A new hereditary disease with progressive dementia and polycystic osteodysplasia: neuroradiological analysis of seven cases
| Autor: | M. Iivanainen, H. P. A. Hakola |
|---|---|
| Rok vydání: | 1973 |
| Předmět: |
Adult
Male Myoclonus medicine.medical_specialty Pathology Chondrodysplasia Punctata Neurology medicine.medical_treatment 03 medical and health sciences 0302 clinical medicine Atrophy Cortex (anatomy) Basal ganglia medicine Aphasia Humans Radiology Nuclear Medicine and imaging Pneumoencephalography 030304 developmental biology Neuroradiology 0303 health sciences Epilepsy business.industry Amyotrophic Lateral Sclerosis Cerebral degeneration Brain Electroencephalography Syndrome medicine.disease Hand Radiography medicine.anatomical_structure Agnosia Dementia Female Neurology (clinical) Neurosurgery Cardiology and Cardiovascular Medicine business 030217 neurology & neurosurgery |
| Zdroj: | Neuroradiology. 6(3) |
| ISSN: | 0028-3940 |
| Popis: | Seven adult patients, 4 males and 3 females aged from 32 to 41 years, suffering from a new hereditary disease characterized by progressive dementia and polycystic osteodysplasia were studied neuroradiologically. Pneumoencephalography revealed diffuse generalized atrophy both in the supratentorial and infratentorial spaces, consisting of central periventricular brain tissue, cortex, and brain stem. The atrophy was most striking in the frontal and parietal areas. In one case, intracranial calcifications were found in the basal ganglia. Carotid angiography, performed in one case, did not reveal any changes indicative of a vascular origin. There appears to be a remarkably positive correlation between the degrees of clinical and PEG findings. The findings suggest progressive cerebral degeneration. PEG is likely to be of decisive aid in the early diagnosis of cerebral changes. |
| Databáze: | OpenAIRE |
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