Case Report: Callosal disconnection syndrome manifesting as mixed frontal-callosal-posterior alien hand syndrome following extensive corpus callosum infarct. 2023

Muhamad Faizal Zainudin, and Kar Chuan Soo, and Khin Nyein Yin
Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh, Selangor, Malaysia.

Alien hand syndrome (AHS) is a rare neurological phenomenon first described by Goldstein over a century ago. The most widely recognized variants in literature are frontal, callosal, and posterior AHS. AHS due to the corpus callosum lesion can occur alone or as part of callosal disconnection syndrome (CDS). This report presents a unique CDS case manifesting clinical features from all three AHS variants, resulting from an extensive corpus callosum infarct. Our patient exhibited various clinical features from the three AHS variants, which include grasping, groping, and difficulty releasing objects from the hand (anterior); intermanual conflict (callosal); arm levitation, mild hemiparesis, and hemisensory loss (posterior). Additionally, the extensive disruption of the corpus callosal fibers produced neurological manifestations of CDS, such as cognitive impairment, ideomotor and constructional apraxia, behavioral disorder, and transcortical motor aphasia. We employed a range of rehabilitation interventions, such as mirror box therapy, limb restraint strategy, verbal cue training, cognitive behavioral therapy, bimanual hand training, speech and language therapy, and pharmacological treatment with clonazepam. The patient showed almost complete resolution of CDS and AHS features by nine months post-stroke Our case report highlights distinctive clinical variations of AHS and the challenging correlation between clinical manifestations and neuroanatomical substrates. Future studies are necessary to explore the intricate neural connections and the precise function of the corpus callosum. This can be achieved by combining comprehensive neuropsychological testing with diffusion tensor tractography studies. It is also essential to develop a validated tool to standardize AHS assessment. Finally, the scarcity of evidence in rehabilitation interventions necessitates further studies to address the wide knowledge gap in AHS and CDS management.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D003337 Corpus Callosum Broad plate of dense myelinated fibers that reciprocally interconnect regions of the cortex in all lobes with corresponding regions of the opposite hemisphere. The corpus callosum is located deep in the longitudinal fissure. Interhemispheric Commissure,Neocortical Commissure,Callosum, Corpus,Callosums, Corpus,Commissure, Interhemispheric,Commissure, Neocortical,Commissures, Interhemispheric,Commissures, Neocortical,Corpus Callosums,Interhemispheric Commissures,Neocortical Commissures
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D055964 Alien Limb Phenomenon An apraxia characterized by the affected limb having involuntary, autonomous, and seemingly purposeful behaviors that are perceived as being controlled by an external force. Often the affected limb interferes with the actions of the normal limb. Symptoms develop from lesions in the CORPUS CALLOSUM or medial frontal cortex caused by stroke, infarction, and neurodegenerative diseases (e.g., CREUTZFELDT-JAKOB SYNDROME, corticobasal degeneration). Alien Hand Phenomenon,Alien Hand Syndrome,Alien Leg Syndrome,Alien Limb Syndrome,Phenomenon, Alien Hand,Phenomenon, Alien Limb,Syndrome, Alien Hand,Syndrome, Alien Leg,Syndrome, Alien Limb,Syndrome, Anarchic Hand,Anarchic Hand Syndrome,Alien Hand Phenomenons,Alien Hand Syndromes,Alien Leg Syndromes,Alien Limb Phenomenons,Alien Limb Syndromes,Anarchic Hand Syndromes,Leg Syndrome, Alien

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