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Sensory Alien Hand Syndrome: Case Report and Review of the Literature

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Sensory Alien Hand Syndrome: Case Report and Review of the Literature 366 J Neurol Neurosurg Psychiatry 1998;65:366–369 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.3.366 on 1 September 1998. Downloaded from SHORT REPORT Sensory alien hand syndrome: case report and review of the literature Hakan Ay, Ferdinando S Buonanno, Bruce H Price, Dean A Le, Walter J Koroshetz Abstract hand. She made no attempt to look at her hand An 81 year old right handed woman devel- to correct the movements. She held her left oped a left alien hand syndrome character- hand with the right, claiming to keep “him” ised by involuntary movements of choking from hitting her. When asked what trouble and hitting the face, neck, and shoulder. brought her to the hospital, she said that her The patient showed multiple disorders of “left hand tried to strangle her”. She repeated primary sensation, sensory processing, that “someone” was hitting and choking her hemispatial attention, and visual associ- neck, face, and shoulder. She asked the nurse ation, as well as a combination of sensory, to restrain her left hand, fearful that “hitting of optic, and cerebellar ataxia (triple ataxia) the breast will cause cancer”. These intermit- of the left arm in the absence of motor tent movements had an irregular speed with neglect or hemiparesis. Imaging studies slow and smooth onset but became more jerky disclosed subacute infarction in the right and rapid before hitting her body. thalamus, hippocampus, inferior temporal On examination, she was alert, with right lobes, splenium of corpus callosum, and gaze preference, severe left dysmetria with side occipital lobe due to right posterior cer- to side swaying and dysdiadocochinesia, hemi- ebral artery occlusion. This rare syndrome spatial neglect, hemianopia, and was anosog- copyright. should be considered as a “sensory” or nosic of any deficits. She moved less often on “posterior” form of the alien hand syn- the left but was not hemiparetic. She displayed drome, to be distinguished from the severe misreaching with either arm. She “motor” or “anterior” form described showed the right hand when asked to show the more commonly. left and visually could not distinguish her left (J Neurol Neurosurg Psychiatry 1998;65:366–369) hand from the examiner’s. Her left hand could not correctly imitate postures imposed on the Department of Keywords: alien hand syndrome; posterior; ischaemic Neurology, Stroke stroke; posterior cerebral artery right hand. Her ability to copy simple diagrams Service, VBK-802 with eyes open was characterised by a series of Massachusetts General irregular pen strokes; constructions were some- Hospital, Harvard Although several studies have reported on what better when performed with her eyes http://jnnp.bmj.com/ Medical School, patients with alien hand syndrome since Gold- closed. She could not recognise the faces of 32 Fruit Street, Boston 1 well known persons from photographs. When MA 02114, USA stein’s initial description, all but three were 2–5 HAy due to frontal or anterior callosal lesions. shown an advertisement with two children in a F S Buonanno Here we describe the clinical, neuroanatomi- car, she saw only the rightward child and did DALe cal, and PET findings in a patient with alien not perceive the car. Visual identification of W J Koroshetz hand syndrome due to multiple disorders of colours and objects were normal. Her attempts primary sensation and sensory processing after to visually fix on objects in the room to Department of command were delayed and inaccurate. There on September 26, 2021 by guest. Protected Neurology, McLean right posterior cerebral artery infarction. Hospital, was severe primary sensory loss on the left side. 115 Mill Street, Case report She extinguished to double simultaneous Belmont, MA An 81 year old right handed woman, with an stimuli over the left side. 02178–9106, USA unremarkable medical history, developed spells On the fifth hospital day, she was still afraid BHPrice of left face, arm, and leg tingling. On admission of her left hand despite cessation of the abnor- Correspondence to: elsewhere, she was confused and disoriented to mal movements. She placed a pillow on her Dr Ferdinando S Buonanno, place; she misidentified her daughter as a chest for protection. Her misreaching with Department of Neurology, nurse; and had left sided numbness, mild either hand and ability to fixate visually Stroke Service, VBK-802 Massachusetts General weakness, and clumsiness of the arm, spatial improved. Left arm dysmetria was less promi- Hospital, 32 Fruit Street, neglect, and a homonymous hemianopsia. On nent. Anosognosia, sensory deficits, left hemis- Boston MA, 02114, USA. the second day, she complained that her left patial neglect, and the left visual field cut Telephone 001 617 726 84 59; fax 001 617 726 50 hand was acting as if under someone else’s remained unchanged. She continued to exhibit 43. control; it hit her face and head and she intermittent disorientation to