Cranial nerve involvement in systemic sclerosis (scleroderma): a report of 10 cases.

Medicine (Impact Factor: 4.87). 04/1980; 59(2):149-59. DOI: 10.1097/00005792-198003000-00006
Source: PubMed

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.

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    ABSTRACT: Progressive systemic sclerosis (PSS) is a chronic inflammatory disease of the connective tissue with involvement of the skin and other organs. The disease is characterized by an abnormal accumulation of collagen in all tissues and by microangiopathy. The involvement of the peripheral nervous system during PSS is very unusual and few cases are reported in the literature. A morphological study on the neuropathy associated with sclerodermia has been performed in rare cases. In this paper we demonstrate the role that the vascular lesions have in the pathogenesis of neuropathy during scleroderma. In particular, the primary role of the peripheral microangiopathy during PSS (observed in different clinical cases) is verified.
    Acta Neuropathologica 06/1986; 72(2):103-110. DOI:10.1007/BF00685970 · 9.78 Impact Factor
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    ABSTRACT: To perform a systematic review of neurologic involvement in Systemic sclerosis (SSc) and Localized Scleroderma (LS), describing clinical features, neuroimaging, and treatment. We performed a literature search in PubMed using the following MeSH terms, scleroderma, systemic sclerosis, localized scleroderma, localized scleroderma "en coup de sabre", Parry-Romberg syndrome, cognitive impairment, memory, seizures, epilepsy, headache, depression, anxiety, mood disorders, Center for Epidemiologic Studies Depression (CES-D), SF-36, Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Patient Health Questionnaire-9 (PHQ-9), neuropsychiatric, psychosis, neurologic involvement, neuropathy, peripheral nerves, cranial nerves, carpal tunnel syndrome, ulnar entrapment, tarsal tunnel syndrome, mononeuropathy, polyneuropathy, radiculopathy, myelopathy, autonomic nervous system, nervous system, electroencephalography (EEG), electromyography (EMG), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Patients with other connective tissue disease knowingly responsible for nervous system involvement were excluded from the analyses. A total of 182 case reports/studies addressing SSc and 50 referring to LS were identified. SSc patients totalized 9506, while data on 224 LS patients were available. In LS, seizures (41.58%) and headache (18.81%) predominated. Nonetheless, descriptions of varied cranial nerve involvement and hemiparesis were made. Central nervous system involvement in SSc was characterized by headache (23.73%), seizures (13.56%) and cognitive impairment (8.47%). Depression and anxiety were frequently observed (73.15% and 23.95%, respectively). Myopathy (51.8%), trigeminal neuropathy (16.52%), peripheral sensorimotor polyneuropathy (14.25%), and carpal tunnel syndrome (6.56%) were the most frequent peripheral nervous system involvement in SSc. Autonomic neuropathy involving cardiovascular and gastrointestinal systems was regularly described. Treatment of nervous system involvement, on the other hand, varied in a case-to-case basis. However, corticosteroids and cyclophosphamide were usually prescribed in severe cases. Previously considered a rare event, nervous system involvement in scleroderma has been increasingly recognized. Seizures and headache are the most reported features in LS en coup de sabre, while peripheral and autonomic nervous systems involvement predominate in SSc. Moreover, recently, reports have frequently documented white matter lesions in asymptomatic SSc patients, suggesting smaller branches and perforating arteries involvement.
    Seminars in arthritis and rheumatism 07/2013; 43(3). DOI:10.1016/j.semarthrit.2013.05.002 · 3.63 Impact Factor
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    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.
    Otolaryngologia polska. The Polish otolaryngology 05/2011; 65(3):202-207. DOI:10.1016/S0030-6657(11)70676-X