Behçet’s Syndrome and the Nervous System Involvement

Uygunoğlu, Uğur; Síva, Aksel

doi:10.1007/978-3-030-19515-1_22

Cited by 1 publication

(1 citation statement)

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“…Neurological involvement is a rare manifestation but an important cause of longterm morbidity. NeuroBehcet's disease is more common in males and neurological symptoms typically present 3 to 6 years after other systemic manifestations [93,94]. Movement disorders including chorea and parkinsonism have been reported in neuroBehcet's disease and are believed to be related to antibasal ganglia antibodies [95].…”

Section: Behcet's Diseasementioning

confidence: 99%

Review of Hereditary and Acquired Rare Choreas

Martínez-Ramírez

Walker

Rodríguez-Violante

et al. 2020

Tremor and Other Hyperkinetic Movements

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The first step in diagnosis is recognizing the movement disorder as chorea [1]. Chorea is characterized by irregu lar, purposeless, arrhythmic, nonstereotyped involuntary movements that flow from one body part to another [2]. These movements can affect any part of the body, although usually brief, can also be of long duration, and can have small or large amplitude. Movements with large amplitude localized proximally in the arms are called "ballismus" [3]. Once the movement disorder has been recognized as chorea, we then recommend classifying the age of onset as either childhood (prior to the age of 16) or adultonset [1]. Multiple causes of chorea exist in both age groups, many of which fit in the definition of rare diseases [4]. Since the most common cause of acute chorea in childhood is Sydenham's chorea and of chronic progressive chorea in adults is Huntington's disease (HD), we recommend these disorders should be tested for, as appropriate, and excluded before proceeding to other diagnostic pathways. The next step is to determine, if possible, whether the chorea is hereditary (genetic) or acquired (nongen etic) in nature. The family history should be carefully and

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Section: Behcet's Diseasementioning

confidence: 99%