place; talked to reported that she was afraid of it. herself as if she was working at home, misiden- Received 8 September 1997 On transfer to the Massachusetts General tified her hospital room as her home library or and in revised form 6 January 1998 Hospital, she occasionally scratched her left kitchen, and was unable to point to the window Accepted 14 January 1998 face and stroked her left forehead with her left and door as directed. By the end of the second Sensory alien hand syndrome 367 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.3.366 on 1 September 1998. Downloaded from copyright. http://jnnp.bmj.com/ Figure 1 Axial FLAIR-MR images (TR:10002, TE:141/Ef) show high signal indicating a subacute infarction in the right thalamus, hippocampus, inferior-medial temporal lobes, splenium of the corpus callosum, and occipital lobe (from top right to bottom left in ascending order). hospital week, she no longer feared her left FDG uptake in the regions known to be hand. Her appendicular ataxia, visuo-spatial infarcted (fig 2); in addition, there was a subtle on September 26, 2021 by guest. Protected orientation, and left hemineglect improved. decrease of FDG uptake in the right posterior Primary sensory deficits remained. frontoparietotemporal regions. Brain MRI at admission showed a subacute infarction in the right thalamus, hippocampus, Discussion inferior temporal lobes, splenium of the corpus Alien hand syndrome is defined as unwilled, callosum, and occipital lobe (fig 1). Two uncontrollable, but seemingly purposeful dimensional phase contrast magnetic reso- movements of an upper limb. Two major crite- nance angiography visualised the proximal P-1 ria for the diagnosis are complaint of a foreign but not distal P-1 or P-2 segments of the right limb and complex, autonomous, involuntary posterior cerebral artery. A definite cause for motor activity that is not part of an identifiable the posterior cerebral artery occlusion was not movement disorder. A verbally expressed uncovered, although a polymerase chain reac- feeling that the movements are not under self tion based test disclosed that she was hetero- control and personification of the arm, also zygous for factor V Leiden mutation. Ten days occur.6 According to this definition, our patient after her admission, a brain PET study was fulfills the criteria for alien hand syndrome. performed using 5.3 mCi 18F-fluorode- Our patient diVers substantially from those oxyglucose (FDG). It showed focally decreased described in the literature. A recent review of 368 Ay, Buonanno, Price, et al J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.3.366 on 1 September 1998. Downloaded from copyright. Figure 2 Brain PET images (18FDG; 6 mm thick sections reconstructed at 8 mm resolution FWHM; GE Medical Systems 4096 tomograph) show regions of decreased uptake in the right occipital lobe, the right thalamus, and the right inferior mesial temporal lobe. There was also subtle but widespread decrease in uptake in the right hemispheric cortex. (Courtesy of S Weise,MGH PET Centre.) 20 cases showed that all but one of the respon- involving the corpus callosum and posterior sible cerebral lesions were frontal and always internal capsule sparing the thalamus. The exact involved the anterior corpus callosum, usually arterial territory involved was not specified. http://jnnp.bmj.com/ with additional involvement of the supplemen- There were left hemisensory deficits and hemi- tary motor area, anterior cingulum, and medial inattention. The patient thought that the hand prefrontal cortex.7 In the classic syndrome, the with coarse involuntary movements had hostile main symptom is the report of alienness with motivations. Ventura et al11 described a patient abnormal movements of the hand; persistent with a right thalamomesencephalic haemor- groping, compulsive manipulation of tools, rhage, moderate hemisensory deficits, and intermanual conflict, and restraining actions of movements that were interpreted as imitation 68 the normal limb. synkineses; also, the patient presented spontane- on September 26, 2021 by guest. Protected As opposed to the frontal/callosal alien hand ous left arm movements perceived as a levitation cases in which motor plans are abnormally acti- “as if someone else was moving the arm”; there vated and callosal pathology prevents normal was no anosognosia, nor hemiasomatognosia; a transfer to the opposite hemisphere, our patient PET scan 7 weeks after admission showed right had a complicated sensory disorder with self frontoparietotemporal cortical hypometabo- stimulation from the neglected side. A similar lism, most pronounced in the sensorimotor area. clinical syndrome due to posterior cerebral However, it is not only the misperception of artery infarction was reported by Levine and abnormal movement that defines alien hand Rinn9 in a right handed woman with a homony- syndrome, but what makes it unique is the mous hemianopia, hemianaesthesia, and optico- patient’s subjective acknowledgement that the sensory ataxia due to proximal right PCA limb is “not self”, and the self destructive or infarction.
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