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BACKGROUND: To report a case of IgG4-related pachymeningitis presenting with cystic lesions mimicking neurocysticercosis. CASE PRESENTATION: A 40-year-old female patient with tetraparesis, dysphagia and dysphonia was evaluated with clinical examination, magnetic resonance imaging, and meningeal biopsy. Magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement involving the cranial, cervical, thoracic, and lumbar segments with spinal cord compression and cystic lesions. CSF immunology was initially positive for cysticercus cellulosae. After disease progression a meningeal biopsy was compatible with IgG4 related disease. The patient had partial response to rituximab and needed multiple surgical procedures for spinal cord decompression and CSF shunting. CONCLUSIONS: This case highlights the possibility of IgG4-related disease in patients with diffuse pachymeningitis causing spinal cord compression, even with cystic lesions on MRI. Diagnosis of IgG4-related pachymeningitis is paramount due to the possibility of treatment response to immunotherapy, particularly to anti-CD20 agents.

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Background: Hypertrophic pachymeningitis (HP) is a disease with diverse aetiologies, including the autoimmune one, either associated with antineutrophil cytoplasmic antibodies or immunoglobulin G4. Case description: A 65-year-old woman with a history of systemic arterial hypertension, presented with intense progressive headaches. HP and hemispheric vasogenic oedema were observed by nuclear magnetic resonance (NMR) study. During the six months before the headache, she had developed progressive hearing loss which she attributed to age. A biopsy of dura mater showed necrotising vasculitis with peripheral inflammatory infiltrate, made up of accumulations of epithelioid cells and multinucleated giant cells, and abundant eosinophils. A final diagnosis of HP with eosinophilic granulomatosis with polyangiitis (EGPA) was made. Discussion: The patient had eosinophilic granulomatosis with polyangiitis (EGPA) histology, ANCA-negative serology and HP. This case is important because it shows that EGPA seems to have a spectrum of clinical diseases, including HP with negative serology, and bilateral sensorineural hearing loss. Conclusion: We are facing a wide spectrum of EGPA, breaking the paradigm of only systemic involvement. LEARNING POINTS: Hypertrophic pachymeningitis (HP) has several aetiologies; if the systemic investigation is not contributory to a diagnosis, a meningeal biopsy is necessary.This is the first case report of HP, associated with eosinophilic granulomatosis with polyangiitis (EGPA), and ANCA-negative serology.EGPA is probably a spectrum of diseases with predominant systemic involvement, but there may be cases where there is histological evidence, without the systemic context or positive serology.

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OBJECTIVES: We aimed to characterize the clinical and radiological features, and outcomes, of a large cohort of hypertrophic pachymeningitis (HP) patients from a single center. METHODS: We conducted a retrospective study at a tertiary referral center, encompassing patients diagnosed with HP between 2003 and 2022. The diagnosis of HP relied on the identification of thickening of the dura mater via magnetic resonance imaging (MRI) of the brain or spine. RESULTS: We included 74 patients with a mean age of 43.6 ± 14.2 years, of whom 37 (50%) were male. Among them, 32 (43.2%) had an immune-mediated origin, including 21 with granulomatosis with polyangiitis (GPA) (predominantly PR3-ANCA positive), four with systemic lupus erythematosus, three with IgG4-related disease, three with idiopathic HP, and one with rheumatoid arthritis. Non-immune-mediated HP accounted for 45 cases (56.8%). Within this category, 21 (28.4%) were infectious cases, with 14 being Mycobacterium tuberculosis infection (TB-HP), and 21 (28.4%) were malignancy-associated HP. Clinical and MRI characteristics exhibited variations among the four etiological groups. Hypoglycorrhachia was primarily observed in infectious and malignancy-associated HP. Immune-mediated HP was associated with a peripheral pattern of contrast enhancement and the Eiffel-by-night sign. MRI features strongly indicative of TB-HP included leptomeningeal involvement, brain parenchymal lesions, and arterial stroke. MPO-ANCA GPA was associated with a higher prevalence of spinal HP. CONCLUSIONS: Within our cohort, GPA and Mycobacterium tuberculosis emerged as the predominant causes of HP. We identified significant disparities in clinical and radiological features among different etiologies, which could have implications for diagnosis.

