EEG and cranial MRI findings in heidenhain variant of Creutzfeldt-Jakob disease [Creutzfeldt-Jakob hastali{dotless}ǧi{dotless}-heidenhain varyanti{dotless}: EEG ve kraniyal MRI bulgulari{dotless}]

Creutzfeldt jakob disease (cjd) is the most common prion disease. the heidenhain variant of cjd's clinical feature is the association with visual symptoms such as progressive visual decline,cortical blindness,visual agnosia and visual hallucinations. case: sixty-year old man presented to us with progressive dementia,gait disturbances,audio-visual hallucinations and behavioral changes. on his neurological examination,the patient was confused,his attention span was decreased and accompanied by disorientation to time and place. there was tetraparesis and deep tendon reflexes were absent. the babinski's sign was bilaterally negative. the myoclonic jerks are induced tactile or auditory stimulus. his cranial magnetic resonance imaging (cmri) and diffusion-weighted imaging (dwi) showed cerebral and cerebellar atrophy,t2 and flair-weighted sequences of basal ganglia,thalamus and occipital cortex showed increased signal intensity and diffusion limitation. his electroencephalography (eeg) findings showed low-amplitude slow wave activity in both hemispheres and periodic sharply contoured slow-wave in the occipital regions. the cerebrospinal fluid's (csf) protein was high level and the result of csf protein 14-3-3 was negative. the patient died in 6th month of clinical follow-up. conclusion: the heidenhain variant of cjd should be considered in all patients who present with dementia,ataxia and visual phenomena. close follow-up as well as serial eeg can help clarify the underlying disease process. and dwi and flair sequences should be included in the cmri protocol.