El Departamento de Psiquiatría y Salud Mental del Hospital Central de la Defensa «Gómez Ulla» durante la pandemia por SARS-CoV-2 / The Department of Psychiatry and Mental Health of the Military Hospital «Gómez Ulla» during the SARS-CoV-2 pandemic

En el presente trabajo se exponen las medidas estructurales y logísticas, así como la práctica clínica planificada, para poder responder a la pandemia producida por el virus SARS-CoV-2 en el Departamento de Psiquiatría y Salud Mental del Hospital Central de la Defensa «Gómez Ulla». La planificación de la función asistencial se dividió en cinco grupos: los pacientes psiquiátricos ingresados en la Unidad de Hospitalización Breve; los pacientes con patología psiquiátrica ingresados en otros Servicios diferentes de Psiquiatría; los pacientes ambulatorios atendidos en Consultas Externas; los familiares de los pacientes ingresados por la COVID-19; el personal sanitario del Hospital Central de la Defensa «Gómez Ulla». En función de las necesidades de estos grupos asistenciales se realizó una planificación integral de la atención a los mismos. Durante el periodo 14 de marzo al 30 de mayo el 13% del grupo de profesionales del departamento de psiquiatría y salud mental, presentó síntomas moderados- graves de COVID-19; el 19% de los pacientes psiquiátricos ingresados en la unidad de hospitalización fueron COVID19 positivos, no falleciendo ninguno. El 74% de las interconsultas realizadas fueron sobre pacientes ingresados por COVID-19 que presentaron mayoritariamente cuadros confusionales de diversa intensidad o psicosis secundarias al uso de fármacos en el tratamiento activo del COVID-19. Se hicieron 4.185 llamadas a familiares, de las cuales el 14% (n=575) fueron a demanda de los propios familiares. Se hicieron más de 200 videollamadas y se mantuvo de forma telemática el 100% de las consultas externas

In this work the structural and logistical measures are exposed, as well as the planned clinical practice, to be able to respond to the pandemic caused by the virus SARS-CoV-2 in the Department of Psychiatry and Mental Health of the Central Defense Hospital «Gómez Ulla». The planning of the care function was divided into five groups: psychiatric patients admitted to the Brief Hospitalization Unit; patients with psychiatric pathology admitted to other different Psychiatric Services; outpatients treated in Outpatient Consultations; the relatives of the patients admitted by COVID-19; the health personnel of the Central Defense Hospital «Gómez Ulla». Based on the needs of these care groups, comprehensive care planning was carried out. During the period March 14 to May 30, 13% of the staff presented moderate-severe symptoms of COVID-19; 19% of the psychiatric patients admitted to the hospitalization unit were COVID19 positive, none of whom died. 74% of the inter-consultations carried out were on patients admitted for COVID-19 who presented mostly confusional symptoms of varying intensity or psychosis secondary to the use of drugs in the active treatment of COVID-19. 4.185 calls were made to family members, of which 14% (n = 575) were at the request of the family members themselves. More than 220 video calls were made and 100% of the external consultations were kept online

[The role of MRI in the early diagnosis of pyomyositis in children]. / Papel de la resonancia magnética en el diagnóstico precoz de la piomiositis en niños.

OBJECTIVE: To evaluate the role of MRI in the early diagnosis and therapeutic assessment of pyomyositis. MATERIAL AND METHODS: We reviewed the cases of 11 patients with proven pyomyositis presenting from January 2002 to March 2006. We describe the characteristic findings at plain-film radiography, ultrasound, and MRI, as well as the clinical presentation and laboratory findings. RESULTS: The mean age of presentation was 10 years (range 3-14 years); there was a male predominance (7 cases). Only 4 patients had predisposing conditions. The most common clinical symptom was fever (9 cases). The most common laboratory findings were elevated erythrocyte sedimentation rate (10 cases) and leukocytosis (8 cases). Plain-film and US findings were nonspecific in all cases. Scintigraphy was positive in 4 cases. MRI showed osteomuscular involvement in all cases and enabled the abscesses to be identified. CONCLUSIONS: Pyomyositis is a primary bacterial infection of skeletal muscle. In recent years pyomyositis has increasingly been diagnosed even in patients without predisposing factors. There is a male predominance. Imaging is essential for the diagnosis of the pyomyositis, and radiologists play a crucial role in the early diagnosis of this infection. MRI is the imaging method of choice for characterizing the infection.

