Fortunately, this group of disorders is often medically or surgically treatable. In this article, we review the current data regarding clinical phenotypes, differential diagnosis, neurodiagnostic workup, and potential therapeutic options for this unique, most disturbing, and infrequently reported disorder.Ĭopyright © 2020 by the American Academy of Pediatrics. These abnormalities can lead to vestibular symptoms such as dizziness, imbalance and difficulty focusing the eyes, as well as hearing symptoms such as hearing loss and sensitivity to loud sounds. Both psychiatric therapy and immunotherapies have been described as DSDD treatments, with both revealing potential benefit in limited cohorts. The etiology of DSDD is unknown, but in several hypotheses for regression in this population, psychological stress, primary psychiatric disease, and autoimmunity are proposed as potential causes of DSDD. No strict criteria or definitive testing is currently available to diagnose DSDD, although a comprehensive psychosocial and medical evaluation is warranted for individuals presenting with such symptoms. The acute phase is followed by a chronic phase in which baseline functioning may not return. This condition has a subacute onset and can include symptoms of mood lability, decreased participation in activities of daily living, new-onset insomnia, social withdrawal, autistic-like regression, mutism, and catatonia. Initially reported in 1946 as "catatonic psychosis," there has been an increasing interest among the DS community, primary care, and subspecialty providers in this clinical area over the past decade. Motor-sensory cortex: model and validation.Down syndrome disintegrative disorder (DSDD), a developmental regression in children with Down syndrome (DS), is a clinical entity that is characterized by a loss of previously acquired adaptive, cognitive, and social functioning in persons with DS usually in adolescence to early adulthood. Kanazawa I (2001) Hemispheric lateralization KR (1999) Localization and characterization Perisylvian cortex: a functional magnetic (1999) Brain regions involved in articulation. Stroke: lesion topography, clinicoradiologic Syndrome (pure anarthria): anatomo-clinical report of a historical Cortico-cortical fibers connecting face-M1 with the lower premotor areas including Broca's area may also be important for articulatory control. The cases suggest the existence of a localized brain region specialized for articulation near face-M1. Overlay of magnetic resonance images revealed a localized cortical region near face-M1, which displayed high intensity on diffusion weighted images, while the main portion of the corticobulbar fibers arising from the lower third of the motor cortex was preserved. The speech disturbance was limited to verbal output, without aphasia or orofacial apraxia. There was a mild deficit in tongue movements in the sagittal plane that impaired palatolingual contact and rapid tongue movements. Speech was slow, effortful, lacking normal prosody, and more affected than expected from the degree of facial or tongue palsy. Here we describe three cases of patients who developed severe dysarthria, temporarily mimicking speech arrest or aphemia, due to a localized brain lesion near the left face representation of the human primary motor cortex (face-M1). No clinical data have yet been presented to show that a lesion localized to the primary motor area (M1) can cause severe transient impairment of articulation, although a motor representation for articulation has been suggested to exist within M1.
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