Pathophysiology and Treatment of Alien Hand Syndrome

Harini Sarva; Andres Deik; William Lawrence Severt

doi:10.5334/tohm.225

Figures & Tables

Table 1

Summary of Reported Cases of Alien Hand Syndrome

Reference	Case No.	Diagnosis	Localization of Lesion or Imaging Findings	Hand	Diagnostic Dyspraxia or Intermanual Conflict	Grasping	Levitation	Groping	Utilization Behavior
Goldstein69		Infarction		L	X	X
Akelaitis70	1	Callosotomy	Body of corpus callosum, genu of corpus callosum	L	X
	2	Callosotomy	Complete section of corpus callosum	R	X
Goldberg et al.71	1	Infarction	L medial frontal cortex	R	X	X		X
	2	Infarction	L medial frontal cortex	R		X			X
Levine and Rinn23		Infarction	R temporal/occipital cortex, posterior thalamus	L				X
McNabb, et al.72		Infarction	L superior and medial frontal cortex and parietal	R		X		X
Banks et al.36	1	Gunshot wound	Medial frontal white matter bilaterally, corpus callosum, R basal ganglia, internal capsule and thalamus	L		X
	2	Ruptured anterior communicating Artery Aneurysm	Genu of corpus callosum, bilateral medial frontal cortex, R gyrus rectus	L	X			X	X
Goldberg and Bloom73	1	Infarction	Body of corpus callosum, R medial frontal cortex, supplementary motor area, anterior cingulate gyrus	L	X	X			X
	2	Infarction	L medial frontal cortex, supplementary motor area, anterior cingulate gyrus	R		X		X
	3	Infarction	Corpus callosum, R medial frontal cortex, supplementary motor area, anterior cingulate gyrus	L		X		X
	4	Infarction	Corpus callosum, L medial posterior frontal cortex, supplementary motor area, anterior cingulate gyrus	R		X		X
Kuhn et al.74		Infarction	Body of corpus callosum, genu of corpus callosum, bilateral anterior medial frontal cortex, supplementary motor area	R		X
Della Sala et al.75		Ruptured anterior communicating artery aneurysm	Anterior corpus callosum, bilateral frontal cortex, L medial frontal cortex, supplementary motor area	R		X		X
Hanakita and Nishi76		Infarction	Corpus callosum, L medial frontal cortex, cingulate gyrus	L	X
Feinberg et al.14		Infarction	Corpus callosum, L medial frontal cortex	R		X		X
Gottlieb et al.77	1		Possible corpus callosum, R fronto-parietal cortex	L	X			X	X
	2		Body of corpus callosum, bilateral medial frontal cortex	L	X
Leiguarda et al.39	1	Idiopathic cortical atrophy	L medial frontal cortex atrophy	R		X
	2	Diffuse lymphocytic lymphoma	R medial frontal cortex	L		X
	3	Seizures after the removal of an arteriovenous malformation	L posterior parietal cortex	R			X
	4	Ruptured arteriovenous malformation	R parietal/temporal cortex	L			X
Dolado et al.78		Infarction	Bilateral parietal	L			X	X
Green et al.79		Alzheimer's Disease	R parietal cortex, mild frontal	L
Ventura et al.80		Hemorrhagic stroke	R capsulothalamic area, R frontal/temporal/parietal cortex	L			X
Geschwind et al.16		Infarction	Body of corpus callosum, R cingulate cortex	L				X
Giroud and Dumas81	7	Infarction	Corpus callosum, fronto-parietal ischemia	L		X
	8	Infarction	R anterior corpus callosum, R frontal cortex	L		X
Nagumo and Yamadori82		Infarction	L body of corpus callosum, genu of corpus callosum	L		X
Papagno and Marsile83		Ruptured Anterior Communicating Artery Aneurysm	Anterior corpus callosum, R medial frontal cortex	L	X
Chan et al.2		Infarction	Body of corpus callosum, genu of corpus callosum, isthmus of corpus callosum, R supplementary motor area, anterior cingulate gyrus, bilateral medial prefrontal cortex	L	X	X		X
Kischka et al.84		Infarction	Anterior corpus callosum, L medial frontal cortex, parieto-occipital, supplementary motor area	R		X		X	X
Tanaka et al.85	1	Infarction	Post body of corpus callosum, anterior splenium of corpus callosum, L cingulate gyrus, temporoparietal	L	X
	2	Infarction	Body of corpus callosum, R cingulate gyrus, L anterior cingulate gyrus, bilateral medial frontal cortex	L	X			X
	3	Infarction	Body of corpus callosum, genu of corpus callosum, L anterior cingulate gyrus	L	X
	4	Infarction	Anterior body of corpus callosum, L medial frontal cortex, anterior cingulate gyrus	R		X		X	X