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INTRODUCTION: Hypertrophic pachymeningitis (HP) is a clinico-radiological entity characterized by a thickening of the dura mater that may be focal or diffuse and manifested by a variety of neurological syndromes. Aetiologically, it is classified as infectious, neoplastic, autoimmune, and idiopathic. Many of these formerly idiopathic cases have been shown to fall into the spectrum of IgG4-related disease. OBJECTIVE: To describe the case of a patient attended for neurological involvement due to hypertrophic pachymeningitis with initial diagnosis of inflammatory myofibroblastic tumour and final diagnosis of IgG4-related disease. CASE: A 25-year-old woman with neurological symptoms of 3 years' evolution characterized initially by right hypoacusis, evolving with headache and diplopia. Magnetic resonance imaging (MRI) of the encephalon showed pachymeningeal thickening with involvement of vasculo-nervous structures in the tip of the cerebellum, cavernous sinus, ragged foramen, and optic chiasm. The patient presented for consultation with the result of an incisional biopsy that reported a proliferative lesion combining fibrous elements of fascicular or swirling arrangement with collagenized streaks with dense, lymphoplasmacytic infiltrate and some macrophages, with negative staining for ALK 1, with a diagnosis of inflammatory myofibroblastic tumour. Due to suspicion of IgG4-related disease (IgG4-RD) the biopsy was sent for review and pertinent complementary studies were requested. BIOPSY REVIEW: Non storiform fibrosis, predominantly lymphoplasmacytic infiltrate, histiocytes, and polymorphonuclear infiltrate in sectors, without granulomas or atypia. Staining for germs negative. Immunohistochemistry with 50-60 IgG4+/HPF cells and range of 15%-20%, CD68+ in histiocytes, CD1a-, S100-. The patient presented deterioration of visual acuity due to ophthalmic nerve involvement, so glucocorticoid treatment was started in pulses and rituximab with regression of symptoms and imaging improvement of the lesions. CONCLUSION: HP is a clinical imaging syndrome with variable symptoms and aetiologies that poses a diagnostic challenge. In this case the initial diagnosis was inflammatory myofibroblastic tumour, which is a neoplasm of variable behaviour, locally aggressive, and can metastasize; it is one of the main differential diagnoses of IgG4-related disease because they share anatomopathological features, including storiform fibrosis. IgG4-RD is an immune-mediated condition that can have single or multiple involvement. Its diagnosis is complex when it presents with single organ involvement or in non-typical organs (CNS, meninges) in which data are scarce, as in the case of our patient with single organ involvement of the CNS. Although there are classification criteria to guide non-specialists in the diagnosis, the sum of the clinical picture, imaging, laboratory, pathological anatomy, and immunohistochemistry will always be evaluated together for a definitive diagnosis.

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Paciente de 65 años, con diagnóstico de Granulomatosis con Poliangeitis (GPA) de 18 años de evolución cuyo debut fue por insuficiencia respiratoria aguda asociado a hemoptisis recibiendo tratamiento con corticoides sistémicos y ciclofosfamida de inducción. Luego recibió mantenimiento con azatioprina 150 mg día, con periodos de recrudecimiento de enfermedad que respondieron al tratamiento con corticoides por períodos cortos. Acude a consulta por cefalea crónica de tres meses de evolución refractaria al tratamiento con antiinflamatorios no esteroides (AINES), asociado a proptosis ocular izquierda y dolor orbitario homolateral, presentando reactantes de fase aguda elevados (eritrosedimentación y Proteína C reactiva). Se evidencia por resonancia magnética nuclear cerebral con gadolinio, realce de la duramadre cerebral y tienda de cerebelo, presentando además una formación orbitaria izquierda.

A 65-year-old patient, with a diagnosis of Granulomatosis with Polyangeitis (GPA) of 18 years of evolution, whose debut was with respiratory failure and hemoptysis, receiving induction treatment with corticosteroids together with cyclophosphamide, and then maintenance treatment with azathioprine 150 mg per day, with periods of flare-up of the disease that responded to treatment with corticosteroids for short periods. He came to the clinic for a 3-month-long chronic headache refractory to treatment with non-steroidal anti-inflammatory drugs (NSAIDs), associated with left ocular proptosis and ipsilateral orbital pain, presenting elevated acute phase reactants (ers and c-reactive protein). It is evidenced by brain magnetic resonance with gadolinium, enhancement of the cerebral dura and cerebellum store, also presenting formation in the left orbit.

