Cranial nerve involvement in systemic sclerosis (scleroderma): a report of 10 cases.
ABSTRACT Ten patients with the diagnosis of systemic sclerosis developed cranial nerve involvement. A trigeminal sensory neuropathy evolved insidiously in all patients and in five of these it was a presenting complaint. The glossopharyngeal nerve was involved in one patient. Taste was impaired in one patient and a unilateral loss of taste with fasciculations of the tongue were noted in another. Tinnitus was a complaint in three patients, two of whom had bilateral impairment of hearing. Facial weakness was noted in five patients. In three, this weakness was bilateral, while in the others the weakness was unilateral, and a past history of acute onset was obtained. The microangiopathy of systemic sclerosis is felt to be primarily responsible for these neurological deficits. The deposition of fibrous tissue may be a secondary phenomenon and contribute to the process by compression of nerves.
- SourceAvailable from: Elzbieta Waszczykowska[show abstract] [hide abstract]
ABSTRACT: Objectives In systemic sclerosis (SSc) there may occur hearing and balance disorders as a result of the immune-mediated inner ear damage, the etiology being vasculitis and fibrosis. The objective is the vestibular organ evaluation in patients with SSc regarding their prevalence and relationship to duration of the disease and Raynaud phenomenon and also to type and severity of SSc. Material Twenty unselected, consecutive patients with diagnosed SSc, complying with international diagnostic criteria of the American Rheumatism Association (1982), were enrolled into the study. The control group consisted of 26 otologically healthy persons matched to the SSc group for age and sex. Methods In all patients the questionnaire about audiovestibular history, otolaryngological examination, static and dynamic vestibular tests and the electronystsgmography (ENG) were performed. The patients were investigated with the electronystsgmography (ENG) for spontaneous, positional and caloric-induced nystagmus. Also visual ocular-motor tests were performer. Results In the anamnesis 65% of patients reported vertigo, 55% – headaches, 50% – tinnitus, 40% – hyperacusis, 40% – hearing loss and 30% – ear fullness. Vertigo, dizziness balance disturbance and uncorrect results of Romberg and Utenberger tests were more frequent in patients with vestibular organ lesion. Abnormalities in vestibular organ in SSc patients were fund in 14 (70%) persons – the central type of lesion −8 (40%), mixed type in 3 (15%) and peripheral in 3 (15%). Conclusions Ear involvement is frequent in systemic sclerosis and should be taken into consideration during diagnostic and therapeutic procedures.Journal of Electrocardiology - J ELECTROCARDIOL. 01/2011; 65(3):202-207.
- [show abstract] [hide abstract]
ABSTRACT: To describe the differential diagnosis of recurrent or bilateral peripheral facial palsy. Case report and literature review. Two patients with recurrent, alternating, peripheral facial palsy are described. In both patients, additional investigation was performed to search for a specific diagnosis. In the first patient, only a positive family history was found, indicating a possible familial susceptibility. In the other patient, diabetes mellitus and hypertension were identified as risk factors. There is an important and extensive differential diagnosis of recurrent or bilateral facial palsy. However, in a large proportion of patients the cause remains unknown.The Journal of Laryngology & Otology 08/2012; 126(8):833-6. · 0.68 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: In systemic sclerosis (SSc), there may develop hearing and balance disorders as a result of the immune-mediated vasculitis and fibrosis in the inner ear. The objective of the study was evaluation of the hearing organ function in patients with SSc with relationship to duration of the disease and Raynaud phenomenon and also to type and severity of the disease. Twenty unselected, consecutive patients with SSc diagnosed in compliance with the international diagnostic criteria of the American Rheumatism Association (1982), were enrolled into the study. The control group consisted of 26 otologically healthy persons matched to the SSc group for age and sex. Case history was recorded for all patients from questionnaire data. Otolaryngological examination and battery of audiological tests (pure tone audiometry, speech audiometry, impedance audiometry and auditory brainstem response-ABR) were performed. In the anamnesis 60% of patients reported vertigo, 55% headaches, 50% tinnitus, 40% hyperacusis, 40% hearing loss and 30% ear fullness. It was found that patients with SSc had significantly poorer mean hearing thresholds than the control group for 0.5, 1, 6 and 8 kHz. In ABR there were no differences between SSc and control groups although an increase of latency averages in the group of limited patients with SSc compared with the diffuse patients with SSc was observed. In eight patients (40%) sensorineural hearing loss, mostly bilateral and symmetrical was found. Furthermore, no relation was seen between hearing level and duration, type and severity of the disease. Ear involvement is frequent in systemic sclerosis and should be taken into consideration during diagnostic and therapeutic procedures.Rheumatology International 05/2010; 31(11):1423-8. · 2.21 Impact Factor