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Alien hand syndrome is a rare condition associated with lesions of the corpus callosum and of the supplementary motor area, which can be caused by ruptured cerebral aneurysms. We present a novel case of a patient with a subarachnoid haemorrhage from an aneurysm of the anterior communicating artery who developed alien hand syndrome and intermanual conflict. In addition, we performed a systematic literature search and evaluated data on clinical presentation, treatment and radiological findings from relevant papers. To date, 17 cases of alien hand syndrome in aneurysmatic subarachnoid haemorrhage have been reported. Aneurysms of the anterior communicating artery were the most common origin of subarachnoid haemorrhage (10/17), followed by pericallosal artery aneurysms (7/10). The prognosis appears to be favourable; however, damage to the supplementary motor area during treatment should be avoided to keep symptoms minimal.

Alien hand syndrome (AHS) is a rare condition, which is defined as involuntary meaningful movements of a limb associated with loss of ownership over the limb. It affects mostly the left hand. Corticobasal degeneration, stroke and Creutzfeldt-Jakob disease are the three leading causes of AHS. AHS is classically divided into three subcategories: frontal, callosal and posterior. Loss of inhibitory tone and loss of bilateral hemisphere activation are the hypothesised pathophysiologic causes of AHS. This is a case report of a posterior AHS in a 47-year-old woman after stroke in the right parietal lobe.

Background Alien hand syndrome (AHS) is a disabling condition in which one hand behaves in a way that the person finds "alien". This feeling of alienation is related to the occurrence of movements of the respective hand performed without or against conscious intention. Most information on AHS stems from single case observations in patients with frontal, callosal, or parietal brain damage. Methods Retrospective analysis of distinctive clinical features of three out of 18 epilepsy patients who developed AHS with antagonistic movements of the left hand after corpus callosotomy (CC) (one anterior, two complete) for the control of epileptic seizures, particularly epileptic drop attacks (EDA). Results Remarkably, these three patients, two men and one woman, displayed atypical language dominance with a bilateral, left more than right hemisphere language representation in intracarotidal amobarbital testing before surgery. The overall additional distinctive feature of the target patients was genuine left-handedness, with writing retrained to right-handedness in two patients. After surgery the left hands became alien. The problem was permanent, despite strategies for compensation. Conclusion From this observation we suggest that under the conditions of dissociation of language and motor dominance, loss of both intentional control of contralateral action and physiological inhibition of antagonistic movements lead to post-callosotomy alien-hand-like motor phenomena. The dissociation pattern posing this risk seems rare but needs to be considered when evaluating candidates for callosotomy.

An alien limb is a debilitating disorder of volitional control. The core feature of alien limb is the performance of simple or complex semi-purposeful movements which the patient reports to be unintentional or unwanted, or occasionally in opposition to their intentions. Theories of the mechanism of alien limb phenomena have emphasised the role of disinhibition in the brain, and exaggerated action 'affordances'. However, despite advances in cognitive neuroscience research and a large public and media interest, there has been no unifying computational and anatomical account of the cause of alien limb movements. Here, we extend Bayesian brain principles to propose that alien limb is a disorder of 'predictive processing' in hierarchical sensorimotor brain networks. Specifically, we suggest that alien limb results from predictions about action outcomes that are afforded unduly high precision. The principal mechanism for this abnormally high precision is an impairment in the relay of input from medial regions, predominantly the supplementary motor area (SMA), which modulate the precision of lateral brain regions encoding the predicted action outcomes. We discuss potential implications of this model for future research and treatment of alien limb.

A previously independent 66-year-old right-handed male presented with right-sided weakness, preferring the lower-extremities with additional impaired gait and dysarthria for 1-day duration. Imaging found a large left hemispheric anterior cerebral artery ischemic infarction with multiple lacunar infarcts. He exhibited frontal, callosal, and posterior variants of Alien Hand Syndrome which impeded activities of daily living. Although limited in evidence, a trial of clonazepam was initiated based on previous case reports describing suspected efficacy. Botulinum toxin A was not used given the patient's immediate need and limited hospital length of stay. Right upper extremity constricting therapies improved intermanual conflict and spontaneous grasping and levitation (arm elevation in retroflexion) activity; however, concomitant left upper extremity motor apraxia complicated task-oriented activities. The combination of pharmaceutical and therapeutic interventions improved the patient's quality of life as assessed by clinical observation, functional independence measures from 41 to 57, and patient-reporting. This case report aims to increase awareness of a potential barrier to rehabilitation of a debilitating and rare condition and to discuss current assessment tools and treatment options supported by available evidence.