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ABSTRACT Granulomatosis with polyangiitis (GPA) is a vasculitic disease with an infrequent involvement of the central nervous system. This can lead, in rare cases, to hypertrophic pachymeningitis (HP), which is characterized by inflammation and fibrosis that cause a thickening of dura mater. At present, it is crucial to consider GPA in the differential diagnosis of elderly patients with intracranial hypertension. The case is presented of a 60-year-old male with progressive severe headache, vomiting, and wasting syndrome. Physical examination showed pallor, weight loss, and unilateral papilloedema. A gadolinium-enhanced brain MRI scan showed sinusitis, chronic otomastoiditis, and hypertrophic pachymeningitis. Finally, a meningeal biopsy concluded a necrotising granulomatous vasculitis compatible with GPA. However, PR3- and MPO-ANCA were negative. After corticosteroid therapy was initiated, the patient had a favorable outcome during his hospital stay.

RESUMEN La granulomatosis con poliangeítis (GPA) compromete excepcionalmente el sistema nervioso central, conllevando en raras ocasiones a una paquimeningitis hipertrófica (PH), caracterizada por inflamación y fibrosis, que originan un engrosamiento de la duramadre. Actualmente, su consideración es crucial en el diagnóstico diferencial de pacientes ancianos con hipertensión endocraneana. Presentamos el caso de un adulto de 60 anos con cefalea severa progresiva, vómitos, papiledema unilateral y síndrome consuntivo en donde la resonancia magnética cerebral contrastada con gadolinio muestra sinusitis, otomastoiditis y PH. Finalmente, la biopsia de meninges reveló vasculitis granulomatosa necrosante de pequenos y medianos vasos compatible con GPA. Empero, PR3- y MPO-ANCA resultaron negativos. Se inició terapia con corticoides, presentando una evolución clínica favorable durante su hospitalización.

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ABSTRACT Sjögren syndrome (SS) is an autoimmune disease, rarely affecting the central nervous system. The case is reported on a 68-year-old male patient who presented with keratocon-junctivitis sicca and xerostomia, during neuropsychological evaluation showed moderate to severe multidomain cognitive impairment. The magnetic resonance showed evidence of hypertrophic pachymeningitis. The CSF study showed pleocytosis, mild proteinuria, and negative cultures. The immune positive profile, Anti SS-A, SS-B and salivary gland biopsy was compatible with SS. The treatment was started with methylprednisolone pulses at a dose of 1g every 24 h for three days, and controlled with maintenance therapy, showing a favorable response. Chronic meningitis is a diagnostic challenge; among the less common causes is hypertrophic pachymeningitis in SS, and less frequently in male patients (1/9). Non-specific neurological manifestations should be considered, such as headaches or neuropsychiatric symptoms, as on some occasions rare cases may be encountered, such as the one described.

RESUMEN El síndrome de Sjögren (SS) es una enfermedad autoinmune que rara vez afecta al sistema nervioso central. En este informe de caso describimos a un paciente varón, de 68 años, que presentó queratoconjuntivitis seca y xerostomía, y a la evaluación neuropsicológica mostró deterioro cognitivo multidominio en grado de moderado a grave. La resonancia magnética mostró evidencia de paquimeningitis hipertrófica. El estudio del LCR mostró pleocitosis, proteinuria leve y cultivos negativos; el perfil inmunológico positivo anti SS-A, SS-B y la biopsia de la glándula salival compatible con SS. El tratamiento se inició con pulsos de metilprednisolona a una dosis de 1 g/cada 24 h/durante 3 días, control con terapia de mantenimiento, mostrando una respuesta favorable. La meningitis crónica es un desafío para el diagnóstico, entre las causas menos frecuentes está la paquimeningitis hipertrófica en el SS, y con menor frecuencia en los pacientes varones (1/9). Se deben considerar manifestaciones neurológicas inespecíficas, como dolores de cabeza o síntomas neuropsiquiátricos, ya que en algunas ocasiones podemos enfrentarnos a casos raros como el descrito.

