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5 results

  • A rare complication of Behçet’s disease in a 12-year-old girl: a large intracardiac thrombosis

  • Şenay Akbay, Filiz Ekici, Elif Çomak
  • Journal:
    Cardiology in the Young / Volume 33 / Issue 6 / June 2023
    Published online by Cambridge University Press:
    15 November 2022, pp. 1033-1035
    • Article
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  • Behcet’s disease is a multi-systemic inflammatory disease with a clinical spectrum as a triple complex of recurrent oral, genital ulcers, and uveitis. Cardiac involvement in patients with Behcet’s disease is extremely rare and often associated with poor prognosis. Behcet’s disease should be considered in the differential diagnosis of right ventricular mass especially in young adults, even there is no typical clinical features of Behcet’s disease. In this case, a 12-year-old girl who admitted with chest pain and haemoptysis and then was diagnosed with intracardiac thrombus related to Behcet’s disease during follow-up was described.

  • Chapter 18 - CNS Syndromes at the Frontier of Autoimmune Encephalitis

  • from Section 3 - Specific Syndromes and Diseases
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book:
    Autoimmune Encephalitis and Related Disorders of the Nervous System
    Published online:
    27 January 2022
    Print publication:
    17 February 2022, pp 476-502

  • Summary

    In this chapter we review several CNS disorders of probable autoimmune origin or of unclear aetiology that sometimes are considered in the differential diagnosis of autoimmune encephalitis. These syndromes include the CNS complications of systemic autoimmune disorders: IgG4-related disease, Behçet disease, systemic lupus erythematosus (SLE), and sarcoidosis. In each of them, neurological symptoms may precede the onset of systemic symptoms. Other ‘frontier disorders’ include several diseases associated with primary involvement of the vascular endothelium or blood vessels: cerebral amyloid angiopathy-related inflammation, Susac’s syndrome, and primary angiitis of the CNS, which can all present with isolated neurological manifestations. These syndromes are immune-mediated, do not present specific or pathogenic neuronal antibodies, and their diagnosis is based on well-established clinical criteria that sometimes include neuroimaging and histopathological features. The clinical presentation of these syndromes may mimic that of several autoimmune encephalitis: SLE can present with psychosis (thus, it may need the differential diagnosis with anti-NMDAR encephalitis); IgG4-related disease can present with meningoencephalitis; and Behçet disease can present with brainstem dysfunction and neuroimaging findings resembling those of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). The clinical presentation of primary angiitis of the CNS and Susac syndrome can be indistinguishable from that of autoimmune encephalitis.

  • Olfactory bulb volume and olfactory sulcus depth in patients with Behçet's disease

  • A Doğan, N Bayar Muluk, N Asal, M H Şahan, M Inal, Ö Gündüz, O K Arıkan
  • Journal:
    The Journal of Laryngology & Otology / Volume 132 / Issue 12 / December 2018
    Published online by Cambridge University Press:
    18 December 2018, pp. 1088-1092
    Print publication:
    December 2018
  • Objective

    To investigate olfactory bulb volume and olfactory sulcus depth in patients with Behçet's disease, using magnetic resonance imaging.

    Methods

    Cranial magnetic resonance imaging scans of 27 adults with Behçet's disease (10 males and 17 females) and 27 healthy controls were examined. Olfactory bulb volume and olfactory sulcus depth were measured on coronal, T2-weighted, spectral pre-saturation with inversion recovery sequences.

    Results

    Bilateral olfactory bulb volume and right-sided olfactory sulcus depth were significantly lower in the Behçet's disease group than in the control group (p < 0.05). Left-sided olfactory sulcus depth increased with Behçet's disease duration. In both groups, olfactory bulb volume was significantly higher in the left than the right side. There were no gender differences for olfactory bulb volume and olfactory sulcus depth. Positive correlations were determined between right- and left-sided olfactory bulb volume values and between right- and left-sided olfactory sulcus depth values.

    Conclusion

    Behçet's disease may decrease olfactory functions, related to lower olfactory bulb volume and olfactory sulcus depth. The affected vascular system and possibly damaged neural system, nasal mucosal lesions, and prolonged nasal mucociliary clearance time may cause olfactory dysfunction. Patient follow up is recommended, with magnetic resonance imaging examinations of the olfactory system if necessary.

  • Assessment of nasal mucociliary activity in patients with Behçet's disease

  • I Ozbay, C Kucur, F Temİzturk, Y Ozkan, C Kahraman, F Oghan
  • Journal:
    The Journal of Laryngology & Otology / Volume 130 / Issue 4 / April 2016
    Published online by Cambridge University Press:
    04 February 2016, pp. 348-351
    Print publication:
    April 2016
  • Objective:

    To investigate nasal mucociliary clearance in patients with Behçet's disease.

    Methods:

    Thirty patients with Behçet's disease, diagnosed on the basis of criteria defined by the International Study Group for Behçet's Disease, and 30 healthy individuals were recruited. In both groups, individuals with conditions that could affect nasal mucociliary clearance were excluded. Nasal mucociliary clearance time was evaluated using saccharin tests. The time between placement of a saccharin tablet and the tasting of the saccharin was measured and recorded as the saccharin clearance time.

    Results:

    The mean nasal mucociliary clearance time was significantly longer in the Behçet's disease patients than in the control subjects (p < 0.001). Furthermore, there was a positive correlation between Behçet's disease duration and nasal mucociliary clearance time (p < 0.001, r = 0.882).

    Conclusion:

    The results suggest that nasal mucociliary clearance time is longer in Behçet's disease patients than in control subjects, and is positively correlated with disease duration.

  • Audiological evaluation in patients with Behçet's disease

  • N D Sonbay, C Saka, S Tatlican, E Vuralkan, N Aygener, C Eren, I Akin
  • Journal:
    The Journal of Laryngology & Otology / Volume 128 / Issue 8 / August 2014
    Published online by Cambridge University Press:
    17 July 2014, pp. 694-697
    Print publication:
    August 2014
  • Objective:

    This study aimed to determine the characteristics of hearing loss in patients with Behçet's disease.

    Methods:

    Twenty-six consecutive patients with Behçet's disease and a control group consisting of 25 age-matched healthy subjects were prospectively included in this study. Pure tone and speech audiometry, tympanometry, distortion product otoacoustic emission testing, and auditory brainstem-evoked response assessment were performed in the patients and controls.

    Results:

    The pure tone audiograms and the results of distortion product otoacoustic emission testing showed statistically significant hearing loss in the Behçet's disease patients (p < 0.05). Auditory brainstem-evoked response results were not significantly different between the patients and controls (p > 0.05).

    Conclusion:

    The findings of the present study demonstrated that audiological involvement is more frequent in patients with Behçet's disease than in healthy controls. Therefore, all patients with Behçet's disease should be regularly monitored by an otolaryngologist and be given information about the possibility of inner-ear involvement.