Clinical Reasoning: A demure teenager and her dystonic foot
A 13-year-old girl presented with a 4-year history of abnormal gait. At age 9, her parents noticed that she would run awkwardly "on the balls of her feet" and subsequently, that the rhythm of her running would break down with sustained exercise. There was no diurnal variation in her symptoms. There was no history of perinatal insults and early development was normal. There was no significant medical or psychiatric comorbidity and her family history was unremarkable. Examination of the patient's gait is demonstrated in video 1 at Neurology.org.
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Conclusions: We suggest that patients with dystonia have abnormal homeostatic inhibitory plasticity within the sensorimotor cortex and that this is responsible for their paradoxical response to high ‐frequency repetitive sensory stimulation. © 2018 International Parkinson and Movement Disorder Society
CONCLUSIONS: RC-IPGs can provide DBS patients with long-term therapeutic benefit while minimizing the need for battery replacement surgery. The authors have implanted rechargeable stimulators in 206 patients undergoing DBS surgery, and here they demonstrate the cost-effectiveness and high patient satisfaction associated with this procedure. PMID: 30265199 [PubMed - as supplied by publisher]
er EA Abstract Time perception is an essential element of conscious and subconscious experience, coordinating our perception and interaction with the surrounding environment. In recent years, major technological advances in the field of neuroscience have helped foster new insights into the processing of temporal information, including extending our knowledge of the role of the cerebellum as one of the key nodes in the brain for this function. This consensus paper provides a state-of-the-art picture from the experts in the field of the cerebellar research on a variety of crucial issues related to temporal processin...
CONCLUSION: Pallidal LF and beta burst amplitudes have different characteristics in PD and dystonia. The presence of increased burst amplitudes could be employed as feedback for GPi-aDBS. PMID: 30227227 [PubMed - as supplied by publisher]
Prion diseases are neurodegenerative in nature and progress inexorably once clinical symptoms are evident . Unlike thalidomide, lenalidomide rarely associates with neurotoxicicity. Up to now, thalidomide, but not lenalidomide, has been reported to be linked to acutely worsening Parkinson's disease . Two cases have been reported with transient memory loss due to lenalidomide so far . Here we describe a patient with reversible asymmetric parkinsonism, dystonia, and rapidly progressive dementia that appeared after administration of lenalidomide, and completely resolved shortly after its discontinuation.
Conclusion: The rates of both early and late complications of DBS surgery are acceptably low and decrease significantly with cumulative experience. PMID: 30140425 [PubMed]
Recently we have published a case report describing a 10-year-old child who had juvenile-onset parkinsonism with rigidity, bradykinesia, dystonia, gait disturbance, and cognitive impairment. Whole exome sequencing showed compound heterozygosity for two ATP13A2 novel mutations supporting the diagnosis of ATP13A2-related juvenile-onset Parkinson disease . Subsequently, a letter was published with comments related to our publication . Herein, we respond to the comments and queries raised in that letter.
CONCLUSION: Provision of implant-supported prostheses improves chewing and quality of life for patients with movement disorders and should be considered as an option in the treatment planning for tooth loss in this group of patients. However, straightforward designs that lend themselves to easier long-term maintenance should be adopted. PMID: 30109299 [PubMed - in process]
During the past two decades, deep brain stimulation (DBS) has been recognized as an efficient therapy that alleviates the symptoms of various treatment-resistant movement disorders such as Parkinson ’s disease (PD), dystonia, and tremor (Lyons, 2011; Vidailhet et al., 2013; Aviles-Olmos et al., 2014; Fasano et al., 2014; Kalia et al., 2013; Larson, 2014). Recent reports suggest that DBS can also be effective for treating psychiatric disorders such as depression, obsessive-compulsive disorder, Tourette’s syndrome (Holtzheimer and Mayberg, 2011) as well as dementia-related disorders and Alzheimer’s disease ...