Application of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorders in a cohort of Latin American patients.

BACKGROUND: The 2015 International Panel for neuromyelitis optica (NMO) spectrum disorders (NMOSD) diagnosis (IPND) criteria was recently proposed. However, because there are no studies evaluating application of the IPND criteria in Latin American populations, we aimed to assess whether these new criteria improve the diagnostic rate and reduce the time taken to make the diagnosis in a cohort of Latin American patients. METHODS: We reviewed medical records and applied both the 2006 and 2015 diagnostic criteria to all patients seen in four centers in Argentina, Brazil and Venezuela. Patients with multiple sclerosis (MS, n = 915) or other well-established central nervous system (CNS) inflammatory diseases were excluded. AQP4-ab status was measured using indirect immunofluorescence (23%) and cell-based assay (CBA, 77%). In addition, data on gender, ethnicity, age and symptoms at onset, relapses, neuroimaging and immunosuppressive therapy were collected. RESULTS: A total of 104 patients were classified as presenting NMOSD (2015 IPND). Of these, 64 patients (61.5%) fulfilled the 2006 NMO criteria (32 AQP4-ab positive, 17 AQP4-ab negative and 15 unknown). Thus, 40 new patients (38.5%) were classified as presenting NMOSD using the 2015 IPND criteria (33 AQP4-ab positive, 5 AQP4-ab negative and 2 unknown AQP4-ab status), with a median time taken to fulfill the 2015 NMOSD criteria (n = 104) of 1 month (95% CI: 0.6-1.3) and a median time taken to fulfill the 2006 NMO criteria (n = 64) of 18 months (95% CI: 9-26) (log-rank test: p < 0.0001). Females, with median age of 37 years, white ethnicity and recurrent course, predominated in all samples. Ninety-nine patients (95.1%) had at least 1 of the 3 major core clinical characteristics, of which optic neuritis (56.7%) was the most frequent symptom at disease onset. CONCLUSION: This study showed that there was a 62.5% increase in the rate of diagnosing NMOSD through the 2015 IPND criteria, in comparison with the 2006 NMO criteria, with a shorter median time to diagnosis.

Serial MRI findings in the Costa Rican variant of neuronal ceroid-lipofuscinosis.

Widespread cerebral atrophy and basal ganglia involvement are highly suggestive imaging features of the variants of late infantile type neuronal ceroid-lipofuscinosis. In the presence of clinical findings indicative of neuronal ceroid-lipofuscinosis, neuroimaging procedures are highly recommended to differentiate the variants from classic late infantile neuronal ceroid-lipofuscinosis. The clinical features and follow-up magnetic resonance imaging studies in a patient with the Costa Rican variant of late infantile neuronal ceroid-lipofuscinosis is presented. These procedures were of the utmost importance to observe the progression of the neurologic ailment and the extent of the cerebral and cerebellar abnormalities.

Extraventricular cerebral anaplastic ependymomas.

BACKGROUND: Anaplastic ependymomas are considered to be uncommon cerebral tumors by most authors. We have had the opportunity to study 34 cases of such lesions in 13 years. METHODS: 34 cases of anaplastic ependymoma operated in different hospitals of Maracaibo, Venezuela, during the period of 1983-1995 were analyzed. Cases of ependymoblastoma were excluded. RESULTS: Adult patients made up most of the present series. All patients harbored supratentorial growths in locations distant from the ventricular system. The microscopic pattern was of limited value to establish prognosis, for there was no constant correlation between the histologic features and tumor relapse; only in sporadic cases in which high cell density and conspicuous mitotic activity were maximally expressed, did tumor relapse occur shortly after removal of the lesion. CONCLUSION: This type of paradoxical behavior being the rule makes all attempts at predicting prognosis in these entities a disappointing task.

[The classical clinical and neuroimaging features of meningiomas are mimicked by other intracranial, supratentorial expansive lesions]. / Las características clínico-imaginológicas clásicas de los meningiomas son imitadas por otras lesiones expansivas.

OBJECTIVE: To analyze the surgical neurology cases in which both the clinical and neuroimaging features suggested supratentorial meningioma, and resulted in different entities from the neuropathological point of view. PATIENTS AND METHODS: The clinical histories of patients harboring intracranial, supratentorial mass lesions diagnosed as meningioma operated in different hospitals of Maracaibo, Venezuela, during the period 1993-1997 were reviewed. RESULTS: Our analysis revealed 15 cases with different neuropathological diagnosis. They were distributed as follows: three cases of hemangiopericytoma, two cases each of anaplastic ependymoma, metastatic carcinoma, and solitary intracranial plasmacytoma, and one case each of plasma cell granuloma, pleomorphic xanthoastrocytoma, chondroma, actinomycetoma, meningeal fibroma and chronic inflammation not otherwise specified. CONCLUSION: A heterogeneous group of intracranial, supratentorial expansive mass lesions can masquerade as meningiomas both from the clinical and neuroimaging points of view.

[Venezuelan equine encephalitis. 1995 outbreak: clinical profile of the case with neurologic involvement]. / Encefalitis equina venezolana. Epidemia de 1995: perfil clínico de los casos con compromiso neurológico.

INTRODUCTION: Venezuelan equine encephalitis virus has caused periodic epidemics and epizootics in the American continent since the 1920s. Such events have been profusely documented from the epidemiologic point of view, however, reports concerning the clinical features of this disease are rather scarce. OBJECTIVE: To analyze the clinical characteristics evidenced by Venezuelan equine encephalitis patients from Zulia state (western Venezuela) studied during the outbreak that occurred in Colombia and Venezuela in 1995. These cases, classified as complicated, were hospitalized at the Hospital Universitario de Maracaibo, state of Zulia, Venezuela. PATIENTS AND METHODS: The clinical charts of 313 Venezuelan equine encephalitis patients hospitalized during the period January 1st 1995-March 31st 1996 were reviewed. These cases accounted for 2.82% of 11,072 patients that were medically assisted during the outbreak. The following variables were analyzed: age, gender, signs and symptoms, contact history, complications and evolution. RESULTS: Intracranial hypertension signs became eloquent in 55.9% of these patients. Neurologic complications were represented by two cases of cerebellitis, two cases of meningoencephalitis and one case of encephalomyelitis. The mortality rate was 1.7%. CONCLUSION: Our results corroborate the benign evolutionary profile that is typical of this entity.