[Clinical features of 314 patients with relapsing-remitting multiple sclerosis]. / Esclerosis múltiple recurrente remitente en el sector público de salud de Chile: Descripción clínica de 314 pacientes.

BACKGROUND: Immunomodulatory drugs (IMD), Interferon ß1a, ß1 b and glatiramer acetate are available in the Chilean public health system since June 2008 for patients with relapsing-remitting multiple sclerosis (RR-MS). Diagnostic confirmation and programmed follow up of these patients is carried out at a public national reference center. AIM: To describe the epidemiological and clinical features of 314 patients evaluated in this center between 2008 and 2012. PATIENTS AND METHODS: Review of clinical records, to obtain information about demographic background, medical history, expanded disability status scale of Kurtzke (EDSS), multiple sclerosis functional composite (MSfic), intensity fatigue scale of Krupp, Rao’s Brief Repeatable Battery of Neuropsychological Tests (BNR-R) and anxious-depressive manifestations using Hamilton and Beck questionnaires. RESULTS: The ages of patients ranged from 12 to 63 years and 67% were women. The initial symptoms were sensory disturbances in 20%, motor alterations in 18% and optical neuritis in 16%. In 9% of patients, the disease began with several manifestations. The EDSS was 4 or less in 73% of patients and cognitive impairment was observed in 34%. Treatment failure during the first and second years, occurred in 23 and 26% of patients, respectively. Male gender, age under 40 and brainstem malfunction at the onset of disease, were predictive of treatment failure during the second year. CONCLUSIONS: The features of these patients are very similar to those reported abroad.

[Neuropsychological assessment of patients with relapsing remitting multiple sclerosis prior to the use of immunomodulatory drugs]. / Evaluación neuropsicológica en 129 pacientes chilenos con esclerosis múltiple recurrente remitente previo a inicio de fármacos inmunomoduladores.

BACKGROUND: The detection of cognitive changes (CC) and psychiatric disorders in relapsing remitting multiple sclerosis (MS-RR) contributes to patient clinical monitoring. AIM: To assess the frequency and characteristics of CC and psychiatric disorders in Chilean patients with MS-RR, before starting immunomodulatory treatment. PATIENTS AND METHODS: Retrospective review of data that was obtained following a standard assessment protocol. It consisted in the application of the Expanded Disability Status Scale of Kurtzke (EDSS), Multiple Sclerosis Functional Composite (MSFC), fatigue intensity scale of Krupp, brief repeatable battery of neuropsychological Rao (BRN-R) and Hamilton's depression and anxiety questionnaires. RESULTS: We evaluated 129 patients aged between 12 and 60 years of age (69% women). Ninety four percent of patients had eight or more years of schooling. The average EDSS score was 2.83. CC were detected in 62% of participants, in at least one subtest of the BRN-R. The main changes were verbal memory and speed in the processing information. The frequency of cognitive impairment (CI), defined as at least two BRN-R subtests altered, was 36%. The figures decreased to 17% when significant major depression or associated fatigue were excluded. Depressive symptoms were observed in 58% and anxiety in 76.7%. CONCLUSIONS: The results are consistent with those described in the literature. The type of instruments used in the investigation of CC and the definition of CI in MS should be standardized.

Esclerosis múltiple recurrente remitente en el sector público de salud de Chile: Descripción clínica de 314 pacientes / Clinical features of 314 patients with relapsing-remitting multiple sclerosis

Background: Immunomodulatory drugs (IMD), Interferon β1a, β1 b and glatiramer acetate are available in the Chilean public health system since June 2008 for patients with relapsing-remitting multiple sclerosis (RR-MS). Diagnostic confirmation and programmed follow up of these patients is carried out at a public national reference center. Aim: To describe the epidemiological and clinical features of 314 patients evaluated in this center between 2008 and 2012. Patients and Methods: Review of clinical records, to obtain information about demographic background, medical history, expanded disability status scale of Kurtzke (EDSS), multiple sclerosis functional composite (MSfic), intensity fatigue scale of Krupp, Rao’s Brief Repeatable Battery of Neuropsychological Tests (BNR-R) and anxious-depressive manifestations using Hamilton and Beck questionnaires. Results: The ages of patients ranged from 12 to 63 years and 67% were women. The initial symptoms were sensory disturbances in 20%, motor alterations in 18% and optical neuritis in 16%. In 9% of patients, the disease began with several manifestations. The EDSS was 4 or less in 73% of patients and cognitive impairment was observed in 34%. Treatment failure during the first and second years, occurred in 23 and 26% of patients, respectively. Male gender, age under 40 and brainstem malfunction at the onset of disease, were predictive of treatment failure during the second year. Conclusions: The features of these patients are very similar to those reported abroad.

