Alien hand syndrome and migraine with aura: A case report

Abstract

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.

Introduction

Alien Hand Syndrome (AHS) is an uncontrollable and involuntary but seemingly purposeful motor control disorder of the upper limb (1,2). Although it is frequently seen in neurodegenerative diseases, midline tumors, and post-stroke patients, it is rarely defined in paroxysmal conditions such as epilepsy and migraine with aura (2). In this report, we would like to draw attention to this uncommon coexistence by presenting the case of a patient with a history of migraine with aura and describing findings compatible with identical AHS clinical presentation before the beginning of two separate migraine attacks.

Case

A 42-year-old, right-handed male patient presented to our clinic complaining of severe headache with a pulsating and throbbing character, mostly bilateral but sometimes unilaterally located, and increasing with physical activity. He described former episodes of migraine attacks with aura since he was 18 years old. His headache was accompanied by photophobia and phonophobia. He described blurred vision, including the appearance of dots and lines and the impression of viewing everything from behind frosted glass, and vision loss in the right visual hemifield, occurring usually less than 1 hour before pain onset.

The patient’s headache attacks recurred 1–2 times per year and were aggravated during seasonal shifts. He described them as lasting about 24 hours and improving with sleep. He had not presented to a hospital before because his attacks were not frequent and improved with sleep. Except for simple analgesics as symptomatic therapy, he used no migraine preventative medication such as triptans, ergots, or calcium channel blockers.

The patient presented to our clinic because of complaints of blurred vision and lines in the visual fields of both eyes, sudden involuntary movements of his right arm after visual loss in the right visual hemifield, and feeling as if “his arm belonged to somebody else” during two separate migraine attacks in the last week. The patient also mentioned that his right arm was acting like a puppet’s arm after a visual aura when he was driving alone. He implied that the extremity symptoms followed 10–15 minutes after the beginning of the visual aura. When he tried to change the car’s gear, his hand behaved as if it belonged to someone else, and the gear was changed involuntarily by his hand. He could not control his right hand; he was only watching it.

These complaints lasted about half an hour and concluded about 10–15 minutes before the headache began. There was no other neurologic or psychiatric comorbidity in his background other than migraine with aura, and he had no other drug or narcotic medication history and no family history of migraine. The results of the patient’s neurological and psychiatric examinations were normal, and his routine laboratory tests were normal. Brain magnetic resonance imagining (MRI) – including diffusion weighted imaging sequence (DWI) and time of flight magnetic resonance angiography (TOF-MRA) – was performed within 24 hours after the onset of symptoms, and electroencephalography (EEG) was performed within one week from the onset of symptoms, but these examinations were also normal.

The clinical condition described by the patient in his right hand immediately after the visual aura and before the beginning of the headache was assessed as an alien hand phenomenon. Only simple analgesic treatment was started as a first-line therapy because the patient did not have frequent episodes. He had no other migraine attacks with this symptomatology, and the neurological examination of the patient was completely normal after six months.

Discussion

AHS, a rare clinical condition, was first described by Goldstein in 1908. In this condition, the patient perceives his or her hand as belonging to someone else; movements of the hand appear purposeful but are actually involuntary (1,3). Patients know that their limbs are their own, but they feel like those limbs are being controlled by an external agent and are aware of these involuntary hand movements. Other movement disorders should be excluded before a final diagnosis of AHS (4).

In the clinical presentation of AHS, different concepts are defined. These include alien hand, or a feeling that the hand belongs to someone else; anarchic hand syndrome, which entails involuntary aggressive movements; conflict between hands (diagonistic dyspraxia, inter-manual conflict); and supernumerary hands, which is defined as a feeling of having three or more hands (5).

Although there are many definitions for AHS, there is no consensus on classification and diagnostic criteria (3,5). The symptoms are generally associated anatomically with the locations of brain lesions, and there is a wide anatomic distribution (3). Different locations – including the supplementary motor area (SMA), the anterior cingulate, the corpus callosum, the anterior prefrontal cortex, the posterior parietal cortex, and the thalamus – have been reported in the literature (6).

According to the clinical features and location of the lesion, three subgroups of AHS are defined: Frontal, callosal, and posterior. The frontal variant is associated with cingulate cortex or medial prefrontal cortex lesions. There is constant searching and grasping of objects, generally of the dominant hand. Conflict and opposition of hand movements is a feature of the callosal variant, and generally occurs after corpus callosum lesions. Posterior AHS is observed after cortical and subcortical involvement of the thalamus and the posterior parietal and occipital areas. In the posterior group, the feeling of the extremity belonging to a stranger, involuntary movements, and abnormal posture of the hand can be seen, and hemianopsia or neglect can coexist (2,7). In the presented case, the patient described hemianopsia as a visual aura symptom with the sense of foreign hand 10–15 minutes before the migraine headache episode, so we assumed that the patient’s condition could be compatible with the posterior variant.

