[Idiopathic hypertrophic pachymeningitis: a case report]. / Paquimeningitis hipertrófica idiopática: a propósito de un caso.

INTRODUCTION: Hypertrophic pachymeningitis is a clinical condition that is caused by a diffuse or localised thickening of the dura mater. It predominantly affects males and manifests as chronic headache, with or without association to neurological manifestations, such as paralysis of the cranial nerves, cerebellar ataxia and neuro-ophthalmic complications. CASE REPORT: A 61-year-old male, with no relevant past history, who, one month before admission, had begun to suffer from right frontotemporal headache that irradiated to the ipsilateral orbital region and was more pronounced at night. A week later he was affected by a decrease in visual acuity in the right eye and two weeks later he noted the presence of right palpebral ptosis, while the headaches increased. The examination showed: right palpebral ptosis with global ophthalmoparesis with predominance of adduction and abduction, and diminished photomotor reflex in the right eye. The visual acuity of the right eye was reduced and the palpebral fissure was 0 in the right eye. The fundus oculi was normal. Infectious and non-infectious causations of meningitis were precluded. Magnetic resonance imaging revealed a diffuse thickening of the supratentorial and infratentorial meninges, as well as diffuse uptake of the paramagnetic substance; thickening of the mucus in both paranasal maxillary sinuses was also observed. A meningeal biopsy study confirmed the existence of hypertrophic pachymeningitis. Treatment was established with prednisone and the clinical symptoms improved. CONCLUSIONS: Idiopathic hypertrophic pachymeningitis is an underdiagnosed condition that must be taken into consideration in cases of patients with a history of subacute or chronic meningitis in which infectious and non-infectious causations have been precluded, and high-dose steroid treatment must be established.

Paquimeningitis hipertrófica idiopática: a propósito de un caso / Idiopathic hyperthophic pachymeningitis: a case report

Introducción. La paquimeningitis hipertrófica es una entidad clínica que se debe a un engrosamiento difuso o localizado de la duramadre; suele afectar predominantemente a hombres y se manifiesta como una cefalea crónica, con o sin asociación a manifestaciones neurológicas, como parálisis de nervios craneales, ataxia cerebelosa y complicaciones neuroftalmológicas. Caso clínico. Varón de 61 años, sin antecedentes de interés, que comienza, un mes antes del ingreso, con cefalea frontotemporal derecha irradiada a la región orbitaria ipsilateral, de predominio nocturno. Una semana después se ve afectado por una disminución de la agudeza visual en el ojo derecho y dos semanas después nota ptosis palpebral derecha, con incremento de la cefalea. En la exploración se evidencian: ptosis palpebral derecha con oftalmoparesia global derecha con predominio de la aducción y abducción, y disminución de la respuesta fotomotora derecha. La agudeza visual en el ojo derecho se encuentra disminuida y la hendidura palpebral es 0 en el ojo derecho. El fondo de ojo es normal. Se descartan etiologías infecciosas y no infecciosas de meningitis. En la resonancia magnética se observa un engrosamiento difuso de las meninges supratentoriales e infratentoriales, así como una captación difusa de sustancia paramagnética; también se evidencia un engrosamiento de la mucosa en ambos senos paranasales maxilares. En la biopsia meníngea se confirma la existencia de paquimeningitis hipertrófica. Se instaura tratamiento con prednisona y la sintomatología mejora. Conclusiones. La paquimeningitis hipertrófica idiopática es una entidad subdiagnosticada que debe tenerse en cuenta en pacientes con historia de meningitis subagudas o crónicas en quienes se hayan descartado etiologías infecciosas y no infecciosas, y debe instaurarse tratamiento con esteroides en altas dosis (AU)

Introduction. Hypertrophic pachymeningitis is a clinical condition that is caused by a diffuse or localised thickening of the dura mater. It predominantly affects males and manifests as chronic headache, with or without association to neurological manifestations, such as paralysis of the cranial nerves, cerebellar ataxia and neuro-ophthalmic complications. Case report. A 61-year-old male, with no relevant past history, who, one month before admission, had begun to suffer from right frontotemporal headache that irradiated to the ipsilateral orbital region and was more pronounced at night. A week later he was affected by a decrease in visual acuity in the right eye and two weeks later he noted the presence of right palpebral ptosis, while the headaches increased. The examination showed: right palpebral ptosis with global ophthalmoparesis with predominance of adduction and abduction, and diminished photomotor reflex in the right eye. The visual acuity of the right eye was reduced and the palpebral fissure was 0 in the right eye. The fundus oculi was normal. Infectious and non-infectious causations ofmeningitis were precluded. Magnetic resonance imaging revealed a diffuse thickening of the supratentorial and infratentorial meninges, as well as diffuse uptake of the paramagnetic substance; thickening of the mucus in both paranasal maxillary sinuses was also observed. A meningeal biopsy study confirmed the existence of hypertrophic pachymeningitis. Treatment was established with prednisone and the clinical symptoms improved. Conclusions. Idiopathic hypertrophic pachymeningitis is an underdiagnosed condition that must be taken into consideration in cases of patients with a history of subacute or chronic meningitis in which infectious and non-infectious causations have been precluded, and high-dose steroid treatment must be established (AU)