Evoked potentials can predict future disability in people with clinically isolated syndrome.

BACKGROUND AND PURPOSE: The aim was to evaluate whether the addition of evoked potentials (EPs) which evaluate brainstem function to the EP score improves its ability to predict disease progression in people with clinically isolated syndrome (pwCIS). METHODS: For 94 pwCIS, data on disease activity and progression after 2.9 (1.4-4.1) years of follow-up were available. Baseline characteristics included magnetic resonance imaging (MRI) parameters, visual EPs, auditory EPs, somatosensory EPs of the median and tibial nerves, vestibular evoked myogenic potentials and tongue somatosensory EPs. RESULTS: A multivariable regression model including age, sex, total number of T2 lesions on baseline MRI and EP score >13 showed that the total number of T2 lesions on baseline MRI and EP score >13 increase the likelihood for sustained accumulation of disability (SAD). After controlling for age, sex and the total number of T2 lesions on baseline MRI, the hazard of SAD for participants with EP score >13 is 4.093 times that of participants with EP score ≤13. EP score >13 also increases the likelihood for progression measured with a composite measure of progression which uses the Expanded Disability Status Scale, the nine-hole peg test and the timed 25-ft walk (exp(B) = 5.577, 95% confidence interval 1.520-20.468, P = 0.01). CONCLUSION: The addition of EPs that evaluate brainstem function to the EP score enables prediction of the progression of disability in pwCIS.

Longitudinal assessment of autonomic nervous system in patients with first demyelinating event suggestive of multiple sclerosis.

BACKGROUND AND PURPOSE: As a high proportion of people with clinically isolated syndrome (pwCIS) exhibit sympathetic adrenergic and sudomotor dysfunction, the aim of this study was to investigate the evolution of autonomic nervous system (ANS) abnormalities in pwCIS over a 2-year follow-up. METHODS: This was a prospective cohort study in which 121 pwCIS were enrolled and followed for 2 years. After 2-year follow-up, data were available for 84 pwCIS. ANS symptoms were evaluated with the Composite Autonomic System Score-31 (COMPASS-31) and results of the ANS tests were expressed using the Composite Autonomic Scoring Scale (CASS) at baseline and visit at month 24. Symptomatic dysautonomia was defined if the patient had a COMPASS-31 value above the median of the whole cohort at baseline evaluation (COMPASS-31 > 6.79) and CASS score >0. RESULTS: Complete CASS data at baseline and month 24 were available for 62 patients; in 24 (38.7%) patients there was worsening, in 16 (25.8%) there was improvement and in 22 (35.5%) there was no change in CASS score. In 90% of pwCIS (72 of 80) there was no change in parasympathetic nervous system tests, whereas 47.3% (35 of 74) had either worsening or improvement in sympathetic adrenergic and 28.6% (20 of 70) had either worsening or improvement in sudomotor function. A multivariable regression model identified the total number of T2 lesions as an independent predictor for worsening of symptomatic dysautonomia. No predictors for worsening or improving of CASS score were identified. CONCLUSION: A substantial proportion of pwCIS experienced worsening of ANS abnormalities during the 2-year follow-up and magnetic resonance imaging parameters seemed to predict these abnormalities.

The vestibular evoked myogenic potentials (VEMP) score: a promising tool for evaluation of brainstem involvement in multiple sclerosis.

BACKGROUND AND PURPOSE: Concerning the great importance of brainstem involvement in multiple sclerosis (MS), the aim of this study was to explore the role of the newly developed vestibular evoked myogenic potentials (VEMP) score as a possible marker of brainstem involvement in MS patients. PATIENTS AND METHODS: This was a prospective case-control study which included 100 MS patients divided into two groups (without and with clinical signs of brainstem involvement) and 50 healthy controls. Ocular VEMP (oVEMP) and cervical VEMP (cVEMP) measurements were performed in all participants and analyzed for latencies, conduction block and amplitude asymmetry ratio. Based on this the VEMP score was calculated and compared with Expanded Disability Status Scale (EDSS), disease duration and magnetic resonance imaging data. RESULTS: Multiple sclerosis patients with clinical signs of brainstem involvement (group 2) had a statistically significant higher percentage of VEMP conduction blocks compared with patients without clinical signs of brainstem involvement (group 1) and healthy controls (P = 0.027 and P < 0.0001, respectively). Similarly, the VEMP score was significantly higher in group 2 compared with group 1 (P = 0.018) and correlated with EDSS and disease duration (P = 0.011 and P = 0.032, respectively). Multivariate linear regression analysis showed that the VEMP score has a statistically significant influence on the EDSS score (P < 0.001, R(2) = 0.239). CONCLUSIONS: Interpretation of the oVEMP and cVEMP results in the form of the VEMP score enables better evaluation of brainstem involvement than either of these evoked potentials alone and correlates well with disability.

