Incidence Of Nervous System Involvement Research Articles

Glucocorticoid reduction induced chorea in pediatric-onset systemic lupus erythematosus: A case report

BACKGROUND Pediatric-onset systemic lupus erythematosus (SLE) is typically more severe than adult-onset SLE, with a higher incidence of nervous system involvement. Chorea is a relatively rare neurological complication reported in 2.4%-7% of SLE patients. In particular, chorea induced by glucocorticoid dose reduction is even rarer. Herein, we report the case of a girl with SLE, who developed chorea during the process of glucocorticoid therapy reduction. CASE SUMMARY We describe a 14-year-old girl who was diagnosed with SLE. She was treated with methylprednisolone and rituximab, and her symptoms improved. On the second day after the methylprednisolone dose was reduced according to the treatment guidelines, the patient developed chorea. Her condition improved after adjusting her glucocorticoid regimen. CONCLUSION This case is a reminder that extra attention to chorea is required in SLE patients during glucocorticoid dose reduction.

Neurosarcoidosis.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology first named and described in the 19th century. The diagnosis usually requires the finding of noncaseating epithelioid cell granulomas in more than one organ and exclusion of other disorders known to cause granulomatous disease. The incidence of nervous system involvement in sarcoidosis is about 5%. Sarcoidosis may involve any part of the nervous system. Corticosteroids remain the mainstay of treatment of neurosarcoidosis.

Multimodal Electroneurophysiological Studies of Systemic Lupus erythematosus

The purpose of this study was to obtain electrophysiological documentation of possible involvement of central and peripheral nervous system (CNS and PNS) in systemic lupus erythematosus (SLE) patients even in the absence of neurological manifestations. The study included 30 consecutive patients with SLE and 25 age- and sex-matched volunteers as a control group. They were subjected to neurological and rheumatological tests and an extensive battery of neurophysiological tests, besides Wechsler adult intelligence scale. Overt neurological manifestations were observed in 14 patients (46.7%). Neurophysiological data revealed that 25 patients (83%) had at least 2 abnormal tests; 11 (68.8%) patients of the asymptomatic group and 14 patients (100%) of the symptomatic group with no significant differences between them. Seventeen patients (56.7%) had evidence of PNS dysfunction either in nerves (46.7%) or muscles (10%); 7 of them in the asymptomatic group. Twenty-four patients (80%) had evidence of CNS dysfunction. Twenty-two patients (73%) had abnormalities recorded on electroencephalography; 9 patients in the asymptomatic group and 13 patients in the symptomatic group. Eleven patients (37%) had abnormal values of P100 of visual evoked potential; 5 patients in the asymptomatic group and 6 in the symptomatic group. Eight patients (26.7%) had abnormal latency of wave I of brain stem response; 3 of them in the asymptomatic group. Abnormal prolongation of the P300 component of event-related potentials was recorded in 2 patients (12.5%) of the asymptomatic group, while low IQ was observed in 8 patients of each group. Neurophysiological abnormalities are fairly common in SLE patients whether symptomatic or asymptomatic. The use of such tests favors a true incidence of nervous system involvement, more accurate diagnosis, and may lead to better clinical care before the development of debilitating CNS and PNS changes.

Central nervous system sarcoidosis successfully treated with prednisone.

Sarcoidosis is defined as a chronic indolent and benign disease of unknown cause involving skin, lymph nodes, eyes, salivary glands, lungs, and bones of the hands and feet. 1 It is also well known that sarcoidosis can involve practically every organ system of the body, including the central nervous system. 2,3 The incidence of nervous system involvement in this disease is estimated between 1% and 5%, with lesions commoner in peripheral and cranial nerves than in the central nervous system. 2,4,5 Colliver, Hook, and Meyer et al. discuss the problem of nervous system sarcoidosis but do not report any cases treated with steroid hormones. 2,4,6 Pennell reported two cases of central nervous system sarcoidosis treated with corticotropin (ACTH) and cortisone without beneficial effect. 7 Olsen, on the other hand, discussed a case of sarcoidosis with Bell's palsy and spinal fluid changes treated with corticotropin and streptomycin with rapid recovery. 8 On review of the literature, we

INFECTIOUS MONONUCLEOSIS ENCEPHALITIS

A case of severe encephalitis due to infectious mononucleosis is reported. An eight year old white boy developed meningeal signs, intermittent coma, opisthotonos, extensor spasms, mild papilledema and hyperpyrexia two weeks after the onset of a sore throat and cervical lymphadenopathy. The diagnosis of infectious mononucleosis was confirmed by a heterophile antibody titer of 1:1024. The neurologic complications of the disease are uncommon in adults and are even more unusual in children. Only seven cases were found in the literature. Any or all parts of the nervous system may become involved; cerebrospinal fluid abnormalities may be the only manifestations of such involvement. The prognosis in children has been uniformly favorable with little or no neurologic residuals. Electroencephalographic and cerebrospinal fluid studies made on patients with infectious mononucleosis indicate a higher incidence of nervous system involvement than is evident by clinical examination. Serial heterophile agglutination determinations in patients with unexplained nervous system manifestations may clarify the diagnosis.