	5	Infarction	Anterior body of corpus callosum, L medial frontal cortex	R		X		X
	7	Infarction	Anterior body of corpus callosum, R medial frontal cortex, anterior cingulate gyrus	L		X		X	X
Andre and Domingues86		Reversible ischemia	L internal capsule, R occipital cortex, L cerebellum	L				X
Chan and Ross87	1	Infarction	Genu of corpus callosum, isthmus of corpus callosum, L medial frontal cortex, supplementary motor area, anterior cingulate gyrus	R, L	X (L)	X (R)
	2	Infarction	Body of corpus callosum, genu of corpus callosum, R medial frontal cortex, supplementary motor area, anterior cingulate gyrus	L		X		X
	3	Infarction	Genu of corpus callosum–isthmus of corpus callosum, R prefrontal cortex, supplementary motor area, anterior cingulate gyrus	L	X	X		X
Feinberg et al.9		Seizures in the setting of a glioblastoma multiforme	R frontotemporal cortex	L		X		X
Nicholas et al.88		Infarction with hemorrhagic conversion	Corpus callosum	L		X		X
Tow and Chua89		Infarction	L medial frontal cortex and posterior parietal cortex	R		X		X
Ay et al.90		Infarction	Splenium of corpus callosum, R posterior frontal/temporal/occipital/parietal cortex, thalamus	L				X
Groom et al.52		Infarction	R medial posterior temporal cortex, posterior parietal/occipital cortex	L				X
Marti-Fabregas et al.91		Infarction	R inferior parietal cortex	L		X		X
Fisher92	1	Corticobasal syndrome		L, R	X (R)	X (L)
	2	Corticobasal syndrome		R	X
	3	Corticobasal syndrome		L	X		X
	4	Corticobasal syndrome		L				X
	5	Corticobasal syndrome		L, R	X (L)	X (both)
	6	Corticobasal syndrome		L	X	X
Ong Hai and Odderson93		Infarction	Anterior corpus callosum, R medial frontal cortex	L		X		X
Bundick and Spinella43		Infarction	R posterior parietal/temporal cortex, medial frontal cortex	L			X
Inzelberg et al.94		Creutzfeld–Jakob disease	Mild cerebral atrophy	L			X
Carrilho et al.3	1	Corticobasal syndrome	R temporal/parietal cortex	L			X
	2	Corticobasal syndrome	R temporal/parietal cortex	L			X
	3	Alzheimer's disease	L parietal cortex	R			X
	4	Infarction	R parietal cortex	L			X
Kumral27		Moya-Moya leading to a hemorrhage	Body of corpus callosum, genu of corpus callosum, splenium of corpus callosum, R medial frontal cortex (unclear)	L		X		X
Nishikawa et al.95	1	Infarction	Body of corpus callosum, genu of corpus callosum, splenium of corpus callosum	L	X
	3	Resection of a central neurocytoma via transcallosal and transcortical approaches	Anterior body of corpus callosum, splenium of corpus callosum, L parietal cortex	R				X	X
Lavados et al.96		Hemorrhage	Corpus callosum, F medial (inferred)	L, R	X (L or R)	X (R)		X (R)	X (R)
Suwanwela and Leelacheavasit97	1	Infarction	Body of corpus callosum, splenium of corpus callosum	L	X
	2	Infarction	Body of corpus callosum	L	X
Marey-Lopez et al.50		Infarction	R thalamus	L			X	X
Pack et al.68		Infarction	R lateral thalamus	L			X
Rohde et al.20		Presumed vascular-mediated cortical atrophy	L central/parietal cortical and subcortical lesions and extensive atrophy of the pre- and post-central gyri	R			X
Pappalardo et al.56		Infarction	L cortical–subcortical parieto-occipital lobes	R	X	X
Giovannetti et al.37		Infarction	L medial frontal extending into the corpus callosum	R	X	X		X	X
Iwashita et al.98		Infarction	R frontal lobe	L
Espinosa et al.99		Infarction	R parietal lobe and anterior corpus callosum	L	X
Kikkert et al.64	1	Infarction	Presumed R parietal lesion	L	X		X		X
	2	Infarction	L frontal lobe in the area of the anterior cerebral artery	R	X	X
Kurne et al.100		Multiple Sclerosis	Non-enhancing multiple hyperintense lesions in the corpus callosum, periventricular white matter and centrum semiovale on MRI	L			X
Caixeta101		HIV	R frontal and parietal hypodensity on CT	L	X	X	X
Bejot et al.102		Infarction	Bilateral hyperintense signals in diffusion-weighted images in the anterior cerebral artery territories involving the R anterior corpus callosum	L	X
