Rapidly progressive dementia and ataxia in an elderly man
A 76-year-old man, a university educated retired engineer, was referred with a 2-month history of deteriorating balance and memory. He took metformin for type 2 diabetes mellitus. He attended with his wife within 2 days of the referral and brought his recent MRI scans of the brain and abdomen. He was not concerned by any memory problems (his wife certainly was), but felt off-balance when walking. It became apparent that he had actually been losing balance and memory for about 12 months, but this had progressed more rapidly in the last 2 months. He had no headaches, sensory or urinary symptoms. He was sleeping and eating well and had not lost weight. He was an accomplished pianist and his musical abilities had not deteriorated. He had never smoked and drank little alcohol.\nHis wife said that he was his usual jocular self—possibly even fatuous. General examination, including heart, chest and abdomen, was normal. There was no cranial nerve abnormality, no papilloedema, no muscle wasting, normal muscle tone and strength throughout; reflexes were normal in the upper limbs, but knee jerks and ankle jerks were absent. His right plantar was extensor. Sensation was intact to pinprick; there was slight impairment of vibration and position sense at the toes. He walked with a wide-based unsteady gait and could not tandem walk; Romberg's test was positive. He scored 22/30 on the Mini-Mental State Examination, losing points for orientation, memory (recall) and repetition. On the Addenbrooke's Cognitive Examination he scored 62/100, losing points on memory (recall, anterograde and retrograde). Verbal fluency was decreased. Language, naming and comprehension were intact. His scores were: attention and orientation 14/18, memory 10/26, fluency 0/14, language 24/26, visuospatial 14/16.\nThe MRI scan of brain () he brought with him had been reported as follows: ‘There is moderate cerebral atrophy with prominence of the cortical sulci and of the ventricular system with dilatation of the bodies and temporal horns of the lateral ventricles and of the third ventricle. The fourth ventricle is prominent. There is increased signal intensity in the peri-ventricular white matter suggesting periventricular cerebrospinal fluid (CSF) resorption. There is a CSF collection in the cisterna magna deforming the inferior aspects of the cerebellar hemispheres, suggesting an arachnoid cyst. The images show no small vessel ischaemia. The distal left vertebral artery is occluded. Comment: despite the cerebral atrophy, the appearances suggest normal pressure hydrocephalus.’\nThe abdominal MRI report described ‘four cysts in the pancreas, without enhancement, mass effect, surrounding infiltration or duct dilatation’.