Papel de la resonancia magnética en el diagnóstico precoz de la piomiositis en niños / The role of MRI in the early diagnosis of pyomyositis in children

Objetivos. Valorar el papel de la resonancia magnética (RM) en el diagnóstico precoz y el manejo terapéutico de la piomiositis. Material y métodos. Revisión retrospectiva de 11 casos de piomiositis, vistos entre enero de 2002 y marzo de 2006. Se analizan los hallazgos en radiología simple, ecográficos y por RM, así como la presentación clínica asociada. Resultados. La edad media de presentación fue de 10 años, con predominancia masculina en 7 casos. Sólo 4 de nuestros pacientes presentaron antecedentes de interés. El motivo de consulta más común presentado en 9 de los pacientes fue fiebre. Los hallazgos analíticos al ingreso fueron aumento de la velocidad de sedimentación globular (VSG) en 10 casos y leucocitosis en 8. Las radiografías y la ecografía resultaron inespecíficas en todos los pacientes evaluados, y la gammagrafía resultó positiva en 4 casos. La RM mostró afectación osteomuscular en todos los pacientes y permitió la identificación de abscesos. Conclusiones. La piomiositis es una infección bacteriana que aparece en nuestra sociedad incluso sin factores predisponentes, con predominancia masculina, en la que el radiólogo desempeña un papel fundamental para realizar un diagnóstico precoz de la misma, siendo la RM la técnica de elección para caracterizar la infección

Objective. To evaluate the role of MRI in the early diagnosis and therapeutic assessment of pyomyositis.Material and methods. We reviewed the cases of 11 patients with proven pyomyositis presenting from January 2002 to March 2006. We describe the characteristic findings at plain-film radiography, ultrasound, and MRI, as well as the clinical presentation and laboratory findings. Results. The mean age of presentation was 10 years (range 3-14 years); there was a male predominance (7 cases). Only 4 patients had predisposing conditions. The most common clinical symptom was fever (9 cases). The most common laboratory findings were elevated erythrocyte sedimentation rate (10 cases) and leukocytosis (8 cases). Plain-film and US findings were nonspecific in all cases. Scintigraphy was positivein 4 cases. MRI showed osteomuscular involvement in allcases and enabled the abscesses to be identified.Conclusions. Pyomyositis is a primary bacterial infectionof skeletal muscle. In recent years pyomyositis has increasingly been diagnosed even in patients without predisposing factors. There is a male predominance. Imaging is essential for the diagnosis of the pyomyositis, and radiologists play a crucial role in the early diagnosis of this infection. MRI is the imaging method of choice for characterizing the infection

[Benign idiopathic external hydrocephalus (benign subdural collection) in 39 children: its natural history and relation to familial macrocephaly]. / Hidrocefalia externa idiopática benigna (efusión subdural benigna) en 39 niños: evolución natural y relación con la macrocefalia familiar.

INTRODUCTION: Benign idiopathic external hydrocephalus (BIEH) is an age-dependent disorder that is self-limiting in time and has an uncertain aetiology. PATIENTS AND METHODS: A retrospective study was conducted involving 39 patients (16 girls and 23 boys) with BIEH. The following data were analysed for each patient: age, sex, family history, history concerning pregnancy, childbirth and neonatal period, postnatal history, data from clinical records and from physical examinations, progress of psychomotor development, findings from the first and successive neuroimaging studies, results of other complementary examinations, clinical and neuroimaging situation at the last check-up that was carried out, length of clinical control, age at which subdural effusion disappeared, and any other relevant associated facts. RESULTS: Age at diagnosis ranged from 1.33 and 25 months (mean: 8.4 months); in 38.46% of cases there was a history of macrocephalia in one of the progenitors; in four of them the presence of congenital macrocephalia was noted; in five, there was motor retardation and one of them displayed psychomotor retardation; in 15, there was an association with a slight dilatation of the lateral ventricles; the mean time of clinical control was 3.36 years; the process was seen to resolve in 14 cases; the minimum age for the disappearance of the subdural effusion was 9 months and the maximum was 8 years; macrocephalia persisted until the clinical control ended in 22 of the cases. We also noted the presence of two cases of mitochondrial encephalomyopathy, one craniosynostosis of the sagittal suture, one microdeletion 22q11.2, one a-1 antitrypsin deficiency, and one case of idiopathic bilateral congenital palpebral ptosis. CONCLUSIONS: The subdural effusion and/or macrocephalia persist in a high percentage of these patients and sometimes there is a close relationship between this condition and benign familial macrocephalia. Despite its benignity, it can influence psychomotor or motor retardation and behavioural disorders. On rare occasions it may be associated to mitochondrial encephalomyopathy and to the microdeletion 22q11.2.

