- Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging. [Journal Article]
- CCortex 2019 Feb 25; 117:33-40
- Alien limb phenomenon is a rare syndrome associated with a feeling of non-belonging and disowning toward one's limb. In contrast, anarchic limb phenomenon leads to involuntary but goal-directed movem…
Alien limb phenomenon is a rare syndrome associated with a feeling of non-belonging and disowning toward one's limb. In contrast, anarchic limb phenomenon leads to involuntary but goal-directed movements. Alien/anarchic limb phenomena are frequent in corticobasal syndrome (CBS), an atypical parkinsonian syndrome characterized by rigidity, akinesia, dystonia, cortical sensory deficit, and apraxia. The structure-function relationship of alien/anarchic limb was investigated in multi-centric structural magnetic resonance imaging (MRI) data. Whole-group and single-subject comparisons were made in 25 CBS and eight CBS-alien/anarchic limb patients versus controls. Support vector machine was used to see if CBS with and without alien/anarchic limb could be distinguished by structural MRI patterns. Whole-group comparison of CBS versus controls revealed asymmetric frontotemporal atrophy. CBS with alien/anarchic limb syndrome versus controls showed frontoparietal atrophy including the supplementary motor area contralateral to the side of the affected limb. Exploratory analysis identified frontotemporal regions encompassing the pre-/and postcentral gyrus as compromised in CBS with alien limb syndrome. Classification of CBS patients yielded accuracies of 79%. CBS-alien/anarchic limb syndrome was differentiated from CBS patients with an accuracy of 81%. Predictive differences were found in the cingulate gyrus spreading to frontomedian cortex, postcentral gyrus, and temporoparietoocipital regions. We present the first MRI-based group analysis on CBS-alien/anarchic limb. Results pave the way for individual clinical syndrome prediction and allow understanding the underlying neurocognitive architecture.
- Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration. [Case Reports]
- BCBMJ Case Rep 2019 Mar 07; 12(3)
- Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease that can mimic other neurological disorders. We present a case of sCJD in a 64-year-old man that presented with corticoba…
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease that can mimic other neurological disorders. We present a case of sCJD in a 64-year-old man that presented with corticobasal syndrome and survived for 3 years. He presented initially with dementia, hemiparkinsonism and alien limb phenomenon and was diagnosed with corticobasal degeneration, ultimately progressing to immobility and akinetic mutism. With a normal MRI 1 year before onset, his neuroimaging 1 year later revealed abnormal DaTscan, cortical and hippocampal atrophy with ventricular dilatation on MRI, and diffusion-weighted cortical ribboning and thalamic hyperintensity. Postmortem, the patient's brain was collected by the Parkinson's UK Tissue Bank. Prion protein immunohistochemistry revealed widespread diffuse microvacuolar staining without kuru-type plaques. Hyperphosphorylated tau was only found in the entorhinal cortex and hippocampus. This case highlights the clinical heterogeneity of sCJD presentation and the important inclusion of CJD in the differential diagnosis of atypical presentations of neurodegenerative disease.
- Arm Levitation as Initial Manifestation of Creutzfeldt-Jakob Disease: Case Report and Review of the Literature. [Case Reports]
- TOTremor Other Hyperkinet Mov (N Y) 2018; 8:572
- Arm levitation is an involuntary elevation of the upper limb, a manifestation of the alien-limb phenomenon. It has rarely been reported in Creutzfeldt-Jakob disease (CJD), less so as an initial manif…
Arm levitation is an involuntary elevation of the upper limb, a manifestation of the alien-limb phenomenon. It has rarely been reported in Creutzfeldt-Jakob disease (CJD), less so as an initial manifestation.
- Reversible Corticobasal Syndrome due to Coeliac Disease. [Case Reports]
- MDMov Disord Clin Pract 2018 Sep-Oct; 5(5):551-554
- Bringing order to higher order motor disorders. [Review]
- JNJ Neurol 2019; 266(4):797-805
- Majority of movements in everyday situations are complex and involve volition, planning of the movement and selection of the motor programme, all occurring before movement execution. Higher order mot…
Majority of movements in everyday situations are complex and involve volition, planning of the movement and selection of the motor programme, all occurring before movement execution. Higher order motor disorders may be defined as abnormal motor behaviours resulting from disruption of any of the cortical processes that precede execution of the motor act. They are common in patients with neurodegenerative disorders, psychiatric diseases and structural brain lesions. These abnormal behaviours may be overlooked in the clinic, unless specifically evoked by the examiner. We discuss clinical and pathophysiological aspects of higher order motor disorders including: (1) disorders of disinhibition, such as grasp reflex and grasping behaviour, utilisation and imitation behaviour, motor preservations and paratonia; (2) disorders of motor intention such as motor neglect and motor impersistence; (3) alien limb syndrome; and (4) motor overflow phenomena, such as mirror movements and synkinesias. A video illustration of each phenomenon is provided. We place the findings from recent neurophysiological studies within the framework of theories of motor control to provide better insight into pathophysiology of different disorders.
- A Wolf in Sheep's Clothing: An "Alien Leg" in Corticobasal Syndrome. [Journal Article]
- TOTremor Other Hyperkinet Mov (N Y) 2017; 7:455
- CONCLUSIONS: The inclusion of this clinical finding in recently proposed diagnostic criteria highlights the need for increased clinical awareness.
- Alien limb syndrome induced by a dopamine agonist in a patient with parkinsonism and agenesis of the corpus callosum. [Case Reports]
- PRParkinsonism Relat Disord 2017; 39:91-92
- Corticobasal syndrome due to sporadic Creutzfeldt-Jakob disease: a review and neuropsychological case report. [Case Reports]
- CNClin Neuropsychol 2017; 31(3):676-689
- CONCLUSIONS: This literature review and case report highlighted the importance of staying abreast of developments in neurological literature and the added value of neuropsychology, when integrated with newer procedures, for confirming and excluding diagnostic considerations.
- Alien limb phenomenon in pontine hemorrhage: A rare presentation. [Letter]
- NINeurol India 2016 May-Jun; 64(3):554-6
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- Novel transient alien limb phenomenon heralding a diabetic hyperosmolar non-ketotic state with leukoaraiosis: a video presentation. [Case Reports]
- BCBMJ Case Rep 2015 Nov 11; 2015