Simultaneous conus medullaris ependymoma and cerebellar astrocytoma in the same patient.

Multiple primary tumors in the central nervous system of different histological cell types are uncommon. We report a patient who had pilocytic astrocytoma in the cerebellum and ependymoma in the cauda equina region, occurring simultaneously. The suggested mechanism of this association is that primitive multipotent cells might have been displaced in the different CNS areas and developed in different tumor cells. Multiplicity of primary CNS tumors should be considered in certain occasions, when clinical symptoms and signs are pointing in that direction.

Avellis syndrome due to borreliosis.

Avellis syndrome is a rare form of alternating hemiparesis that is usually because of atherosclerosis. We report a 67-year-old man who developed paresthesiae of the left arm, dysphagia and dysphonia. The clinical picture, magnetic resonance imaging and cerebrospinal fluid findings were consistent with Avellis syndrome caused by brain stem arteritis because of late stage Borrelia burgdorferi infection, an extremely unusual aetiology for Avellis syndrome; this may well be the first such instance. It may be unrecognized in elderly patients with other risk factors for cerebrovascular disease.

Management of Fournier's gangrene--report of 7 cases and review of the literature.

Fournier's gangrene is a rare infection characterized with fast-progressing myonecrosis, that affect regions of perineum, genitalia and perianal area. This retrospective study presents authors' experiences and their principles in early diagnosis and treatment of Fournier's gangrene. The goal of this paper is to point out numerous diagnostically and therapeutic difficulties that lead to a high mortality if not recognized in time. We here describe seven male patients with myonecrosis and necrotising fasciitis in scrotal, perianal and perineal regions. Average age was 61 years (form 57 to 66 years of age), and average length of treatment was 25.8 days (from 14 to 36 days), with lethality of 14% (one case). We have recognised diabetes mellitus as risk factor, together with urethrostenosis, and other diseases of the perianal region (hemorrhoids, anal fissure, abscesses). Our hypothesis is that the key of the successful treatment is to treat as soon as symptoms onset, early and aggressive necrectomy under broad antibiotic protection. We also emphasize the possibility of recurrence of this disease even several years after treatment.

Utilization of physical rehabilitation among people with multiple sclerosis.

BACKGROUND: The goal in rehabilitating patients with multiple sclerosis (MS) is to minimize their physical and mental impairments and keep them integrated into their social environment. However, rehabilitation is not systematically utilized in MS patients. OBJECTIVES: This study aimed to determine how many patients diagnosed with MS use rehabilitation as a way of treatment and to evaluate correlation between use of rehabilitation and level of impairment. METHODS: We analyzed data regarding the use of rehabilitation in the last 2 years in 63 MS patients. Data were gathered using questionnaires during regular visits to neurological outpatient clinic from October to December 2011. Expanded Disability Status Scale (EDSS) was determined for all patients. RESULTS: One or more types of rehabilitation were used in 41.3% of questioned patients: inpatient, outpatient and home-based rehabilitation were used in 28.5, 17.4 and 4.7% of patients, respectively. Average EDSS in group with inpatient rehabilitation was 2.9, in group with outpatient rehabilitation 3.0 and in group without rehabilitation 1.0. We found that patients who used inpatient, outpatient and home-based rehabilitation had higher level of impairment comparing to patients who were not rehabilitated (p=0.002, p=0.004 and p=0.021, respectively). CONCLUSION: Rehabilitation of MS patients is not systematically provided, especially in early stages of disease when best results can be achieved.

Recurrent optic neuromyelitis with endocrinopathies: a new syndrome or just a coincidence?

The spectrum of optic neuromyelitis (ONM) ranges from monophasic or recurrent idiopathic forms of the disease, to ONM associated with autoimmune disorders. A distinct form of the disease, called recurrent ONM with endocrinopathies, characterized by spinal cord involvement (cavitations with syringomyeloid sensory disturbance), rapid evolution to blindness and paraplegia, characteristic cerebrospinal fluid (CSF) findings, and association with hypothalamus-pituitary dysfunction, has recently been described. The first case of ONM with endocrinopathies in a female Caucasian from Europe is presented, supporting the existence of this syndrome as a separate entity.