Hidrocefalia externa idiopática benigna (efusión subdural benigna) en 39 niños: evolución natural y relación con la macrocefalia familiar / Benign idiopathic external hydrocephalus (benign subdural collection) in 39 children: natural history and relationship to familial macrocephaly

Introducción. La hidrocefalia externa idiopática benigna (HEIB) es un trastorno dependiente de la edad, autolimitado en el tiempo y de etiología incierta. Pacientes y métodos. Se llevó a cabo un estudio retrospectivo de 39 pacientes, 16 niñas y 23 niños, con HEIB, en los que se analizaron los siguientes datos: edad, sexo, antecedentes familiares, antecedentes del embarazo, el parto y el período neonatal, antecedentes posneonatales, datos de la historia clínica y de la exploración física, evolución del desarrollo psicomotor, hallazgos de la primera y sucesivas pruebas de neuroimagen, resultados de otras exploraciones complementarias, situación clínica y de neuroimagen en el último control realizado, tiempo de control clínico, edad a la que desapareció la efusión subdural, y otros hechos relevantes asociados. Resultados. La edad en el momento del diagnóstico osciló entre 1,33 y 25 meses (media: 8,4 meses); en el 38,46% existía el precedente de macrocefalia en alguno de los progenitores; en cuatro se observó la presencia de macrocefalia congénita; en cinco se detectó la presencia de retraso motor, y en uno, de retraso psicomotor; en 15 se asociaba una ligera dilatación de los ventrículos laterales; el tiempo medio de control clínico fue de 3,36 años; se observó la resolución del proceso en 14; la edad mínima para la desaparición de la efusión subdural fue de 9 meses, y la máxima, de 8 años; la macrocefalia persistió al final del control clínico en 22 niños; resalta la presencia de una encefalomiopatía mitocondrial en dos, de craneosinostosis de la sutura sagital en uno, de microdeleción 22q11.2 en otro, de deficiencia de -1 antitripsina en otro, y ptosis palpebral congénita bilateral idiopática en otro. Conclusiones. En un alto porcentaje de los pacientes persiste la efusión subdural y/o la macrocefalia; en ocasiones existe una estrecha relación entre esta entidad y la macrocefalia familiar benigna; a pesar de su benignidad, puede condicionar retraso psicomotor o motor y trastornos conductuales; de forma excepcional, se puede asociar a una encefalomiopatía mitocondrial y a la microdeleción 22q11.2

Introduction. Benign idiopathic external hydrocephalus (BIEH) is an age-dependent disorder that is self-limiting in time and has an uncertain aetiology. Patients and methods. A retrospective study was conducted involving 39 patients (16 girls and 23 boys) with BIEH. The following data were analysed for each patient: age, sex, family history, history concerning pregnancy, childbirth and neonatal period, postnatal history, data from clinical records and from physical examinations, progress of psychomotor development, findings from the first and successive neuroimaging studies, results of other complementary examinations, clinical and neuroimaging situation at the last check-up that was carried out, length of clinical control, age at which subdural effusion disappeared, and any other relevant associated facts. Results. Age at diagnosis ranged from 1.33 and 25 months (mean: 8.4 months); in 38.46% of cases there was a history of macrocephalia in one of the progenitors; in four of them the presence of congenital macrocephalia was noted; in five, there was motor retardation and one of them displayed psychomotor retardation; in 15, there was an association with a slight dilatation of the lateral ventricles; the mean time of clinical control was 3.36 years; the process was seen to resolve in 14 cases; the minimum age for the disappearance of the subdural effusion was 9 months and the maximum was 8 years; macrocephalia persisted until the clinical control ended in 22 of the cases. We also noted the presence of two cases of mitochondrial encephalomyopathy, one craniosynostosis of the sagittal suture, one microdeletion 22q11.2, one -1 antitrypsin deficiency, and one case of idiopathic bilateral congenital palpebral ptosis. Conclusions. The subdural effusion and/or macrocephalia persist in a high percentage of these patients and sometimes there is a close relationship between this condition and benign familial macrocephalia. Despite its benignity, it can influence psychomotor or motor retardation and behavioural disorders. On rare occasions it may be associated to mitochondrial encephalomyopathy and to the microdeletion 22q11.2

[Aicardi-Goutières syndrome: report of two new cases]. / Síndrome de Aicardi-Goutières: aportación de dos nuevas observaciones.

INTRODUCTION: Aicardi-Goutières syndrome is an early onset autosomal recessive progressive encephalopathy, clinically characterized by acquired microcephaly, severe psychomotor delay and involvement of pyramidal and extrapyramidal tracts. Intracranial calcifications, especially at the level of the basal ganglia, white matter abnormalities, lymphocytosis and raised interferon (IFN)-alpha in blood and cerebrospinal fluid (CSF) form part of this syndrome. CASES REPORTS: We describe two unrelated infants (a 3-month-old boy and an 11-month-old girl) who both presented with hypotonia, microcephaly, and psychomotor delay. Mild choreic and dystonic movements, as well as progressive spasticity, were also observed in the girl. Extensive investigations revealed intracranial calcifications, mild CSF lymphocytosis in the boy, and raised IFN-a in blood and CSF in both patients. COMMENTS: Aicardi-Goutières syndrome should be kept in mind when investigating microcephalic and retarded patients with cerebral calcifications initially suggestive of TORCH infection. Appropriate genetic counseling should be provided.

