Abstract
Context: We present a noteworthy reminder of Wilson disease’s classical manifestations, which may become rarer in clinical practice as availability of genetic tests increases, allowing timely diagnosis and treatment. Case report: A 29 year-old woman developed progressive and asymmetric upper limb tremor and dystonia over 1 year, along with speech and feeding impairment in the last two weeks. Examination revealed segmental dystonia with risus sardonicus, open-jaw oromandibular and severe left arm dystonia, along with wing-beating tremor. Bilateral Kayser-Fleischer ring, low serum ceruloplasmin level, high urinary copper level, bilateral putaminal lesions on brain MRI and detection of ATP7B mutation confirmed Wilson disease (WD). A nasoenteric tube was inserted and D-penicillamine was started. Conclusion: This case illustrates the hallmark neuro-ophtalmological signs of WD: wing-beating tremor, risus sardonicus and Kayser-Fleischer ring. The former is probably associated with lesions in the dentato-rubro-thalamic pathway¹ and means a low frequency, high amplitude, posture-induced proximal arm tremor. Risus sardonicus means a fixed smile due to risorius muscle dystonia². Although it is a well-known manifestation of cephalic tetanus, it is also frequent in WD¹. Finally, the Kayser-Fleischer ring is caused by copper accumulation in the Descemet membrane and occurs in almost 100% of patients with neurological WD².
Highlights
Capsaicin is able to induce mast cell degranulation, an event probably related to the pathophysiology of a migraine attack
The present review study aimed to address the mechanisms of action of capsaicin and other chemical inducers in mast cell degranulation and an interaction of nerves and events that happen in the dura mater with the activation of mast cells
Conclusion: the analyzed data indicate that the polymorphisms contributed to the susceptibility to Parkinson’s disease (PD), further studies related to the polymorphisms and their relationship to PD are still needed for more ethnic groups, and early diagnosis is possible
Summary
Capsaicin is able to induce mast cell degranulation, an event probably related to the pathophysiology of a migraine attack. Methods: A literature review and an observational, descriptive and transversal data collect on treatment for acute demyelinating polyradiculoneuritis, available at DATASUS from January 2008 to December 2020, and articles available at Scielo and PubMed. Results: There were 7,917 hospitalizations, representing a total expenditure of R$ 9,392,552.04, 2009 being the year with the highest number of hospitalizations (809) and 2017 with the highest amount spent during the period (R$ 967,284.65). Methods: A literature review and an observational, descriptive and transversal data collect on surgical treatment was carried out, available from January 2008 to December 2020 and articles available in Scielo, Lilacs and PubMed. Results: There were 15,148 hospitalizations for surgical procedures for the treatment of chronic subdural hematoma, representing a total expenditure of R$ 45,365,258.21, with 2018 being the year with the highest number of hospitalizations (1,418) and with the highest amount spent during the period (R$ 4,570,334.28). Conclusions: The “Coca-Cola Bottle sign” is a classic sign of Graves’ disease, some signs, such as, unilateral and single orbital musculature involvement, may be suggestive of involvement by other etiologies, suggesting the benefit of an early expanded investigation
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