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ABSTRACT Importance: Hypertrophic pachymeningitis (HP) is a non-usual manifestation of rheumatologic, infectious, and neoplastic diseases. Etiological diagnosis is a challenge, but when made promptly it creates a window of opportunity for treatment, with the possibility of a total reversal of symptoms. Observations: HP is an inflammatory process of the dura mater that can occur as a manifestation of sarcoidosis, granulomatosis with polyangiitis, and IgG4-related disease. The HP case evaluation is extensive and includes central nervous system imaging, cerebrospinal fluid analysis, serology, rheumatologic tests, and systemic survey for other manifestations sites. After systemic investigation, meningeal biopsy might be necessary. Etiology guides HP treatment, and autoimmune disorders are treated with corticosteroids alone or associated with an immunosuppressor. Conclusion: HP is a manifestation of several diseases, and a precise etiological diagnosis is crucial because of the difference among treatments. An extensive investigation of patients with HP helps early diagnosis and correct treatment.

RESUMO Importância: Paquimeningite hipertrófica (PH) é uma manifestação não usual de doenças reumatológicas, infecciosas e neoplásicas. O diagnóstico etiológico por vezes é um desafio, entretanto quando realizado em tempo cria uma janela de tratamento com a possibilidade de reversão total dos sintomas. Observações: A PH é um processo inflamatório da dura-máter que pode ocorrer como manifestação da sarcoidose, granulomatose com poliangeíte e doença relacionada à IgG4. A avaliação dos casos de PH é extensa e inclui imagem do sistema nervoso central, análise de líquor, sorologias, provas reumatológicas e rastreio sistêmico para doença em outros sítios. Por vezes, após toda a investigação sistêmica, a biópsia de meninge é necessária. A etiologia orienta o tratamento da HP, sendo que em doenças autoimunes adota-se o uso de corticosteroides isolados ou associados a um imunossupressor. Conclusão e Relevância: A PH é uma manifestação de várias doenças, e seu diagnóstico etiológico preciso é fundamental, visto a diferença entre os possíveis tratamentos. Uma investigação ampla nos casos de PH ajuda no diagnóstico precoce e tratamento adequado.

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Introducción: la inmunoglobulina G4 es un subtipo de inmunoglobulina G que corresponde a menos del 5% del total de inmunoglo-bulinas. Niveles elevados de esta molécula se han asociado con el desarrollo de diferentes patologías. Método: presentación de caso clínico. Resultados: se presenta el caso de una mujer de 51 años que consultó por disminución de agudeza visual, escotoma central en ojo izquierdo y edema de papila bilateral. Se encontraron signos compatibles con paquimeningitis en la resonancia nuclear magnética. También presentó aumento de presión de apertura en la punción lumbar. En el estudio de laboratorio se observó un aumento en los niveles plasmáticos de IgG4, lo cual llevó al diagnóstico de paquimeningitis relacionada con IgG4. La paciente fue tratada con corticoides y azatioprina, con excelente evolución posterior al normalizar agudeza visual y campos visuales. Conclusión: en este artículo se describen tres elementos importantes a considerar en estos pacientes: pérdida de visión, edema de discos ópticos y cambios en campo visual.

Introduction: G4 immunoglobulin is 5% of total immunoglobulins in plasma. Elevated levels of this protein are associated with several diseases. Methods: clinical case report. Results: we report a case of a 51-year-old woman who presented with loss of vision, a central scotoma over her left eye and bilateral optic disc edema. She had findings compatible with pachymeningitis based on magnetic resonance imaging. She also exhibited an increased opening pressure in lumbar puncture. In the laboratory workup, we found a two-fold increase in IgG4 levels, which led to a diagnosis of pachymeningitis related to IgG4. The patient was treated accordingly with corticosteroids and azathioprine. The patient experienced excellent progression with complete recovery of her visual acuity and normalization of the visual fields. Conclusion: there is three elements to consider in these patients: vision loss, optic disc edema and visual field changes.

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La paquimeningitis hipertrófica (PH), es una manifestación poco frecuente de la vasculitis asociada a anticuerpos anti-citoplasma de neutrófilo (ANCA). La literatura describe compromiso de sistema nervioso central (SNC) en 2-8% de los casos en pacientes con vasculitis pauciinmune. Se presenta el caso de un paciente con antecedente de vasculitis anti-mieloperoxidasa (MPO) con un mes de evolución de cefalea hemicraneana izquierda. La resonancia magnética cerebral contrastada evidencia marcado engrosamiento y realce meníngeo dural en el hemicráneo izquierdo, predominante en el tentorio y la fosa posterior. Se descartaron causas infecciosas por lo que se llegó a la conclusión de compromiso meníngeo asociado a vasculitis. Se inició manejo inmunosupresor con mejoría del cuadro clínico. La rápida identificación y manejo de esta entidad puede cambiar su pronóstico sombrío. Se realizó una revisión de la literatura para brindar una herramienta para la toma de decisiones para los médicos que se enfrentan a esta entidad.

