Case presentation

This 49 year old woman was referred to the Neurology Department in November 1994. Poor memory and speech difficulty had been present for about 18 months. She noticed an inability to remember the names of common household objects. In addition she complained of forgetting people’s names, and noted that her speech was more hesitant, with a stammer. She could not decide what to choose from restaurant menus, she had become increasingly muddled when presented with new information or new tasks, and at times became agitated, restless, and unsettled. She had become more emotionally labile, becoming tearful at the slightest upset. She had no other symptoms. Hypertension had recently been diagnosed, and she was taking an antihypertensive drug and hormone replacement therapy for perimenopausal symptoms. There was no family history of neurological disorder. She ran her own business as a photographer. She was a non-smoker and drank about two units of alcohol a day.

On examination, there were no cardiovascular abnormalities. Blood pressure was 180/100. On testing higher cortical function, her speech was somewhat rambling and hesitant with word finding difficulty. Serial sevens were poor and she could not remember the name of the prime minister. In addition she had poor knowledge of current affairs and could not interpret proverbs appropriately. Cranial nerve examination was normal, there were no primitive reflexes, and the rest of the neurological examination was normal.

Initial investigations were undertaken. The following blood tests were normal: full blood count, erythrocyte sedimentation rate, B₁₂, folate, urea and electrolytes, glucose, liver function, thyroid function, lipids, serum immunoglobulins, vanereal disease research laboratory test, and serum copper. Brain CT and MRI were normal, as was the chest radiography. A SPECT cerebral perfusion (fig 1) disclosed markedly reduced perfusion in the left temporal and parietal and right posterior parietal regions. …