Background: Motor and cognitive deficits and consequently mobility problems are common in geriatric patients. The currently available methods for diagnosis and for the evaluation of treatment in this vulnerable cohort are limited. The aims of the ComOn (COgnitive and Motor interactions in the Older populatioN) study are (i) to define quantitative markers with clinical relevance for motor and cognitive deficits, (ii) to investigate the interaction between both motor and cognitive deficits and (iii) to assess health status as well as treatment outcome of 1000 geriatric inpatients in hospitals of Kiel (Germany), Brescia (Italy), Porto (Portugal), Curitiba (Brazil) and Bochum (Germany).
Corticobasal degeneration (CBD) is a rare and progressive neurodegenerative disease. It was originally described as a distinct clinicopathological entity in 1967 1 and later recognized as a polymorphous disease due to its complexity. In fact, the classical set of symptoms -namely asymmetric motor features associated with higher cortical function -were found to be associated with multiple histological presentations. Furthermore, the distinctive histopathological findings of cortical and striatal neuronal deposition of tau, and glial inclusions, were also linked to a wide variety of clinical phenotypes 2 . Hence, recent literature refers to corticobasal syndrome (CBS) as the constellation of symptoms described originally, limiting CBD to the distinctive histopathological findings .The different terminology with a seemingly paradoxical intrinsic overlap between these conditions makes diagnosis challenging. For instance, only 25% to 56.25% of patients with pathologically-confirmed CBD had previously presented with symptoms of CBS 4,5,6 . On the other hand, the proportion of patients diagnosed with CBS presenting with the histologic hallmarks of CBD is highly variable, ranging from 23.8% to 100% 4,5,6,7 . Considering the enormous difficulty in accurately diagnosing CBD during life, it is clear that current ABSTRACTCorticobasal degeneration (CBD) was originally described as a distinct clinicopathological entity in 1967. Since then, different phenotypic presentations have emerged as possible manifestations of CBD histopathological findings. In addition, pathophysiological findings and the molecular basis have been delineated and several aspects of its cognitive manifestations have been clarified. Thus, not only the spectrum of what is currently designated as CBD has expanded, but overlap with other degenerative and even secondary disorders has made clinical diagnostic certainty even more challenging in the absence of specific and readily-available markers. Cognitive deficits in CBD are now recognized as a frequent initial presentation and may appear up to eight years before the motor symptoms, depending on the phenotypic variant. Characteristic cognitive features of CBD involve language deficits, visuospatial and executive dysfunctions, apraxia, and behavioral disorders. Semantic and episodic memories are usually preserved, while language is often impaired in the early stages.Keywords: dementia; cognition; corticobasal degeneration. RESUMOA degeneração corticobasal (DCB) foi originalmente descrita como uma entidade clínico-patológica distinta em 1967. Desde então, nossa compreensão sobre DCB evoluiu substancialmente. Diferentes apresentações fenotípicas emergiram refletindo possíveis manifestações das anormalidades histopatológicos da DCB. Adicionalmente, dados fisiopatológicos e moleculares foram delineados e aspectos das manifestações cognitivas foram explorados. Assim, não só o espectro do que é atualmente designado DCB foi expandido, mas a sobreposição com outras doenças degenerativas e até mesmo secundárias to...
A 51-year-old man was admitted to the emergency room with an 8-hour history of sudden right hemiparesis. On examination, right upper extremity strength was graded as 1 proximally, 2 distally, 3 for the lower limb, and there were also right central facial paralysis and motor aphasia. He had a previous medical history of hypertension, dyslipidemia, type 2 diabetes, obesity, and a stroke 3 years before with mild residual right hemiparesis. A head CT showed a large fronto-parieto-temporal hypodense area consistent with a left middle cerebral artery (MCA) territory stroke (Fig. 1). A Doppler ultrasound of the carotid and vertebral arteries revealed occlusion of the left internal carotid artery. Arteriography confirmed the left MCA occlusion and revealed two critical intracranial stenosis in the right internal carotid artery. Electrocardiogram showed a slow atrial fibrillation, and an echocardiogram revealed a left ventricular intracavitary thrombus. Before admission, he was on acetyl salicylic acid 100 mg daily, subcutaneous regular insulin 30 U twice-daily, losartan 50 mg daily, and simvastatin 40 mg daily.After approximately 48 hours of admission, he developed patterned involuntary movements in the right upper limb during yawning. The movements, as shown in the video segment (see Video 1), consisted of simultaneous lifting of the entire right upper limb associated with tremor. Upon cessation of yawning, the limb returned to its resting, paretic position. The movement reoccurred continuously with every yawn, and during the episodes, the level of consciousness was preserved, as it was between them. The phenomena ceased spontaneously 12 hours after they were first noticed.The patient remained with the same neurological deficits throughout admission. He underwent proximal and distal right internal carotid artery angioplasty and subsequent anticoagulation targeting the intracavitary thrombus. Unfortunately, after a 2-month hospital stay complicated by recurrent episodes of pulmonary and urinary tract sepsis, the patient died as a result of septic shock.Here, we describe the phenomenon termed parakinesia braquialis oscitans (PBO), recently proposed by Walusinski et al.1 Descriptions of PBO are quite consistent, with jaw opening during the yawn, accompanied by elevation of the paretic limb, and subsequent drop upon ending the movement. 1-3 This phenomenon typically occurs in the upper limbs and may be associated with milder concomitant movement of the lower extremity. In terms of localization of the correspondent brain injury, PBO has been associated with lesions that affect the internal capsule, and basal ganglia and their connections, ranging from cerebrovascular, demyelinating, infectious, and expansive lesions. From a timing perspective, most cases are acute; however, they have been described even within 6 months of the injury. 4Yawning is a stereotyped, involuntary, and repetitive movement observed in almost all vertebrates. 4 It is believed that the paraventricular nucleus in hypothalamus plays a role in yawning, as...
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