Abstract
Case Description Ms PM is a 79yo lady with a 6-weeks history of progressive ataxia. Her mobility deteriorated rapidly from walking independently to requiring assistance. Her past medical history is significant for breast cancer, hypertension, and hypercholesterolemia. On initial examination, Ms PM has significant truncal and limb ataxia with shuffling gait. She also has cogwheel rigidity in her upper limbs and increased tone in her lower limbs. Her power and sensation were normal. Basic biochemistry and CSF analysis were unremarkable.MRI Brain had shown brain atrophy and chronic microvascular ischaemic changes. MRI spine had shown no cord compression. EEG was normal. Ms PM was trialed on levodopa, IVIG and methylprednisolone with no improvement in her mobility. Alarmingly, Ms PM developed rapid cognitive decline and cerebellar signs in her upper limbs over 3-week period while stayed as an inpatient.
Discussion Ms PM’s initial presentation was suggestive of Parkinson-plus syndrome. It accounts for 10% of clinical parkinsonism. It normally has a faster progression and lack of response to levodopa treatment compared to Parkinson disease. However, it is normally not associated with rapid cognitive decline. Therefore, prior disease was considered. Creutzfeldt-Jakob disease (CJD) is the most frequent of prior diseases with an occurrence rate of one in a million per year. To diagnose probable sCJD, one needs to have progressive dementia and at least two of the four clinical features: myoclonus, visual or cerebellar disturbance, pyramidal or extrapyramidal dysfunction and akinetic mutism. Ms PM’s presentation fits well with the clinical diagnosis of sCJD.
