient made a rapid and uneventful recovery within 72 h. MRI showed an area of hyperintensity on T2-weighted images 2 mo after the attack. Based on the hyperintense area on FLAIR (fluid attenuated inversion recovery sequence) images obtained in MRI performed 10 mo after the attack, we diagnosed a cerebral infarction. In the Japan Air Self-Defense Force, cerebral infarction is an aeromedically disqualifying condition. However, in the evaluation 2 mo after the attack, differentiation from reversible ischemic neurological deficit was difficult. We discuss the criteria used for diagnosis and the risk factors for cerebral infarction in young adults, as well as the aeromedical disposition of young pilots.