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Washington University Experience | NEURODEGENERATION | Corticobasal Degeneration (CBD) | 1A0 Case 1 History
Case 1 History ---- This patient was 72yo when he died in a severely demented state in 1995. He described a period of depression lasting two or three months in a difficult work situation beginning in May 1983. As the depression improved he noticed the gradual onset of difficulty with language, both in word finding and trying to articulate properly which worsened over the next two years. He had not noticed any difficulty with memory or thinking. He was noted to have an expressive aphasia with loss of prosody and grammatical structure. There was word-finding difficulty but normal comprehension to oral and written demands. He was also dysarthric. He used abnormal combinations of words and sounds for certain phrases, including some paraphasic substitutions. His recent memory was intact. He did simple calculations and abstract reasoning without a problem. His neurologic examination was entirely normal otherwise. The disorder was thought to represent progressive aphasia. Apart from aphasia, his cognitive function was normal and he was rated as CDR 0 in 1985. By 9/87, he was having more difficulty writing and his oral speech was worse. A head CT scan in 1985 was read as normal. At T4 (11/88), he was unable to utter an intelligible sound and also had difficulty with auditory reception; however, he interpreted language better if it was written. He could still write but had increasing grammatical and spelling errors. He was admitted to a nursing home in May 1993 and was examined there for T9 in 10/93. In the previous year, he was shuffling and had more difficulty using his right foot than the left. There was mild difficulty swallowing, which became gradually worse. He had isolated single jerks of his jaw thought to be myoclonic for about a year. He gradually lost facial expression except for a frequent stereotyped pattern mixing fright and sadness for a few seconds. He was described as having severe cognitive impairment in all spheres. There was rigidity in all limbs, worse on the right, sometimes with a cogwheel character. He had a brief resting tremor in his right hand and forearm. He had normal eye movements. A jaw jerk reflex was not present but a snout reflex was, Tendon reflexes and toe signs were normal. He was rated severely demented, CDR 3. The terminal event appeared to be a severe urinary tract infection with fever. He was started on antibiotics in January 1995, but died soon thereafter.
