[Show abstract][Hide abstract] ABSTRACT: The hippocampus along with other structures of the medial temporal lobe plays an important role in the process of learning and memory consolidation. Bilateral hippocampal lesions lead to persistent anterograde amnesia while unilateral damage results in milder, content-specific forms of amnesia. Hippocampus may be affected by an acute or chronic pathologic process from a wide spectrum of neurological disorders.
A 61-year-old female patient with a long history of hypertension, glucose intolerance, hypercholesterolemia and depression was hospitalized for acute anterograde amnesia, which led to repeated excessive drug taking. By further examinations that included laboratory tests, electroencephalography, Doppler sonography of intra- and extracranial vessels and neurovisualization methods [multislice computed tomography (MSCT) and magnetic resonance imaging (MRI) of the brain] bilateral hippocampal ischemia that occurred at different times was detected. Cognitive and neuropsychological evaluation revealed an isolated severe damage of episodic memory with the inability of retention of new information which persisted at the control examination three months later. The assumed mechanism of occurrence of ischemia in this case could be arterioarterial embolism.
Although ischemic stroke is one of the most common neurological diseases, ischemic stroke of the hippocampus is rare, the isolated bilateral presentation with clinical signs of severe amnestic syndrome in particular. Timely recognition and modern therapeutic approach could have a favorable impact on the recovery from severe neurological, cognitive deficit. It could be suggested that in patients with the clinical image of acute anterograde amnesia and vascular risk factors the MSCT examination of the brain with computed tomography perfusion and angiography is performed immediately upon hospitalization.
[Show abstract][Hide abstract] ABSTRACT: Fast and precise diagnostics of the disease from the large group of adult leukoencephalopathy is difficult but responsible job, because the outcome of the disease is very often determined by its name. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by the mutation of Notch 3 gene on chromosome locus 19p13. Beside the brain arterioles being the main disease targets, extracerebral small blood vessels are affected by the pathological process. Clinically present signs are recurrent ischemic strokes and vascular dementia. CADASIL in its progressive form shows a distinctive pattern of pathological changes on MRI of endocranium. The diagnosis is confirmed by the presence of granular osmiophilic material (GOM) in histopathological skin biopsies.
Two young adult patients manifested ischemic strokes of unknown etiology, cognitive deterioration, migraine and psychopathological phenomenology. MRI of endocranium pointed on CADASIL. Ultrastructural examination of skin biopsy proved the presence of GOM in the basal lamina and near smooth muscle cells of arteriole dermis leading to CADASIL diagnosis. The presence of GOM in histopathological preparation is 100% specific for CADASIL. The patients were not searched for mutation in Notch 3 gene on chromosome 19, because some other leukoencephalopathy was disregarded.
Suggestive clinical picture, distinctive finding of endocranium MRI, the presence of GOM by ultrastructural examination of histopathological skin biopsies are sufficient to confirm CADASIL diagnosis.