Evaluación neuropsicológica en 129 pacientes chilenos con esclerosis múltiple recurrente remitente previo a inicio de fármacos inmunomoduladores / Neuropsychological assessment of patients with relapsing remitting multiple sclerosis prior to the use of immunomodulatory drugs

Background: The detection of cognitive changes (CC) and psychiatric disorders in relapsing remitting multiple sclerosis (MS-RR) contributes to patient clinical monitoring. Aim: To assess the frequency and characteristics of CC and psychiatric disorders in Chilean patients with MS-RR, before starting immunomodulatory treatment. Patients and Methods: Retrospective review of data that was obtained following a standard assessment protocol. It consisted in the application of the Expanded Disability Status Scale of Kurtzke (EDSS), Multiple Sclerosis Functional Composite (MSFC), fatigue intensity scale of Krupp, brief repeatable battery of neuropsychological Rao (BRN-R) and Hamilton's depression and anxiety questionnaires. Results: We evaluated 129 patients aged between 12 and 60 years of age (69% women). Ninetyfour percent of patients had eight or more years of schooling. The average EDSS score was 2.83. CC were detected in 62% of participants, in at least one subtest of the BRN-R. The main changes were verbal memory and speed in the processing information. The frequency of cognitive impairment (CI), defined as at least two BRN-R subtests altered, was 36%. The figures decreased to 17% when significant major depression or associated fatigue were excluded. Depressive symptoms were observed in 58% and anxiety in 76.7%. Conclusions: The results are consistent with those described in the literature. The type of instruments used in the investigation of CC and the definition of CI in MS should be standardized.

[Subacute combined degeneration of the spinal cord caused by vitamin B12 deficiency. Report of 11 cases]. / Mielopatía por déficit de vitamina B12: caracterización clínica de 11 casos.

BACKGROUND: Subacute combined degeneration is a clinical manifestation of vitamin B12 deficiency, that we observe with unusual frequency. AIM: To report a series of eleven patients with subacute combined degeneration. PATIENTS AND METHODS: Retrospective analysis of 11 patients hospitalized in a public hospital in Santiago, between March 2001 and February 2003. All had a myelopathy of more than three weeks of evolution with serum vitamin B12 levels of less than 200 pg/ml. RESULTS: A risk factor was identified in 10 cases and the most common was an age over 60 years old. The main presenting symptom was the presence of paresthesias. On admission, sphincter dysfunction, posterior column and pyramidal syndromes coexisted in nine patients. A level of sensitive deficit was detected in six. Ten patients had macrocytosis and eight were anemic. Serum vitamin B12 was measured in ten and in nine, it was below 200 pg/ml. The mean lapse between onset of symptoms and treatment was eight months. All received intramuscular vitamin B12 in doses on 1,000 to 10,000 IU/day. Sphincter dysfunction and propioception were the first symptoms to improve. CONCLUSIONS: Subacute combined degeneration must be suspected in patients older than 60 years with a subacute myelopathic syndrome and low serum vitamin B12 levels.

Fibrodisplasia craneofacial / Craniofacial fibrous dysplasia

Entre los años 1970 y 1988 se han tratado 28 pacientes con fibrodisplasia ósea craneofacial. Esta enfermedad, de etiología incierta, evoluciona preferentemente en las primeras dos décadas de la vida; corresponde a un tumor que deforma estructuras óseas, estrechando conductos y cavidades y provocando varios signos y síntomas. El diagnóstico se basa en el cuadro clínico y caracteres radiológicos, confirmándose con el estudio histopatológico de la lesión. Hemos realizado remodelación del área tumoral deformada mediante resección parcial del tumor en 11 pacientes y resección amplia en 17 pacientes, de los cuales 14 debieron ser rehabilitados con injertos óseos autólogos. En 7 se practicó más de un procedimiento resectivo. Como resultado de esta actitud terapéutica se obtuvo la detención del deterioro funcional y morfológico provocado por el tumor. La apariencia estética postoperatoria fue buena o satisfactoria en el 90% de los pacientes controlados por más de nueve meses

Fractura por estallido (Blow-out) de órbita / Blow out orbital fractures

Se presentan 18 pacientes con fractura de órbita por estallido, de un total de 300 enfermos que consultan por algún rasgo de fractura de la órbita. El diagnóstico se basó en el estudio radiológico y en los hallazgos intraoperatorios. Su tratamiento quirúrgico, a través de una incisión habitualmente subpalpebral, consistió en la reparación del piso orbitario con diferentes materiales biológicos o sintéticos. Como complicación tardía un paciente presentó rechazo al silastic, 2 casos de persistencia de la limitación de los movimientos oculares y un paciente con enoftalmo, diplopia y descenso pupilar

Secuelas de las fisuras labiopalatinas / Cleft palate sequelae

Se analiza la secuencia histórica de dos período de tratamiento de fisuras labiopalatinas en el Hospital Barros Luco Trudeau, en un total de 251 enfermos. Se ha notado una importante disminución de los casos tratados por años (4 contra 10), del grado lesional decreciente residual o secuelar y del aumento de las correcciones secundarias del orden estática. Se atribuye como explicación a este progreso el mejoramiento de la atención pediátrica multidisciplinaria ofrecida en el Hospital Base del Area