Sirs: Rapidly evolving dementia, myoclonus and typical EEG abnormalities are the main cardinal features of sporadic Creutzfeldt-Jakob disease (CJD) [1]. We report a form of CJD with atypical clinical presentation, that first led to a diagnosis of corticobasal degeneration (CBD) [2]. We also describe an unusual MRI aspect. A 70-year-old woman complained of clumsiness and dysesthesias in both hands. She also described a temporary abnormal behavior of her left hand (“it was doing things I did not ask it to do”), which we interpreted as a wayward hand phenomenon [3]. At the time of first examination, she had: dystonia with flexion of hands and wrists, more marked on the right; slow athetoid-like movements of the right hand; mild cogwheel rigidity of upper and lower limbs on the right; astereognosia and melokinetic apraxia of both hands, and ideomotor apraxia of the left hand; slight paretic dysarthria and hypophonia. Gait was unsteady and writing was impossible. There was no buccofacial apraxia, tremor, myoclonus, ophthalmoplegia, or Babinski sign; tendinous reflexes were normal. Testing of cognitive functions, limited by motor impairment, fatigability and inertia, showed a slight disorder of executive functions and working memory, and a severe impairment of visuoperceptive and calculation abilities. EEG showed pseudoperiodic waves. CSF (including 14-3-3 protein) was normal. On MRI, bilateral atrophy of frontal and parietal lobes was seen, with relative hyperintensity of the cortical ribbon on FLAIR sequences; on inversion-recovery sequences, the signal of the cortical ribbon was unusually low, causing the ribbon to fade into the adjacent CSF. SPECT showed bilateral parietal hypoperfusion. Death occurred within 6 months. The patient was not demented and myoclonus was not seen. Neuropathological examination (Pr. Kopp, Lyon, France) confirmed the diagnosis of sporadic CJD, showing neuronal loss, gliosis and spongiosis, mainly in frontal and occipital cortices; striatum and cerebellum were much less affected; using monoclonal antibody 3F4 demonstrated the presence of pathological prion protein. The interest of these observations lies in: The atypical clinical presentation: there was no dementia or myoclonus, and the majority of signs were related to a dysfunction of parietal lobes (apraxia, astereognosia, visuoperceptive and calculation deficits) and basal ganglia (dystonia, athetosis, rigidity). A diagnosis of CBD was proposed first, but the evolution and EEG data soon allowed a clinical diagnosis of CJD. Only two identical cases, confirmed pathologically, have been described, with longer duration (respectively 18 months and 4 years) [4, 5]. In one of these cases [4], as in ours, protein 14-3-3 was negative; we do not have a definitive explanation for this negativity, but it could reflect the fact that neuronal destruction is limited in these focal cases. The presence of wayward hand phenomenon [3]. Alien-hand syndrome has been described several times in CJD since 1997 [4, 6–8] but it seems more appropriate to use the label of wayward hand (“main capricieuse”) [3, 9, 10]: the hand realises more or less proposiLETTER TO THE EDITORS