Lewy body dementia

Case contributed by Dr Chee Kok Yoon

Presentation

Altered behavior and dementia.

Patient Data

Age: 65 years
Gender: Female
MRI

MRI demonstrates generalized cerebral volume loss, more accentuated in the frontoparietal regions, with relative sparing of the temporal and occipital lobes. The hippocampi and mesial temporal lobes are not particularly volume depleted and have normal signal on FLAIR. 

No convincing atrophy of the striatum or midbrain is present. The hypothalamic region may be a little more volume deplete than the rest of the brain. No convincing iron deposition in the globus pallidi and no 'eye of the tiger sign'.

Case Discussion

Features would be compatible with (although by no means pathognomonic of) the clinical diagnosis of Lewy body dementia

 

The patient had no past psychiatric history but does however have a diagnosis of familial retinitis pigmentosa with strong family history. She was apparently well until 2 years ago when she presented with less than a year depressed mood, anxiety and child-like behavior. Also has a history suggestive of visual and auditory hallucination but this was never fully established. She also has fluctuations of her agitation which in years to come became almost persistent throughout the day.

Based on her case notes, no extrapyramidal signs were elicited but she did develop a resting pill rolling tremor of right hand after started Risperidone at very low dose. Tremor did not resolve despite cessation of Risperidone. Did not become worse with Quetiapine XR 200mg nocte. No other EPS noted. 

Agitation and confusion got worse when vision deteriorated severely last year. She is now legally blind and also has a sensorineural hearing deficit. 

She is unable to carry out  NuCOG because she has extremely poor vision and hearing. Possible ataxia because she is unable to walk steadily and kept on falling. 

Had MMSE done in Dec 2010 when she first presented with depression, at which time she scored 30/30. It has dropped to around 15/26 last month. 

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