Usually, the frontal variant is reported in the dominant hand, the posterior variant in the non-dominant hand, and the callosal variant in both hands. However, posterior variant cases reported in the dominant hand are also present (8). In this posterior variant case, the AHS was observed in the dominant hand of the patient.

AHS clinical symptoms are often brief in duration and end after days or weeks, and the results of the patient’s neurological examination return to normal. During this period, the symptoms may be persistent but rarely paroxysmal (9,10). In the literature, episodic AHS has been described along with epileptic seizures (11), transient ischemic attacks (12), multiple sclerosis (13), parietal lobe infarction (9), and migraine with aura (14) (Table 1). Epileptic seizures and cranial demyelinating pathologies were excluded in the presented case with clinical evaluation, EEG, and imaging findings. The clinical course of the patient, who had no cardiovascular risk factors and no imaging findings, makes a transient ischemic attack (TIA) or a possible stroke an unlikely diagnosis.

Table 1.

Cases with paroxysmal AHS in literature.

	Diagnosis	Variant	Hand	Duration	Symptoms
Raieli V et al. (14)	Migraine with aura	Posterior	L	A few minutes	Grasping
Kurne A et al. (13)	MS	Callosal	L	7–10 days	Alien hand
Demiryürek BE et al. (9)	Right parietal ischemic stroke	Posterior	L	30–40 min	Raising arm Groping object
Andre C et al. (12)	TIA	Anterior?	L	10 min	Grasping Groping
Brazdil M et al. (11)	Seizure	Anterior	L	1–3 min	Grasping Groping Scratching

MS: multiple sclerosis; TIA: transient ischemic attack; L: left.

Migraine is a primary headache disorder with episodic attacks and is accompanied by aura in about 20% of migraine patients. The symptoms of aura usually last from 5–20 minutes to 60 minutes. In some rare cases, this period can also be prolonged. Although visual auras are frequently seen, sensory-motor aura and aphasia can also occur simultaneously with the headache. Visual auras include light flashes, zigzag lines, scotomas, colored broken lines and hemianopsia (15). The underlying mechanism of migraine aura can be explained by the cortical spreading depression hypothesis. Cortical spreading depression (CSD) occurs when the depression wave spreads to the cerebral cortex, which leads to massive depolarisation and hyperexcitability of the gray matter, followed by suppression (14,16). The occipital cortex is usually the starting point of CSD before it propagates to other cortical areas, which explains the occurrence of visual auras before getting a headache. As the depression wave expands to the consecutive cortical areas, besides visual auras, other types of migraine auras associated with the affected brain region such as somatoagnosia, aphasia and misinterpretation of the body image may also be observed (17). The CSD wave involving the posterior parietal cortex and the primary motor area may result in posterior AHS. İn our case, the visual aura persisted for 15 minutes and was followed by the AHS, which also persisted for another 15 minutes before the headache began, consistent with the CSD wave propagation rate.

Reports on the coexistence of AHS with headache are very rare in the literature. Raieli et al. defined a case of migraine with aura and frontal variant AHS (14), and Vincent et al. defined AHS as an accompanying feature of the migraine aura due to CSD (18). In the former, a visual aura is followed by hypoesthesia and a bilateral AHS with bilateral migraine headache (14,18). Clinical features of our case are similar to this case.

No specific treatment exists for AHS. The basics of symptomatic treatment are defined as the correction of the underlying etiology, botulinum toxin injections, benzodiazepines and cognitive rehabilitation (2). The patient’s migraine attacks were aggravated during seasonal shifts, and it was the end of the summer when the patient was admitted. We suggested simple analgesic treatment to the patient because of his rare frequency of migraine attacks (1–2 per year) and observed no other migraine attacks with AHS during a follow-up period of 6 months.

In conclusion, migraine association with AHS is a rare condition, although many diseases have been defined in etiology. Episodic AHS in particular is an exceptional phenomenon, and can be observed in epileptic seizures, TIA and migraine with aura. We presented our case report to draw attention to this rare coexistence.

Clinical implications

Coexistence of AHS and migraine with aura is a very rare paroxysmal condition.

The CSD wave involving the posterior parietal cortex and the primary motor area may result in posterior AHS.