Neuropathologic substrates of ischemic vascular dementia.

Ischemic vascular dementia (IVD) is a relatively uncommon entity, in the course of which multiple ischemic brain lesions result in progressive cognitive and memory impairment. Ischemic brain lesions may also aggravate the neuropsychologic deficit of Alzheimer disease (AD). In this review we summarize our experience based upon autopsy examination of the central nervous system in 20 patients (age range 68-92 years) enrolled in a longitudinal investigation of structural, neurochemical, functional neuroimaging, and neuropsychologic components of IVD, especially dementia associated with cerebral microvascular disease. While cystic infarcts were present in the CNS of 5 patients, the most commonly observed neuropathologic abnormalities were lacunar infarcts and microinfarcts--both types of lesion were encountered in over half of patients' brains. Evidence of (remote) hippocampal injury was found in 11/20 patients. Severe atherosclerosis and arterio/ arteriolosclerosis were both associated with the occurrence of multiple lacunar infarcts. Pronounced cerebral amyloid angiopathy (CAA) was noted in a single patient, who also showed other microscopic changes of severe AD. While fairly unusual as a nosologic entity, IVD appears to correlate with widespread small ischemic lesions distributed throughout the CNS. We furthermore propose an approach to quantifying the burden of ischemic vascular and parenchymal disease that may be associated with a dementia syndrome. A brief review of neuropathologic features of vascular dementia (both familial and sporadic) is presented.

Aggressive enterogenous cyst with extensive craniospinal spread: case report.

OBJECTIVE AND IMPORTANCE: Considered benign and malformative in nature, enterogenous cysts are predominantly spinal lesions. Although recurrences have been reported, especially in the rare intracranial examples, craniospinal dissemination has never been described. CLINICAL PRESENTATION: We report a 63-year-old woman who presented 16 years previously with a histologically typical enterogenous cyst of the cerebellum. Fourteen years after its incomplete excision, numerous supratentorial, infratentorial, and spinal subarachnoid cysts were detected. A histological examination revealed multiple enterogenous cysts without evidence of malignancy. Ki-67 (MIB-1) proliferative indices were less than 1% in all except one specimen, wherein it was 4%; immunohistochemistry demonstrated rare p53 protein overexpression. INTERVENTION: During the last 2 years, four subtotal resections of spinal enterogenous cysts have been performed for progressive paraplegia, pain, paresthesia, and bladder dysfunction. Considering the multiple recurrences and progressive neurological deficits, craniospinal radiation therapy was given. CONCLUSION: To our knowledge, this is the first report of widespread craniospinal dissemination associated with the incomplete resection of an enterogenous cyst. Although a rare complication, it emphasizes the advantages of early, aggressive surgery.

Multiple cystic encephalomalacia of infancy: computed tomographic findings in two cases with associated intracerebral calcification.

Two initially healthy infants developed acute encephalopathic illnesses characterized by stupor, seizures, cerebrospinal fluid (CSF) erythrocytic and monocytic pleocytosis, increased CSF protein, and decreased CSF glucose and progression to chronic decerebration. In one case, herpes simplex virus was recovered from cutaneous lesions. The initial computed tomography (CT) scan revealed widespread subcortical increased attenuation with further increase after contrast medium injection and patchy areas of decreased attenuation in the deep cerebral white matter. Subsequent CT scans demonstrated progressive cortical calcifications and persistence of low attenuation areas. Autopsy revealed multiple cystic encephalomalacia. The second infant had similar clinical, CSF, and CT findings but remains in a chronic decerebrate state at 14 months of age. The CT abnormalities seen in these patients have not been encountered in any of 13 other infants with the clinical diagnosis of meningitis or encephalitis. Multiple cystic encephalomalacia of infancy is a rare condition with a uniformly bleak prognosis. Computed tomography may prove useful in the early diagnosis, thereby aiding clinicians in counseling and in the acute and long-term management of patients with this lesion.