Kessler et al.47		Infarction	L anterior parietal lobule and posterior lip of post-central gyrus	R		X
Soman et al.103		Infarction	R parietal lobe, dorsal putamen, and body of caudate	L	X
Spector et al.24		Infarction	R parietal lobe	L				X
Haq et al.61		Brain herniation	R cerebral peduncle gliosis and L cystic anterior thalamus changes	R		X	X
Takenouchi and Solomon11		Parry Romberg Syndrome	Progressive atrophy and gliosis of the R thalamus	L	X		X
Kim et al.104		Infarction	R parietal	L	X			X
Faber et al.105		Infarction	L paracallosal area	R	X
Terazzi et al.106		Infarction	R thalamus and calcarine cortex	L	X	X	X
Cantagallo et al.65		Hemorrhage	Fronto-basal area bilaterally, L fronto-opercolar area and L frontal superior area	R	X
Prakash et al.19		Corticobasal syndrome	Asymmetrical R fronto-parietal cortical atrophy	L				X
Pooyania et al.62		Infarction	L superior frontal gyrus	R	X	X			X
Bartolo et al.51		Infarction	L posterior thalamus, parahippocampal gyrus, inferior and posterior temporal lobe, lateral portion of the splenium of the corpus callosum, and cortical part of the occipital lobe.	R			X	X
Yuan et al.22		Infarction	Genu, body and splenium of the corpus callosum	L	X
Verleger et al.42		Infarction	L side of corpus callosum from genu to posterior isthmus	L	X
Huang and Jia15		Cerebral venous malformation	Hemorrhage in the genu and body of the corpus callosum	L	X
Nandhagopa et al.17		Infarction	Bilateral parietal parasagittal areas initially, body of corpus callosum found to be involved later	L	X
Rubin et al.26	1	Creutzfeld–Jakob disease	Diffuse cortical T2 signal on MRI	L
	2	Creutzfeld–Jakob disease	Normal	L
	3	Creutzfeld–Jakob disease	Bilateral striatum, diffuse cortex left hemisphere	R
	4	Creutzfeld–Jakob disease	Normal	L
	5	Creutzfeld–Jakob disease	Unknown	L
	6	Creutzfeld–Jakob disease	R posterior parietal cortical hypoattenuation on CT	L
	7	Creutzfeld–Jakob disease	Normal imaging	R
	8	Creutzfeld–Jakob disease	Cortical and striatal DWI/FLAIR hyperintensity Diffusely on MRI	L
	9	Creutzfeld–Jakob disease	Bilateral L> R selective parietal restricted diffusion on MRI	R
	10	Creutzfeld–Jakob disease	Restricted diffusion in bilateral striatal nuclei, left parietal cortex on MRI	R
	11	Creutzfeld–Jakob disease	Diffuse restricted diffusion, T2 hyperintensity of R posterior hemispheric cortex on MRI	L
	12	Creutzfeld–Jakob disease	Restricted diffusion, T2 hyperintensity of bilateral R> L parietal, occipital lobes on MRI	L	X		X
	13	Creutzfeld–Jakob disease	Diffuse cortical restricted diffusion on MRI	L
Nash et al.7		Pneumocephalus	R frontal lobe	L					X
Hertz et al.107		Infarction	L posterior middle cerebral artery territory infarction	R	X
Park et al.1	1	Infarction	L medial frontal white matter infarction	R		X
	2	Infarction	R frontal lobe and anterior corpus callosum infarction	L	X
Shereef and Cavanna48		Infarction	R posterior parietal	L
Jang et al.41		Infarction	Anterior portion of the L cingulated gyrus and the corpus callosum (posterior portion of the genu to the anterior portion of the splenium)	R		X		X	X
Graff-Radford et al.46	108 cases	Corticobasal syndrome
	14 cases	Cerebrovascular	Ischemic strokes, arteriovenous malformation and subdural hematoma
	9 cases	Creutzfeld–Jakob disease
	5 cases	Hereditary Diffuse Leukoencephalopathy with Spheroids
	4 cases	Tumor	Astrocytomas and oligodendroglioma
	10 cases	Other
McBride et al.38		Corticobasal syndrome	Asymmetric atrophy of L parietal regions	R		X
Nowak et al.67	1	Infarction	L anterior corpus callosum, left anterior paramedian periventricular white matter and adjacent paramedian cortex	R	X	X		X
	2	Infarction	L anterior corpus callosum and overlying white matter	R	X	X
Lunardelli et al.108		Multiple Sclerosis	Multiple corpus callosal lesions, especially of caudal portion	L	X
Chokar et al.109		Cerebral vasculitis	R hemispheric cortical and subcortical edema affecting the white matter and temporal, parietal, and occipital lobes	L		X
Romano et al.66		Hemorrhage	L fronto-parietal hemorrhage	R			X	X

[i] Abbreviations: CT, Catscan; DWI, Diffusion-weighted Imaging; FLAIR, Fluid-attenuated Inversion Recovery; HIV, Human Imunodeficiency Virus; L, Left; MRI, Magnetic Resonance Imaging; R, Right; X, finding present in that case.

Table 2

Variants of Alien Hand Syndrome

Type	Commonly Affected Areas	Common Causes	Symptoms and Signs
Frontal	Supplemental motor area	Tumors	Groping
	Cingulate gyrus	Infarction	Grasping
	Corpus callosum	Trauma	Utilization behavior
Callosal	Corpus callosum	Callosotomy	Intermanual conflict
		Tumors
		Infarction
Posterior	Parieto-occipital cortices	Infarction	Levitation
	Thalamus	Creutzfeld–Jakob disease	Cortical sensory deficits
		Corticobasal syndrome	Abnormal posturing of the limb

Table 3

Treatment Modalities Described in Different Alien Hand Syndrome Variants

Variant	Therapeutic Modality
Anterior	Sensory tricks
	Distracting tasks
	CBT for anxiety control
	Verbal cues
Posterior	Botulinum toxin A
	Clonazepam
	Visualization strategies
	Spatial recognition tasks

[i] Abbreviation: CBT, Cognitive Behavioral Therapy.