[Idiopathic hypertrophic pachymeningitis: a diagnostic dilemma]. / Paquimeningitis hipertrófica idiopática: un dilema diagnóstico.

INTRODUCTION: Idiopathic hypertrophic pachymeningitis (IHP) is a rare fibrosing inflammatory process of unknown pathogenesis that causes diffuse thickening of the duramater. In spite of the fact that this pathological entity has been linked to specific etiological agents such as syphilis, tuberculosis, mycosis and sarcoidosis, In recent years there has been an increase in the number of patients with IHP of undetermined cause. CASE REPORT: 58 year-old female with a long date history of oppressive headaches, frontally located at first, and later generalized, accompanied by weakness and asthenia; urinary incontinence, dizziness, vomiting, and gait ataxia. Physical examination revealed bilateral cerebellar signs, and optic atrophy. Magnetic resonance displayed a diffuse thickening of the falx cerebrii, and cerebellar tentorium, hypointense in T1 and T2- weighted images, and markedly enhanced following administration of intravenous Gd-DTPA. A biopsy obtained from the falx revealed conspicuous fibrosis, and focal chronic inflammatory mononuclear infiltrate. Complementary exams carried out in order to reveal a specific etiologic agent were negative. CONCLUSION: Due to the variable clinical presentation, and the difficulty to establish the etiology of the pathological process, to make the diagnosis of IHP usually represents a true dilemma. This diagnosis is often made by exclusion.

[Cerebrovascular disease: its relationship with cardiovascular and renal pathology in 134 clinical autopsies]. / Enfermedad cerebrovascular: su relación con cardiovasculopatías y nefropatías en 134 necropsias clínicas.

OBJECTIVE: To establish the relationship between cardiopathy and chronic nephropathy, and cerebral vascular pathology in clinical necropsies performed in adult patients. MATERIAL AND METHODS: The protocols of 861 clinical autopsies done during the lapse 1990-2000 were reviewed, of these, 134 cases with diagnosis of cerebrovascular disease (CVD) were selected. Analyzed features included: neuropathological study of CVD, renal and cardiovascular pathology, and cause of death. RESULTS: CVD represented 15.5% of all autopsies done during the study period. Ischemic CVD constituted 56.7% of the cases, whereas cases of hemorrhagic CVD accounted for 43.3% of the total. Causes included arterial hypertension (33%), atherosclerosis (19%), emboli and vascular malformations (13% each, respectively), coagulopathies (8%), angeiitis (4%), tumors (1%), and unknown origin (11%). Ischemic CVD was caused by atherosclerosis in 34.2% of the cases, lacunar infarcts in 32.8%, of embolic cause in 14.4% of the cases, angeiitis in 1.3%, and of unknown origin in 17.1% of the cases. Hemorrhagic CVD presented as intraparenchymal hematoma in 50% of the cases, as sub-arachnoid hemorrhage in 28%, as disseminated petechial hemorrhages in 19%, and intraventricular in 3% of the cases. Ischemic CVD was associated with hypertensive cardiopathy in 86.5% of the cases, and with nephrosclerosis in 51.3%, whereas hemorrhagic CVD was seen associated with the same pathological entities in 86.2% and 29.3% of the cases, respectively. Cerebral herniation was the cause of death in 2.6% of the patients with ischemic CVD, and in 74.1% of the patients with hemorrhagic CVD. CONCLUSIONS: CVD was associated with a high frequency of intercurrent pathological processes, namely hypertensive cardiopathy, atherosclerosis, nephroscleroisis, and other chronic nephropathies, that eventually interact, and constitute well known predisposing and/or concomitant factors to the cerebrovascular event