Case Report of CBDer with Alien Hand Syndrome

This article on alien hand syndrome is available at no charge online. See:

http://www.plosone.org/article/info%3Ad … ne.0015010

It contains an interesting case report of someone diagnosed clinically with CBD on the basis of alien hand syndrome and declining response to levodopa (Sinemet). The case report describes in great detail how the features of this patient’s alien hand.

I’ve copied the (understandable) introduction and case report below. Further down is the abstract.

Robin

Here are excerpts:

“The alien hand syndrome (AHS) is a very rare movement disorder. Patients with AHS experience one of their limbs as alien, which acts autonomously and performs meaningful movements without being guided by the intention of the patient. The patients find themselves unable to stop the alien hand from reaching and grabbing objects without using their other hand. Patients are aware that the limb is still part of their body, but they report the feeling as if an external agent is controlling the limb. Consequently, they often describe it in the third person.”

“The phenomenon of AHS is complex and has various clinical manifestations, possibly related to different lesion sites. … The neural mechanisms of this movement disorder still remain unclear. It has been proposed that unwanted movements may arise because of a release of the primary motor cortex (M1) from conscious control by intentional planning systems.”

“Here we report data of a patient diagnosed with corticobasal degeneration and left hand AHS. His left hand showed relatively preserved volitional motor functions. Although there were spontaneous movements of the alien hand, we also had the possibility to elicit alien movements of the hand in a controlled way. We were able to evoke movements of the hand by slightly pushing the hand away from the patient’s body, which then resulted in a small movement into the opposite direction. This behavior is also known as “Gegenarbeiten”, meaning counteracting or working against. Using this reliable behavioural effect we conducted a functional magnetic resonance imaging (fMRI) study to further examine the neural correlates of unconscious or alien movements.”

“The study consisted out of two fMRI experiments. We first examined unwanted movements the way described above. The second experiment was a motor localizer scan to assess brain areas associated with conscious movements.”

Case report
“The 75-year-old right-handed gentleman (WH) was diagnosed with Parkinson’s syndrome five years ago. Within the last six months he reported a rapid loss of control of his left hand. It became much more stiffed and lost fine motor skills. When he walked down a stair he was not able to release the railway voluntary. Playing table tennis became awful. He was not able to serve because the left hand did not loose the grip of the ball. Dopaminergic medication was not as efficient as it used to be at the beginning of disease.”

“Clinically we saw an uplifted arm and reduced arm swing on the left side, strongly left sided rigidity and intermitted irregular myoclonus of the left arm. There were no signs of sensory deficit; reflexes were obtained symmetrical. Tracer studies (DAT Scan and IBZM Spect) revealed loss of presynaptic dopamine as well as a reduction of the post-synaptic dopaminergic receptor state. Structural MRI showed increased and asymmetrical ventricles. Based on the clinic and imaging we diagnosed an atypical Parkinsonian syndrome by possible corticobasal degeneration.”

“After increasing of dopaminergic medication rigidity improved but by now WH reported attacks of his left hand toward his body: the hand grabbed into his face and he could not loose the grip voluntary. When he used his right hand to release the left hand from his face the grip of the left hand became even stronger and he got scratched. He then controlled his hand during night covering up the left arm and keeping the bedside lamp turned on. Neuropsychological testing revealed intermanual conflict (the left hand did not let go objects), transitive dyspraxia using an object (i.e. hole-puncher), only slightly reduced tactile sensory, and tonic grasping. No mirror movements or synkinesis was observed.”

Here’s the abstract:

PLoS One. 2010 Dec 13;5(12):e15010.

Alien Hand Syndrome: Neural Correlates of Movements without Conscious Will.

Schaefer M, Heinze HJ, Galazky I.
Department of Neurology, Otto-von-Guericke University Magdeburg, Magdeburg, Germany.

Abstract
BACKGROUND: The alien hand syndrome is a striking phenomenon characterized by purposeful and autonomous movements that are not voluntarily initiated. This study aimed to examine neural correlates of this rare neurological disorder in a patient with corticobasal degeneration and alien hand syndrome of the left hand.

METHODOLOGY/PRINCIPAL FINDINGS: We employed functional magnetic resonance imaging to investigate brain responses associated with unwanted movements in a case study. Results revealed that alien hand movements involved a network of brain activations including the primary motor cortex, premotor cortex, precuneus, and right inferior frontal gyrus. Conscious and voluntary movements of the alien hand elicited a similar network of brain responses but lacked an activation of the inferior frontal gyrus. The results demonstrate that alien and unwanted movements may engage similar brain networks than voluntary movements, but also imply different functional contributions of prefrontal areas. Since the inferior frontal gyrus was uniquely activated during alien movements, the results provide further support for a specific role of this brain region in inhibitory control over involuntary motor responses.

CONCLUSIONS/SIGNIFICANCE: We discuss the outcome of this study as providing evidence for a distributed neural network associated with unwanted movements in alien hand syndrome, including brain regions known to be related to movement execution and planning as well as areas that have been linked to inhibition control (inferior frontal gyrus) and experience of agency (precuneus).

PMID: 21179436