Video 1
Arm Levitation as Initial Neurological Manifestation of Creutzfeldt–Jakob Disease. The video shows gait ataxia with an involuntary elevation of the left arm (arm levitation). The patient had only mild cognitive impairment in this evaluation.
Figure 1
Brain Magnetic Resonance Images (MRI) and Electroencephalographic Recording (A) Axial diffusion-weighted brain MRI (left) shows hyperintense signal in the cortex, predominantly parietal, and bilateral striatum, which indicates restriction diffusion with low signal on the apparent diffusion coefficient (ADC) map (middle). Axial fluid-attenuated inversion recovery brain MRI (right) reveals gradual anteroposterior hyperintensity gradient on striatum, sparing posterior putamen. (B) Electroencephalographic recordings show disorganization of cerebral activity due to diffuse delta activity, evolving to lateralized and diffuse periodic sharp-wave complexes (PSWC).
Table 1
Characteristics of Patients with Alien-limb Phenomenon as among the Initial Manifestations in Creutzfeldt–Jakob Disease
| Case, Reference | Age, Handedness, Sex | ALP Side, Type† | Associated Deficits at Onset | Latency from ALP to diagnosis | MRI |
|---|---|---|---|---|---|
| 1, Rubin et al.2 | 73, R, man | L, NR | Disorientation, visual hallucinations | NR | Cortical T2 hyperintensity (DWI not done) |
| 2, Rubin et al.2 | 56, R, man | L, NR | Memory impairment, aphasia, tremor, sleep disturbance | NR | Normal |
| 3, Rubin et al.2 | 58, R, woman | R, NR | None | NR | Restricted diffusion in B striatum, and left cortex |
| 4, Rubin et al.2 | 69, R, man | L, NR | None | NR | Normal |
| 5, Rubin et al.2 | 71, R, woman | L, NR | Cognitive impairment, blurred vision | NR | No MRI |
| 6, Rubin et al.2 | 69, R, man | L, NR | Facial paresthesia, blurred vision | NR | No MRI |
| 7, Rubin et al.2 | 73, unknown, man | R, NR | Unknown | NR | Normal |
| 8, Rubin et al.2 | 56, unknown, woman | R, NR | Myoclonus, ataxia | NR | Restricted diffusion in B striatum and cortex |
| 9, Rubin et al.2 | 61, R, man | R, NR | Right arm jerking | NR | L > R parietal restricted diffusion |
| 10, Rubin et al.2 | 39, R, woman | R, NR | None | NR | Restricted diffusion in B striatum, L parietal cortex |
| 11, Rubin et al.2 | 56, R, man | L, NR | None | NR | B restricted diffusion, T2 hyperintensity of R posterior hemispheric cortex |
| 12, Rubin et al.2 | 71, L, woman | R, posterior and callosal | Gait instability | NR | Restricted diffusion, T2 hyperintensity of bilateral R > L parietal, occipital lobes |
| 13, Rubin et al.2 | 72, R, woman | L, NR | Myoclonus, behavioral changes | NR | Diffuse cortical restricted diffusion |
| 14, MacGowan et al.4 | 78, R, woman | L, callosal | L hemiparesis, sensory ataxia, and myoclonus | NR | Normal |
| 15, MacGowan et al.4 | 74, R, woman | L, callosal | Progressive weakness | 6 weeks | Mild cerebral atrophy |
| 16, Fogel et al.9 | 55, R, woman | L, callosal | Gait instability, falls, and limb ataxia | 1 month | Bilateral L > R restricted diffusion, T2 hyperintensity of B parietal cortex |
| 17, Inzelberg et al.12 | 70, R, man | L, posterior | Unsteady gait, frequent falls, and visual hallucinations | At least 6 weeks | Mild cerebral atrophy |
| 18, Oberndorfer et al.14, * | 74, R, woman | L, posterior | L hand ataxia | 3 months | Non-diagnostic clues |
| 19, Anschel et al.15 | 65, R, man | L, callosal | None | 8 months | Restricted diffusion in R putamen, and bilateral R > L cortex |
| 20, Moreaud et al.16 | 70, unknown, woman | L, callosal | Upper limbs clumsiness | 6 months | B frontal and parietal atrophy, and cortical ribbon sign |
| 21, Kleiner-Fisman et al.17 | 73, R, man | L, posterior | L hand ataxia and clumsiness, L hemineglect, and unsteady gait | 4 weeks | Restricted diffusion in R caudate nucleus, and bilateral R > L cortex |
| 22, Avanzino et al.18 | 59, R, woman | L, posterior | L hand numbness | 2 weeks | Normal |
| 23, our case | 56, R, man | L, posterior | Gait instability and ataxia | 2 months | Right parietal cortex ribbon sign, Restricted diffusion in B striatum and R parietal cortex |