A 69-year-old woman presented with a cortical hand syndrome progressing over several weeks. MRI brain showed characteristic appearances of progressive multifocal leukoencephalopathy (PML), confirmed by detection of the JC virus in CSF, despite the absence of any evidence of immunosuppression. Treatment with mirtazapine, mefloquine and cidofovir did not affect the progression of the disease, which was fatal within 7 months of presentation. This report adds to the small case literature that suggests that PML can occur in immunocompetent people, albeit extremely rarely.

Alien limb refers to movements that seem purposeful but are independent of patients’ reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of alien limb symptoms in patients with the corticobasal syndrome (n = 30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease control group. Structured examinations of praxis, motor function, cognition and alien limb were undertaken in patients attending a regional specialist clinic. Twenty-eight patients with corticobasal syndrome (93%) demonstrated significant apraxia and this was often asymmetrical, with the left hand preferentially affected in 23/30 (77%) patients. Moreover, 25/30 (83%) patients reported one or more symptoms consistent with alien limb. The range of these phenomena was broad, including changes in the sense of ownership and control as well as unwanted movements. Regression analyses showed no significant association between the severity of limb apraxia and either the occurrence of an alien limb or the number of alien limb phenomena reported. Bayesian estimation showed a low probability for a positive association between alien limb and apraxia, suggesting that alien limb phenomena are not likely to be related to severity apraxia. Our results shed light on the phenomenology of these disabling and as yet untreatable clinical features, with relevance to theoretical models of voluntary action.

OBJECTIVE Perirolandic atrophy occurs in corticobasal syndrome (CBS) but is not specific versus progressive supranuclear palsy (PSP). There is heterogeneity in the locations of atrophy outside the perirolandic cortex and it remains unknown why atrophy in different locations would cause the same CBS-specific symptoms. In prior work, we used a wiring diagram of the brain called the human connectome to localize lesion-induced disorders to symptom-specific brain networks. Here, we use a similar technique termed "atrophy network mapping" to localize single-subject atrophy maps to symptom-specific brain networks. METHODS Single-subject atrophy maps were generated by comparing cortical thickness in patients with CBS versus controls. Next, we performed seed-based functional connectivity using a large normative connectome to determine brain regions functionally connected to each patient's atrophied locations. RESULTS Patients with CBS had perirolandic atrophy versus controls at the group level, but locations of atrophy in CBS were heterogeneous outside of the perirolandic cortex at the single-subject level (mean spatial correlation = 0.04). In contrast, atrophy occurred in locations functionally connected to the perirolandic cortex in all patients with CBS (spatial correlation = 0.66). Compared with PSP, patients with CBS had atrophy connected to a network of higher-order sensorimotor regions beyond perirolandic cortex, matching a CBS atrophy network from a recent meta-analysis. Finally, atrophy network mapping identified a symptom-specific network for alien limb, matching a lesion-induced alien limb network and a network associated with agency in healthy subjects. INTERPRETATION We identified a syndrome-specific network for CBS and symptom-specific network for alien limb using single-subject atrophy maps and the human connectome. ANN NEUROL 2020;88:1118-1131.

Rationale: Alien Hand syndrome (AHS) is characterized in most patients by seemingly purposeful, involuntary movements of the extremities. It is not well known among physicians on account of its diverse clinical manifestations. Patient concerns: We present a 57-year-old Chinese man who could not stop or turn himself around as he involuntarily and uncontrollably walked forward, which had happened frequently in the month prior to treatment. He had been a heavy drinker for thirty years before the onset of the disease, with an alcohol intake of 600 to 800 ml/day. Diagnoses: History of alcohol intake and the brain magnetic resonance imaging findings indicated a diagnosis of Marchiafava–Bignami disease. The patient was additionally diagnosed with Alien Hand Syndrome according to his clinical symptoms. Interventions: The patient was treated with high doses of vitamin B for 1 month. Outcomes: The patient's abnormal behaviors never appeared during the treatment, and no instance of recurrence was observed during the 6 months of follow-up. Lessons: The clinical manifestation of AHS is non-specific. Only by considering its diverse manifestation can doctors better understand the disease and achieve early intervention.

