Alexander's disease with Rosenthal fibre formation in an adult.
نویسندگان
چکیده
The case of a woman who had a mild mental and physical handicap all her life is described. At the age of 39 years she began a progressive deterioration which terminated with her death after a year. CT scanning revealed basal ganglia calcification and at necropsy there was evidence of widespread generalised myelin loss with extensive Rosenthal fibre formation. The hypothesis that this may be an adult form of Alexander's disease is discussed.
منابع مشابه
Alexander's disease: case report including histopathological and electron microscopic features.
We report a case of Alexander's disease in a black South African boy of 9 years. The child presented with a grossly abnormal stooped posture, generalised weakness, and slurred nasal speech. Computerised tomography revealed diffuse low radio-density confined to the white matter, and enlarged ventricles. Cerebral biopsy confirmed the diagnosis. The histopathological and electron microscopic featu...
متن کاملMelkersson-Rosenthal syndrome: Appropriate response to the combination treatment with intralesional triamcinolone injection and oral azithromycin
Melkersson-Rosenthal syndrome is an uncommon disorder which is presented by a triad of lip swelling, facial nerve palsy, and a fissured tongue. A number of treatments have been reported for this syndrome with variable outcomes. We have reported a 38-year-old female patient with lower lip swelling and a fissured tongue on clinical examination. She was also suffering from facial nerve paralysis s...
متن کاملAn Adult Case of Hydroa Vacciniforme
Hydroa vacciniforme (HV) is a rare acquired photodermatosis, usually with onset in childhood and characterized by vesicles, crust and scar formation that follow exposure to sunlight.Vacciniform scars of face and dorsa of the hands are common features but oral ulcer and eye signs also rarely occur. It usually resolves before adult age. A rare manifestation of the disease would be persisten...
متن کاملA familial disorder associated with palatal myoclonus, other brainstem signs, tetraparesis, ataxia and Rosenthal fibre formation.
Three siblings presented with a progressive neurological disorder beginning in the third decade of life and characterised by palatal myoclonus, nystagmus, bulbar weakness and spastic tetraparesis. There was no evidence of intellectual deterioration or seizures. CT scan showed marked brainstem atrophy in two patients and basal ganglia calcification in one. MRI scan in one showed high signal in t...
متن کاملDevelopment of Eye Position Dependency of Slow Phase Velocity during Caloric Stimulation
The nystagmus in patients with vestibular disorders often has an eye position dependency, called Alexander's law, where the slow phase velocity is higher with gaze in the fast phase direction compared with gaze in the slow phase direction. Alexander's law has been hypothesized to arise either due to adaptive changes in the velocity-to-position neural integrator, or as a consequence of processin...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Journal of neurology, neurosurgery, and psychiatry
دوره 47 4 شماره
صفحات -
تاریخ انتشار 1984