Hypertrophic pachymeningitis (PH) is a rare manifestation of vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA). The literature describes central nervous system (CNS) involvement in 2-8% of cases in patients with pauciimmune vasculitis. We present the case of a patient with a history of anti-Myeloperoxidase (MPO) vasculitis with a 1-month history of left-sided headache. Contrast brain magnetic resonance was performed with evidence of marked thickening and dural meningeal enhancement in the left hemicranium, predominantly in the region of the tentorium and posterior fossa. Infectious causes were ruled out and the meningeal compromise associated with vasculitis was concluded. Immunosuppressive management was started with improvement of the clinical picture. Rapid identification and management of this entity can change its bleak outlook. A systematic review of the literature was carried out in order to provide a decision-making tool for physicians facing this entity.

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BACKGROUND AND PURPOSE: Although incidence rates vary, infectious, autoimmune, and neoplastic diseases can all cause chronic and subacute meningitis (CSM). We report a Latin-American, single center, CSM case series, analyzing the main clinical characteristics as well as ancillary diagnostic methods differentiating neoplastic from non-neoplastic etiologies. METHODS: Retrospective review of CSM cases from a single center in Buenos Aires, Argentina. RESULTS: Seventy patients with CSM diagnosis were identified, 49 with neoplastic and 21 with non-neoplastic meningitis. A history of previous cancer was significantly higher in neoplastic cases, whereas prevalence of autoimmune disease and fever was more common in non-neoplastic meningitis. C-reactive protein values were higher in non-neoplastic CSM, as was pleocytosis in cerebrospinal fluid analysis. The most frequent etiologies were breast and lung cancer for neoplastic meningitis cases; and idiopathic, tuberculous, and fungal infection for non-neoplastic cases. CONCLUSIONS: Chronic and subacute meningitis diagnosis is challenging in daily neurological practice. The results we report contribute information from Latin America regarding etiologies of CSM, which can be identified after a comprehensive evaluation in a majority of cases.

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Hypertrophic pachymeningitis is an infrequent disorder. It can be idiopathic or secondary to infectious, autoimmune or neoplastic disease. The recently described 〈IgG4-related disease〉 could be the origin of many cases considered cryptogenic. We present the case of a 60-year-old man, with a history of headache and episcleritis in both eyes, with partial response to corticoid therapy. The brain MR study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left temporal and occipital lobes to ipsilateral tentorium. Meningeal biopsy showed fibrosis and lymphoplasmacytic infiltrate, with more than 10 IgG4+ plasma cells per high power field. After treatment with rituximab there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging. Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be based on MRI findings if plasma IgG4 are elevated.

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La paquimeningitis hipertrófica es una enfermedad infrecuente. Puede ser idiopática, secundaria a infección o enfermedad oncológica. Recientemente se la describió asociada a la enfermedad por IgG4, pudiendo ser esta la causa de muchas clasificadas como idiopáticas. Se presenta el caso de un hombre de 60 años de edad con historia de cefalea y epiescleritis, con respuesta parcial a corticoides. La resonancia magnética con contraste evidenciaba refuerzo meníngeo a nivel temporal y occipital izquierdo con extensión a la tienda del cerebelo. La biopsia meníngea demostró fibrosis e infiltrado linfoplasmocitario con más de 10 células plasmáticas IgG4 positivas por campo de gran aumento. El tratamiento con rituximab provocó mejoría clínica y radiológica. La paquimeningitis hipertrófica es una manifestación de la enfermedad por IgG4 y debe ser sospechada con la resonancia magnética y niveles plasmáticos altos de IgG4.

