Statistics from Altmetric.com
The clinical picture of Creutzfeldt-Jakob disease (CJD) includes various movement disorders such as myoclonus, parkinsonism, hemiballism, and dystonia. We report on a patient with CJD who manifested the alien hand sign. We suggest that CJD should be included in the differential diagnosis of diseases which present with an alien hand.
Creutztfeldt-Jakob disease, one of the human prion diseases, is characterised by rapidly progressive mental and motor deterioration.1 Involuntary movements occur in above 90% of the patients in the course of the disease, the most common being myoclonus.1 Other movement disorders range from tremor to chorea, athetosis, dystonia, and hemiballism.1 We report on a patient with CJD who presented with an alien hand.
Alien hand is a rare and striking phenomenon defined as “a patient's failure to recognise the action of one of his hands as his own”.2 One of the patient's hands acts as a stranger to the body and is uncooperative. Thus, there is loss of feeling of ownership but not loss of sensation in the affected hand. Originally described in callosal tumours,3 the aetiology of alien hand also includes surgical callosotomy,4 infarction of the medial frontal cortex, occipitotemporal lobe, and thalamus,1 5 infection,6 and corticobasal degeneration.5 7
A 70 year old, right handed Jewish man born in Argentina, living in Israel for the past 20 years, was admitted to the Neurology Department. Until a month before his admission, he was apparently healthy and helped in the accounting office of the village where he lived. His neurological illness had presented insidiously during the past month with unsteadiness of gait and frequent falls. He also manifested behavioural changes, became aggressive, and had visual hallucinations, perceiving insects and mice moving through his visual field. Often, he expressed his fear from seeing that the “ceiling was falling over him”. His wife mentioned bizarre, useless movements of his left hand which were present from the beginning of the disease.
On admission, he was awake, bradyphrenic, and partially collaborative. His conversation was often disrupted by hallucinations. The affect was sad and he had partial insight for his mental dysfunction. He was disoriented for time, place, and situation. He could understand speech and was able to follow oral instructions involving two consecutive components. Naming was preserved. Prominent dysgraphia and dyscalculia were noticed. Immediate recall and short term memory were severely disturbed, whereas long term memory, especially for personal life events, was relatively spared. Abstract thinking was severely affected. Bimanual movements, such as clapping, were extremely difficult.
The cranial nerves were normal as were ocular fundi. The motor examination showed normal force. Deep reflexes were symmetric and plantar responses were flexor. The right arm had a dystonic posture. His gait was ataxic on a wide base.
At times, the left arm would spontaneously rise in front of the patient during speaking or while using his right hand. He was unaware of these movements until they were brought to his attention. When questioned about their purpose, the patient denied that they were voluntary. No grasping of either hand or foot was found. The patient had no cortical sensory loss.
The laboratory data including blood chemistry, haematology, and sedimentation rate were normal, as were folic acid, vitamin B12 concentrations, and thyroid function.Venereal disease research laboratory and HIV tests were negative. The cerebrospinal fluid had normal content. Brain CT showed mild cerebral atrophy. An EEG showed severe diffuse slowing at admission. Within a week, repeated EEGs showed triphasic waves with a periodic pattern of 1- 1.5 Hz.
During the next 2 weeks, the patient developed myoclonic jerks. Severe dysphasia and cognitive decline were accompanied by confusion and aggression. He became grossly ataxic, and unable to walk and perform any of his daily activities even with help. Transferred to a chronic care hospital, he died few weeks later. Postmortem examination was not allowed.
This short fatal neurological disease manifested by fulminant dementia, myoclonic jerks, and extrapyramidal and cerebellar dysfunction was strongly suggestive of CJD. The periodic EEG pattern reinforced this diagnosis. Our patient's alien hand was part of the otherwise characteristic clinical picture of CJD, but it occurred early in the disease course when no myoclonic jerks were present. We are aware of only one report of alien hand in CJD. MacGowanet al 8 described two patients with CJD with a myoclonic alien hand syndrome. In one patient the left arm “was noted to have spontaneous movements which appeared purposeful...wandered out of her view”. In the second, the alien limb performed complex actions such as unbuttoning her blouse and removing a hair pin. Although our patient had no myoclonus or pyramidal signs when the alien hand appeared, in their patients it was associated with spontaneous or stimulus sensitive myoclonus, spastic hemiparesis, and cortical sensory loss.
The literature seems to describe distinct forms of alien hand. All share the occurrence of involuntary movements contrary to the patient's stated intent, but the types of movement differ. In the callosal form, there are purposeful movements of the non-dominant hand.9 In the frontal form, there is grasping and utilisation behaviour of the dominant hand.9 In the corticobasal degeneration, there are aimless movements of either hand.5 7 When a consequence of tumorous or vascular pathology,9 alien hands can perform complex acts such as trying to tear clothes or undoing buttons. The description by MacGowanet al 8 has characteristics of the callosal form (especially in patient 2). However, our case suggests that the alien hand sign in CJD may appear in a different type, performing less complex movements which resemble those reported by Riley et al in corticobasal degeneration.7 These authors described the alien limb as “ involuntarily rising and touching the mouth and eyes” (patient 1). The patient thought that she “was powerless to stop this movement” and when directed to stop responded that “she can't”. Another patient's left arm was at times “elevated in front of him”, while he was “unaware of this situation until his attention was called to it” (patient 10).
Another related phenomenon coined as “arm levitation” was reported in progressive supranuclear palsy. In these patients the arm involuntarily raised and performed semi-purposeful movements.10
One common denominator between CJD, corticobasal degeneration, and progressive multifocal leukoencephalopathy,6 in which an alien hand sign has also been described, is multifocality. In corticobasal degeneration, it was proposed that more than one site is affected or that a “release” phenomenon occurs accounting for the aetiology of alien hand.7 In CJD, bilateral cortical damage to motor areas might be the origin of their subsequent isolation and disconnection.
We suggest that CJD should be added to the differential diagnosis of diseases presenting with an alien hand with or without myoclonus.
We are indebted to Professor Eran Zardel, Department of Physiology, University of California, Los Angeles, USA.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.