Síndrome de Aicardi-Goutières: aportación de dos nuevas observaciones / Aicardi-Goutières syndrome: report of two new cases

Introducción: El síndrome de Aicardi-Goutières es una enfermedad neurodegenerativa de herencia autosómica recesiva, que se caracteriza por presentar encefalopatía grave y progresiva de inicio precoz, microcefalia evolutiva, disfunción piramidal- extrapiramidal, calcificaciones intracraneales especialmente a nivel de ganglios basales, afectación de la sustancia blanca, linfocitosis y elevación del interferón alfa (IFN-alfa) en líquido cefalorraquídeo (LCR) y en sangre. Casuística: Aportamos dos nuevas observaciones (un niño de 3 meses de edad y una niña de 11 meses) con clínica de hipotonía, microcefalia, retraso psicomotor, signos piramidales y extrapiramidales en la niña. Se demostró calcificaciones intracraneales, linfocitosis en LCR en el niño, y en ambos elevación del IFN-alfa en sangre y LCR. Comentarios: Es importante tener presente esta entidad a pesar de su relativa rareza, ante un paciente con afectación del desarrollo psicomotor, microcefalia y presencia de calcificaciones intracraneales, debido a las distintas consideraciones de cara al consejo genético que tiene con respecto a otras entidades más prevalentes y fácilmente confundibles, como las infecciones TORCH (toxoplasmosis, rubéola, citomegalovirus, herpes)

Introduction: Aicardi-Goutières syndrome is an early onset autosomal recessive progressive encephalopathy, clinically characterized by acquired microcephaly, severe psychomotor delay and involvement of pyramidal and extrapyramidal tracts. Intracranial calcifications, especially at the level of the basal ganglia, white matter abnormalities, lymphocytosis and raised interferon (IFN)-alpha in blood and cerebrospinal fluid (CSF) form part of this syndrome. Cases reports: We describe two unrelated infants (a 3-month-old boy and an 11-month-old girl) who both presented with hypotonia, microcephaly, and psychomotor delay. Mild choreic and dystonic movements, as well as progressive spasticity, were also observed in the girl. Extensive investigations revealed intracranial calcifications, mild CSF lymphocytosis in the boy, and raised IFN-alpha in blood and CSF in both patients. Comments Aicardi-Goutières syndrome should be kept in mind when investigating microcephalic and retarded patients with cerebral calcifications initially suggestive of TORCH infection. Appropriate genetic counseling should be provided

[Mitochondrial encephalomyopathies and West's syndrome: a frequently underdiagnosed association]. / Encefalomiopatías mitocondriales y síndrome de West: una asociación frecuentemente infradiagnosticada.

INTRODUCTION: West's syndrome is known to have symptomatic, cryptogenetic and idiopathic forms. Greater knowledge of the different pathologies and the development of new diagnostic techniques have allowed the list of symptomatic forms to be extended and congenital disorders of the metabolism account for a significant percentage as an aetiopathogenic factor. Yet, although it is known that mitochondrial cytopathies can trigger the development of West's syndrome, few reports exist concerning their association. AIMS: Our aim in this paper is to report on four cases of West's syndrome in which a mitochondrial cytopathy was shown to be an aetiopathogenic factor. CASE REPORTS: Two females and two males aged between 2 and 10 months, who were suffering from West's syndrome. Biochemical and neuroimaging findings suggested a possible mitochondrial cytopathy, which was later confirmed in the four cases on observing a partial deficiency of some of the complexes of the mitochondrial respiratory chain in muscles; this was found to be simple in the first three (complexes III, I and IV, respectively) and combined in the fourth (complexes I and IV). CONCLUSIONS: Infantile spasms should be considered as one of the ways mitochondrial encephalomyopathies manifest themselves. As part of the process of diagnosing West's syndrome, we recommend tests be carried out to determine the levels of lactic and pyruvic acid, carnitine and amino acids in plasma, and possibly in the cerebrospinal fluid, as well as those of amino acids and organic acids in urine. A muscular biopsy must also be carried out in patients who are strongly suspected of having a mitochondrial cytopathy, as well as the corresponding molecular genetic study.

Tortícolis persistente / Persistent torticolis

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Masa cervical / Cervical mass

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Adolescente con retraso de crecimiento / Adolescents with secondary growth impairment

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Alteraciones radiográficas de la cortical ósea / Radiographic changes of cortical bone

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