Alien limb phenomenon is a rare syndrome associated with a feeling of non-belonging and disowning toward one's limb. In contrast, anarchic limb phenomenon leads to involuntary but goal-directed movements. Alien/anarchic limb phenomena are frequent in corticobasal syndrome (CBS), an atypical parkinsonian syndrome characterized by rigidity, akinesia, dystonia, cortical sensory deficit, and apraxia. The structure–function relationship of alien/anarchic limb was investigated in multi-centric structural magnetic resonance imaging (MRI) data. Whole-group and single-subject comparisons were made in 25 CBS and eight CBS-alien/anarchic limb patients versus controls. Support vector machine was used to see if CBS with and without alien/anarchic limb could be distinguished by structural MRI patterns. Whole-group comparison of CBS versus controls revealed asymmetric frontotemporal atrophy. CBS with alien/anarchic limb syndrome versus controls showed frontoparietal atrophy including the supplementary motor area contralateral to the side of the affected limb. Exploratory analysis identified frontotemporal regions encompassing the pre-/and postcentral gyrus as compromised in CBS with alien limb syndrome. Classification of CBS patients yielded accuracies of 79%. CBS-alien/anarchic limb syndrome was differentiated from CBS patients with an accuracy of 81%. Predictive differences were found in the cingulate gyrus spreading to frontomedian cortex, postcentral gyrus, and temporoparietoocipital regions. We present the first MRI-based group analysis on CBS-alien/anarchic limb. Results pave the way for individual clinical syndrome prediction and allow understanding the underlying neurocognitive architecture.

Alien limb syndrome following stroke within the territory of the posterior cerebral artery is exceedingly rare. A right-handed female experienced left homonymous hemianopia, visuospatial neglect, and proprioceptive loss of her left hemi-body. She experienced unintended, involuntary movements of her left arm and hand, which interfered with and disturbed motor actions of daily life performed with her right upper limb. There was no denial of ownership, but she interpreted movements of her left upper limb to be annoying, out of her will and unwanted. The alien limb phenomenon improved in parallel with improvement of proprioceptive loss over a 12-week of in-patient rehabilitation. A recently proposed theoretical concept of the alien limb phenomenon after posterior artery stroke is discussed.

An alien limb is a debilitating disorder of volitional control. The core feature of alien limb is the performance of simple or complex semi-purposeful movements which the patient reports to be unintentional or unwanted, or occasionally in opposition to their intentions. Theories of the mechanism of alien limb phenomena have emphasised the role of disinhibition in the brain, and exaggerated action 'affordances'. However, despite advances in cognitive neuroscience research and a large public and media interest, there has been no unifying computational and anatomical account of the cause of alien limb movements. Here, we extend Bayesian brain principles to propose that alien limb is a disorder of 'predictive processing' in hierarchical sensorimotor brain networks. Specifically, we suggest that alien limb results from predictions about action outcomes that are afforded unduly high precision. The principal mechanism for this abnormally high precision is an impairment in the relay of input from medial regions, predominantly the supplementary motor area (SMA), which modulate the precision of lateral brain regions encoding the predicted action outcomes. We discuss potential implications of this model for future research and treatment of alien limb.

Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease with neuropsychological sequelae. This study highlighted a rare presentation of CJD (e.g. corticobasal syndrome [CBS]), reviewed updated diagnostic criteria and procedures for CJD (e.g. diffusion weighted imaging [DWI], real-time quaking-induced conversion [RT-QuIC]), and discussed differential diagnoses.Case report methodology focused on a 68-year-old, Hispanic, right-handed man with 11 years of education. He presented with a 1-2-month history of gait and motor difficulties (e.g. rigidity, myoclonus).After evaluation, a 'cortical ribboning' pattern on DWI and positive RT-QuIC was integrated with performance on neurobehavioral exam (i.e. alien limb phenomenon, unilateral ideomotor apraxia) and neuropsychological testing (i.e. frontal-parietal dysfunction pattern) to reach a diagnosis of sCJD-CBS. The patient expired 3 months after onset of symptoms.This literature review and case report highlighted the importance of staying abreast of developments in neurological literature and the added value of neuropsychology, when integrated with newer procedures, for confirming and excluding diagnostic considerations.

Background: Arm levitation is an involuntary elevation of the upper limb, a manifestation of the alien-limb phenomenon. It has rarely been reported in Creutzfeldt–Jakob disease (CJD), less so as an initial manifestation Case Report: We report a 56-year-old right-handed man with rapidly progressive gait ataxia and involuntary elevation of the left upper limb. During the next few weeks, the patient developed cognitive impairment, apraxia, visual hallucinations, and myoclonus. He met diagnostic criteria for CJD. We evaluated additional published cases of early-appearance of alien-limb phenomenon in the context of CJD; there were 22 such cases and alien-limb phenomenon was the first and exclusive manifestation in only five of them. Discussion: Arm levitation may be a distinct presentation of CJD, appearing earlier than other clinical features., Tremor and Other Hyperkinetic Movements, Tremor and Other Hyperkinetic Movements

A 94-year-old patient developed the alien hand syndrome in the left (nondominant) hand following an ischemic stroke of the right parietotemporal lobe (including postcentral gyrus, superior and inferior parietal lobule) due to an occlusion of the posterior division of the right middle cerebral artery.

We report the case of a 73-year-old right-handed female with a right parietal cerebral infarction and presented symptoms that were challenging to differentiate between alien hand sign (AHS) and sensory ataxia. She presented to our emergency department with chief complaints of abnormal involuntary movements and a feeling of foreignness on her left upper limb. The first neurological examination revealed left spatial neglect, left-side sensory impairment that included superficial and deep sensations, left limb-kinetic apraxia, and left limb ataxia. Furthermore, her symptoms and complaints had characteristics of AHS that includes a sensation that her left upper limb dose not belong to herself and an abnormal behavior of left hand that is contrary to her own intent. Brain MRI revealed an acute cerebral infarction confined to the right postcentral gyrus. This case highlights that sensory ataxia due to the disturbance of deep sensation might present symptoms similar to AHS. Previous studies suggested the involvement of the disturbance of somatosensory pathway in posterior-variant AHS. Therefore, a precise distinction between AHS and sensory ataxia, especially in posterior-variant AHS, is imperative to avoid confusion regarding the term "alien hand sign."

Background Alien Hand Syndrome (AHS) is an uncontrollable, involuntary, but in appearance, purposeful motor control disorder of the upper extremity. Case report A 42-year-old male patient was admitted to our clinic complaining of involuntary motor activity in his right hand. He had a previous history of migraine with visual aura. The uncontrollable motor control disorder was compatible with Alien Hand Syndrome, which was appearing immediately after the visual aura and before the beginning of headache. Conclusion Alien Hand Syndrome is usually observed with anterior cerebral artery infarction, midline tumors, trauma and several neurodegenerative diseases, but is rarely seen in paroxysmal conditions such as migraine with aura.

We report a 65-year-old man with a right cerebral infarction that occurred 15 years ago and a residual left hemiparesis that began with progressive contralateral hemiparesis. During the hospitalization, the patient developed a bilateral alien hand syndrome. Urgent CT, MR, CT angiography, and brain perfusion SPECT were performed that revealed an old right cerebral infarction and a new ischemic lesion in left parietal lobe and adjacent brain territories.

Any injury of a hand more or less disintegrates the injured hand. The rehabilitation of an alienated hand is prolonged. How can the alienation of the own but injured hand and the involuntary and unconscious inhibition of its perception and use be diagnosed, explained, avoided and designated? Medical observations and interviews with patients and therapists on the occasion of rehabilitation of hand injuries resulted in the development of a new concept of biopsychosocial integrating rehabilitation and a creative hand therapy with the objective of integrating injured and alienated hands.The inhibition of the gesture of thinking has been the most revealing sign for the diagnosis of a disintegration of an injured hand. Explanation: The involuntary inhibition to recognize and use an injured hand causes and implicates the alienation of the rested hand.Information by hand surgeons and hand therapists. Creative hand therapy guides the attention to complex and pleasing activities. In complex disintegrations after hand injuries the hand as well as the person need help. Designation: Hypotheses: Posttraumatic Proportionate Regional Disintegration while the tissues heal. Posttraumatic Complex Regional Disintegration, if the disintegration overruns the healing of tissues or if the person suffers. The Complex Regional Pain Syndrome is distinguished as a rare exception of a biopsychosocial disintegration. Posttraumatic regional disintegration seems to be proportionate while the tissues are healing. If the mentioned sensations of the patients and the visible signs of disintegration persist, the disorder spreads onto the biopsychosocial unit of the person. This disorder of hand and person may be designated as a complex disorder. The impact of a proportionate posttraumatic disintegration as well as the formation and exacerbation of a complex posttraumatic disintegration may be prevented by the facilitation of the integration of an injured hand. Scientific prove is needed for: the guiding impact of the healthy hand for physiotherapy; the impact of the gesture of thinking to diagnosis and treatment of disintegrations; the effectivity and the economic efficiency of a biowpsychosocial integrating rehabilitation and creative hand therapy.

A 47-year-old man developed sudden-onset abulia and alien hand syndrome (AHS). MRI revealed bilateral ischemic injury within anterior cerebral artery territory (a complex bilateral corpus callosum infarction1) due to obstruction of a variant bihemispheric A2 branch by a thrombus (figures 1 and 2), also demonstrated on 3D volume-rendered CT angiography (figure 2). The finding of lower-limb deep venous thrombosis in a patient with right-to-left interatrial shunt accounted for the case, given workup was otherwise unremarkable.

The Video NeuroImage by Debray and Demeestere1 is a good demonstration of the callosal alien hand variant, typified by intermanual conflict. This is in contrast to the frontal alien hand variant, where there is compulsive grasping and grabbing, but without fighting over control of an object.

Corticobasal degeneration (CBD) may be expressed as an atypical parkinsonism with a mean disease survival of about 7 years,(1) the shortest reported survival being 24 months.(2) We report a patient with corticobasal syndrome (CBS) and pathology-confirmed CBD whose 10-month course was manifested as a frontal alien hand evolving into agrypnia excitata.

The alien hand syndrome (AHS) is a rare neuropsychological disorder characterized by involuntary, yet purposeful, hand movements. Patients with the AHS typically complain about a loss of agency associated with a feeling of estrangement for actions performed by the affected limb. The present study explores the integrity of the body representation in AHS, focusing on 2 main processes: multisensory integration and visual self-recognition of body parts. Three patients affected by AHS following a right-hemisphere stroke, with clinical symptoms akin to the posterior variant of AHS, were tested and their performance was compared with that of 18 age-matched healthy controls.AHS patients and controls underwent 2 experimental tasks: a same-different visual matching task for body postures, which assessed the ability of using your own body schema for encoding others' body postural changes (Experiment 1), and an explicit self-hand recognition task, which assessed the ability to visually recognize your own hands (Experiment 2).As compared to controls, all AHS patients were unable to access a reliable multisensory representation of their alien hand and use it for decoding others' postural changes; however, they could rely on an efficient multisensory representation of their intact (ipsilesional) hand. Two AHS patients also presented with a specific impairment in the visual self-recognition of their alien hand, but normal recognition of their intact hand.This evidence suggests that the AHS following a right-hemisphere stroke may involve a disruption of the multisensory representation of the alien limb; instead, self-hand recognition mechanisms may be spared. (PsycINFO Database Record

Alien hand syndrome (AHS) is a rare disorder of involuntary limb movement together with a sense of loss of limb ownership. It most commonly affects the hand, but can occur in the leg. The anterior (frontal, callosal) and posterior variants are recognized, with distinguishing clinical features and anatomical lesions. Initial descriptions were attributed to stroke and neurosurgical operations, but neurodegenerative causes are now recognized as most common. Structural and functional imaging and clinical studies have implicated the supplementary motor area, pre-supplementary motor area, and their network connections in the frontal variant of AHS, and the inferior parietal lobule and connections in the posterior variant. Several theories are proposed to explain the pathophysiology. Herein, we review the literature to update advances in the understanding of the classification, pathophysiology, etiology, and treatment of AHS.

A 58-year-old woman with a history of poorly controlled type 2 diabetes, hypertension and smoking presented with a left hemiparesis (grade 4/5). Admission glucose was 22.6 mmol/L. MRI of the brain demonstrated an area of restricted diffusion involving the corpus callosum of the left hemisphere (figures 1 and 2). Investigations into the cause of her stroke including vasculitic and thrombophilic causes, echocardiogram, telemetry and carotid Doppler, did not demonstrate any significant pathology. Rather alarmingly, the patient's glycated hemoglobin was elevated at 134 mmol/mol. Figure 1 Axial …

Background: “Anarchic hand” is a rare condition characterised by non-volitional, goal-directed movements of one arm. We report a case with analysis of structural and functional connectivity. Case presentation: A 15 year old girl developed intermittent symptoms of intermanual conflict or anarchic hand as a result of traumatic brain injury during which she sustained a callosal bleed. Resting-state fMRI and DTI tractography were performed at a stage when symptoms had largely resolved. Conclusion: Structural connectivity between homologous superior frontal areas and functional connectivity between homologous posterior cingulate areas were significantly reduced, which may have contributed to causation. Tractography demonstrated new indirect connections between supplementary motor areas via the cerebellum, which we propose contributed to symptom resolution.

A 76-year-old right-handed non-diabetic woman presented with involuntary upper left limb movements for 2 h. The patient described the limb as having a will of its own. The movements occurred intermittently for 7 days, with each episode lasting 10–20 min. Segment 1 (video 1) showed levitation of the left hand with right hand restraint. Segment 2 displayed the left hand thumping the chest, with neck abrasions. Segment 3, 1 week later demonstrated neck strangulation. Segment 4, 2 weeks later exhibited normality. Physical examination revealed apraxia and dystonia but no parkinsonism. The Montreal cognitive assessment score was 25/30 due to executive impairment. The patient was dyslipidaemic with hypertensive heart disease. However, the …

Supplemental Digital Content is available in the text, Levitation and tentacular movements (LTM) are considered specific, yet rare (30%), features of Corticobasal Syndrome (CBS), and are erroneously classified as alien hand. Our study focuses on these typical involuntary movements and aims to highlight possible neural correlates. LTM were recognizable during functional magnetic resonance imaging (fMRI) in 4 of 19 CBS patients. FMRI activity was evaluated with an activation recognition program for movements, during LTM, consisting of levitaton and finger writhing, and compared with the absence of movement (rest) and voluntary movements (VM), similar to LTM, of affected and unaffected arm-hand. FMRI acquisition blocks were balanced in order to match LTM blocks with rest and VM conditions. In 1 of the 4 patients, fMRI was acquired only during LTM and with a different equipment. Despite variable intensity and range of involuntary movements, evidenced by videos, fMRI showed, during LTM, a significant (P

Anarchic hand syndrome (AHS) is a rare neurological condition characterized by seemingly purposeful, goal-directed hand movements which the person afflicted by the syndrome is not, however, in control of. By extensively examining a patient with AHS we provide novel neuropsychological and lesion mapping data that shed new light on the possibility of modulating specific symptoms associated with AHS, in particular unilateral apraxia and magnetic apraxia. Moreover, we compared lesion mapping data with an in depth analysis of previous studies in order to explore the neural network responsible for the complex symptomatology associated with this syndrome. We found that non-primarily motor variables (e.g. the nature of the object to be grasped and integration of visuo-spatial feedback in action) play an important role in determining AHS symptomatology. Moreover, we found that lesions involving various different parts of the motor control network (the corpus callosum, the anterior cingulate cortex and the supplementary motor area, the parietal areas and thalamus) are closely linked to partially differing AHS symptoms. The comparison of our data with those reported in previous studies indicate that AHS is a multifaceted and complex syndrome in which the influence of non-primarily motor, emotional and higher-order components may be largely underestimated.

We present a 41-year-old man who experienced alien limb syndrome as a complication of treatment for recurrent Grade III oligodendroglioma of the right parietal lobe. Alien limb syndrome is a rare phenomenon in which a limb performs involuntary actions and the affected individual feels a sense of estrangement towards the limb. It occurs most commonly as a result of corticobasal syndrome, though a variety of other etiologies have been reported. It is rarely associated with focal lesions, such as stroke or tumors.

A 64 yr old right-handed mechanic presented with four weeks history of clumsy right hand with ongoing problems at work. He described stiffness and problems with co-ordination. At the time of presentation he was noted to be walking with his right arm behind him and not being aware of this. His initial examination showed Mini Mental Score of 26/30, with ongoing involuntary movements of his right arm (Video).Video shows spontaneous elevation of right arm with occasional myoclonic jerks. Video also demonstrates patient's difficulty in following simple motor tasks and his comments that his arm “has a mind of its own”.His MRI head (Figure 1) showed typical cortical ribboning pattern described in sporadic CJD and CSF Protein for 14-3-3, s100b and RT-QUIC findings were consistent with the diagnosis of CJD.Over next 4 weeks his cognitive function rapidly deteriorated with progressive worsening of his myoclonus. He died 4 weeks after his hospital discharge.There are three broad varieties of alien limb phenomenon (ALP) types described in the literature, frontal, callosal and sensory. Jerky (myoclonic) ALP is well described in patients with CJD, so CJD should be in the differentials in any patient presenting with sub-acute onset of ALP.