1/7. Idiopathic hypertrophic cranial pachymeningitis: clinicoradiological spectrum and therapeutic options.OBJECTIVE: Idiopathic hypertrophic cranial pachymeningitis is a rare disease, of undetermined pathogenesis, that is characterized by inflammation and fibrosis of the dura mater. methods: We encountered six patients with idiopathic hypertrophic cranial pachymeningitis and analyzed their clinical presentations, radiological findings, and treatment. RESULTS: In the six patients, the main manifestations were cranial nerve palsies and headache. Three associations were present, namely optic neuropathy, tolosa-hunt syndrome, and diabetes insipidus. gadolinium-enhanced magnetic resonance imaging was diagnostic, showing intense dural enhancement in a linear or nodular pattern. The responses to corticosteroid therapy were better for patients who exhibited linear, rather than nodular, dural enhancement. For one patient, surgical decompression of the superior orbital fissure provided lasting relief. The course of the disease followed one of three patterns, i.e., sustained remission, relapse with corticosteroid independence, or relapse with corticosteroid dependence. pulse corticosteroid therapy provided significant relief, while reducing the daily corticosteroid requirement and avoiding side effects, for a corticosteroid-dependent relapsing patient. CONCLUSION: Idiopathic hypertrophic cranial pachymeningitis exhibits varied clinical courses. It is important to prevent irreversible cranial neuropathy during the active phase of the disease, using daily administration of corticosteroids, pulse corticosteroid therapy, or surgical decompression.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Treatment of T-prolymphocytic leukemia with nonmyeloablative allogeneic stem cell transplantation.AIM: T-prolymphocytic leukemia (T-PLL) is a rare disease of the elderly characterized by a high white blood cell count and organomegaly, and is currently incurable. Our aim was to elicit graft-versus-leukemia reactions in a patient with T-PLL. methods: A 52-yr-old woman with refractory T-PLL underwent a nonmyeloablative regimen followed by allogeneic peripheral blood stem cell transplantation (a "minitransplant") from her HLA-matched sibling. RESULTS: There was no treatment related toxicity other than neutropenia. Engraftment was successful. The patient experienced no graft-versus-host disease (GVHD) at any time but, on day 84 after transplantation, had a relapse in the central nervous system. Despite infusion of donor lymphocytes and intralumbar chemotherapy, she died on day 157 of systemic disease. CONCLUSION: The reasons why treatment may have failed are discussed (nature of disease, disease progression, treatment schedule).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. recurrence of hypertrophic spinal pachymeningitis. Report of two cases and review of the literature.Hypertrophic spinal pachymeningitis (HSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. The authors report two cases of recurrent HSP and review the English- and Japanese-language literature focusing on the recurrence of HSP. In the first case, a man who presented at 67 years of age with lower-extremity numbness, gait disturbance, and bladder dysfunction experienced two recurrences of HSP during the 11 years of follow up after his initial laminectomy. Both recurrences were successfully treated with laminoplasty and duraplasty. Three years after his last surgical procedure, he was still able to walk with the aid of a walker. In the second case, a man who presented at 62 years of age with lower-extremity numbness and gait disturbance was initially treated successfully with steroid pulse therapy. Approximately 8 months after his initial presentation, his symptoms recurred. He underwent laminoplasty and duraplasty. At the 2.5-year follow-up examination, he had only mild neurological deficits and was still able to walk unaided. To explore possible causes of recurrence, the authors searched the English- and Japanese-language literature for cases of HSP. Of the 96 cases identified, 11 were recurrent. Data on the presence or absence of inflammatory signs were available for 84 patients. A chi-square analysis revealed a significantly increased rate of recurrence for patients who had at least one positive inflammatory sign before surgery (six [20%] recurrent cases of 30) compared with those who had no positive inflammatory signs before surgery (two [3.7%] recurrent cases of 54) (p < 0.05). The authors conclude that HSP recurrence occurs because of active inflammation of the dura before surgery and the influence of chronic inflammation, including residual arachnoiditis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Eosinophilic meningitis and the hypereosinophilic syndrome. Case report and review of the literature.The hypereosinophilic syndrome is a rare disease characterized by infiltration of numerous tissues with eosinophils, leading to organ dysfunction and eventual death. Although patients often have neurologic symptoms and occasionally have been found to have central nervous system infiltration with eosinophils, a well-documented case of the hypereosinophilic syndrome has not been previously reported in association with cerebrospinal fluid eosinophilia and biochemical evidence for meningitis. Such a case is described and the literature dealing with brain and meningeal involvement in the hypereosinophilic syndrome is reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. A case of benign recurrent meningitis of Mollaret.A 72-year-old woman, previously in good health, suffered for 2 years from attacks of a complex neuropsychiatric syndrome lasting from 2 days to 4 weeks. These episodes, which were followed by intervals of nearly complete recovery, were usually marked by fever, headache, mental confusion, vomiting ataxia, anisocoria, epileptic seizures and stiffness of the neck. Neuroradiological investigation revealed only slight ventricular dilatation. In 13 CSF examinations, there was reduced glucose, increased protein, considerable rise of the IgG index and constant oligoclonal bands. There was also an increase of polymorphonuclear leucocytes, lymphocytes or epithelial-like cells. As all microbial, viral or mycotic tests were negative and the patient recovered, the case was considered to be benign recurrent meningitis of Mollaret. The nosological position of this rare disease, as well as its probable pathogenetic mechanisms, are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. The cytodiagnosis of meningeal involvement in familial haemophagocytic lymphohistiocytosis.The cytological appearances of the cerebrospinal fluid (CSF) in two cases of familial haemophagocytic lymphohistiocytosis (FHL) are described. The presence of numerous lymphocytes and immature macrophages in the CSF, some of which showed lymphohistiocytosis, was indicative of meningeal involvement. The appearance of large numbers of immature macrophages indicated rapid deterioration and death within a few weeks. Furthermore, some CSF samples taken at times when the patients were asymptomatic contained suspicious cells, indicating asymptomatic persistence of the meningeal lesions. Thus, cytological examination of CSF can assist with the management of patients with this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Hypertrophic cranial pachymeningitis in a patient with aplastic anemia.We report on a 13-year old girl with severe aplastic anemia and hypertrophic cranial pachymeningitis. She was admitted to our hospital with severe headache and vomiting. A computerized tomographic (CT) scan of the brain on the third day of symptoms showed a hyperdense area in the tentorial region. Magnetic resonance imaging (MRI) showed iso-intensity in the same tentorial region in T1- and T2-weighted images, and gadolinium enhancement of this region suggested a thickened dura mater. Initially, a diagnosis of subdural or subarachnoid hemorrhage was made. Since her platelet count was low (3000/microl) making the patient a poor-risk candidate for surgery, and the area was limited to the dura mater, conservative therapy, including glycerol administration and platelet transfusion, was carried out. Despite clinical improvement 10 days after admission without specific therapy, the iso-intense region on the left side of the tentorial region remained unchanged on MRI. On the other hand, the iso-intense area on the right side of the tentorial region became hyperdense on T1-weighted MRI images and was also enhanced by gadolinium. cerebrospinal fluid findings were normal except for slightly elevated protein at 62 mg/dl. A diagnosis of hypertrophic cranial pachymeningitis of the tentorial dura mater with hemorrhage on the right side was made. Although hypertrophic cranial pachymeningitis is a rare disease, it must be considered in the differential diagnosis of severe headache in a case of aplastic anemia.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |