Paroxysmal Alien Hand Syndrome: Case Report and Literature Review.

Dear Editor,

Alien hand syndrome (AHS) is a complex and unusual neurological phenomenon. In this condition the patient’s limb moves unintentionally, as if it has its own will.1 The common etiologies of AHS are brain tumors, neurosurgeries, and neurodegenerative diseases, and rarely strokes.2 Here we present an unusual case of AHS as an uncommon aura type of migraine, which manifested in a paroxysmal manner.

A 44-year-old male presented with a 2-month history of the thought that his left hand was acting independently of him and did not belong to him. When he was making a hand gesture, he experienced like a puppet hand, a throwing sensation. This condition scared him into thinking that he was going to lose his mind. He said that he had recently visited a psychiatrist, and was started on 5 mg of aripiprazole daily, which resulted in him having a desire to stay still while moving constantly. This was relieved when 5 mg of diazepam was administered intravenously to the patient in the emergency room for his akathisia. Quetiapine was started at 12.5 mg per day, which decreased his anxiety but did not resolve his strange complaint. However, more detailed history-taking revealed that the patient had experienced headaches for about 2 years, for which he had not receive regular treatment. A neurology consultation clarified that each headache attack appeared approximately 20 minutes after the left-sided strange feeling initiate. The patient stated that the headache was generally unilateral (alternating side of the head) but sometimes involved bilateral throbbing and pulsating pattern with nausea, also with photophobia, phonophobia, and osmophobia. The headache resolved with analgesics after 2 or 3 hours; otherwise it took 8–14 hours to resolve. Also, the headaches always aggravated with physical activity. The patient’s psychotropic drugs were discontinued. Electroencephalography, magnetic resonance imagining (MRI), and magnetic resonance angiography findings were within the normal ranges. His past medical history indicated that he had not previously experienced any other type of headache or aura. Over the previous 2 months had he experienced this hand symptom four times but the headache eight or nine times. Prophylactic migraine treatment with topiramate at 50 mg per day was started. Also, migraine-attack treatment (10 mg of domperidone and 600 mg of ibuprofen) was added as needed. The patient was followed up for 6 months with these medications, after that he reported that his left-sided hand symptom and headaches completely resolved.

AHS, which is also called Dr. Strangelove syndrome after one of Stanley Kubrick’s movies, is a rare and unusual condition in which an extremity of a person moves independently as if it has its own will.12 AHS is categorized into anterior (frontal), callosal, and posterior variants. The anterior variant appears as a searching, grasping movement, and is associated with lesions in the cingulate cortex and/or medial prefrontal cortex.23 The callosal variant manifests as disagreement movements of the hand, in the presence of corpus callosum lesions, while the posterior variant feels like the extremity, belongs to a stranger, and appears as an abnormal posture of the hand, which can accompany hemineglect and hemianopsia.

Posterior structures as the occipital, parietal lobes, and thalamus can be affected.345 Functional neuroimaging techniques such as functional MRI (fMRI) normally reveal the involvement of a complex neural network in a simple motor activity; however, fMRI in AHS shows only contralateral activation of the motor cortex.24 Table 1 lists paroxysmal AHS cases reported in the literature with this classification, and also their similarities and differences. The small number of previous case reports highlight that paroxysmal AHS can occur in different types of primary neurological diseases, with the duration ranging from minutes to days. Pathophysiologically, this condition can be observed as a result of direct damage to the relevant neuroanatomical structures, or due to exposure to a pathological wave that changes the polarization of these regions.5678910 A functional neuroimaging study has not been performed because this aura-like symptom occurs infrequently and only temporarily. We speculated that this might be caused by cortical spreading depression (CSD) that occurs because of hyperexcitable cortical neurons in migraine with aura. From the point of view of our case, the CSD might be thought to affect the posterior parietal regions for this aura.156

Table 1

Comparison of paroxysmal AHS cases in the literature

	Age (yr), sex	Duration	Imaging	Etiology	Variant	Management	Outcome
Erdal et al.5	42, male	30 minutes	Normal	Migraine with aura	Posterior	Simple analgesia	No further attack and completely resolved after 6 months
Raieli et al.6	13, female	A few minutes	Normal	Migraine with aura	Posterior	No therapy	Unknown
Kurne et al.7	34, female	7–10 days	Demyelinating CC and PV lesions (no Gd+)	Multiple sclerosis	Callosal	No therapy	Resolved gradually and spontaneously after 10 days
Demiryürek et al.8	71, male	30–40 minutes	Ischemic right posterior parietal lobe lesion	Ischemic stroke (AHS started at 1 month after stroke)	Posterior	ASA 100 mg/day, CLZ 1 mg/day and spatial recognition exercises	Frequency of symptoms decreased but not resolved
André and Dominques9	62, male	10 minutes	Normal MRI. MRA revealed narrowing of right ICA proximal segment	Transient ischemic attack	Anterior	ASA 200 mg/day	No further attack and completely resolved
Brázdil et al.10	61, male	1–3 minutes	Ischemic right rostral CMA lesion	Seizure due to an ischemic lesion	Anterior	LEV (dose unknown)	Symptoms completely disappeared after LEV initiated

AHS, alien hand syndrome; ASA, acetylsalicylic acid; CC, corpus callosum; CLZ, clonazepam; CMA, cingulate motor area; Gd+, gadolinium enhancement; ICA, internal carotid artery; LEV, levetiracetam; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging.

In conclusion, AHS can manifest in a paroxysmal manner as a part of a paroxysmal neurological condition such as migraine with aura. We believe that the present case creates a paradigm for future studies of aura types of migraine, and also could reveal the pathophysiology of this unusual condition.