Hypertrophic pachymeningitis is an infrequent disorder. It can be idiopathic or secondary to infectious, autoimmune or neoplastic disease. The recently described ‹IgG4-related disease› could be the origin of many cases considered cryptogenic. We present the case of a 60-year-old man, with a history of headache and episcleritis in both eyes, with partial response to corticoid therapy. The brain MR study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left temporal and occipital lobes to ipsilateral tentorium. Meningeal biopsy showed fibrosis and lymphoplasmacytic infiltrate, with more than 10 IgG4+ plasma cells per high power field. After treatment with rituximab there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging. Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be based on MRI findings if plasma IgG4 are elevated.

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Abstract Pachymeningitis is an uncommon disease with multiple etiologies with fibrosis of leptomeninges. One type of this disease is related to immunoglobulin G4 (IgG4). The most frequent manifestations include chronic cephalgia and focal neurological deficits. The case of a 31-year-old man with chronic cephalgia localized to the left hemicranium and left dysmetria is presented. Nuclear magnetic resonance imaging showed image hyperintensity and perilesional edema located at the infratentorial and left parietal occipital levels. The results of cerebral angiography and a cerebrospinal fluid examination were normal. A biopsy of the central nervous system (CNS) ruled out neoplastic lesions, granulomatous lesions, infection, and arteriovenous malformation and indicated thickened meningeal fragments. Elevated serum IgG4 levels were found. The antinuclear antibodies (ANAs), antibodies to extractable nuclear antigens (ENAs), anti-native DNA antibodies (anti-DNA), topoisomerase I antibodies (Scl-70), antibodies to cyclic citrullinated peptides (anti-CCP), and rheumatoid factor (RF) were negative. After an initial diagnosis of idiopathic hypertrophic cranial pachymeningi-tis, the patient received treatment with high doses of glucocorticosteroids for two years, without response, and developed Cushing's syndrome, cataracts, and osteoporosis. During the follow-up, dactylitis, distal upper limb sclerosis, and Raynaud's phenomenon occurred. A capillaroscopy indicated capillaries with dilated loops, microhemorrhaging, disorganization of the capillary architecture, and megacapillaries, which led to a diagnosis of limited systemic scleroderma. Although an association between scleroderma and pachymeningitis was originally proposed, review of the literature suggests that these are two different entities. Treatment with methotrexate, 25 mg weekly, allowed the withdrawal of glucocorticoids and resolution of neurological symptoms.

Resumen La paquimeningitis es el compromiso fibrosante de las leptomeninges poco común y de múltiple etiología. Una de ellas, la enfermedad relacionada a inmunoglobulina IgG4. Las manifestaciones clínicas más frecuentes son la cefalea crónica de difícil tratamiento y el déficit neurológico focal. Se presenta el caso de un hombre de 31 años, con cefalea crónica localizada en el hemicráneo izquierdo y disimetría izquierda, resonancia nuclear magnética con imagen hiperintensa y edema perilesional ubicada a nivel infratentorial y parieto-occipital izquierda, angiografía cerebral y estudio de líquido cefalorraquídeo normales. La biopsia del sistema nervioso central (SNC) descartó lesiones neoplásicas, granulomatosas, infección y malformación arteriovenosa, y reportó fragmentos meníngeos engrosados. A nivel sérico se encontraron altos niéveles de IgG4. ANA, ENA, anti-DNA, SCL-70, CCp y FR, negativos. Con el diagnóstico presuntivo inicial de paquimeningitis craneal hipertrófica idiopática, recibió tratamiento con dosis elevadas de glucocorticoides por espacio de 2 anos, sin buena respuesta y el desarrollo de síndrome de Cushing iatrogénico, cataratas y osteoporosis. Durante el seguimiento se documentó dactilitis, esclerosis distal de miembros superiores, síndrome de Raynaud y con capilaroscopia con asas dilatadas, microhemorragia, desorganización de la arquitectura capilar y megacapilares, configurándose el diagnóstico de esclerodermia sistémica limitada. Aunque se propuso inicialmente la asociación entre estas 2 condiciones, la revisión de la literatura indica que se trata de 2 entidades fisiopa-tológicamente diferentes. El tratamiento con metotrexato a dosis de 25 mg semanal permitió el retiro de los glucocorticoides y la resolución completa de los síntomas neurológicos.

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The spinal form of idiopathic hypertrophic pachymeningitis (IHP) is a rare condition characterized by a chronic progressive diffuse inflammatory fibrosis of the dura mater, which may evolve to the compression of the spinal cord. We present a case report about IHP focusing on its features in magnetic resonance imaging, which are determined by an intradural extramedullary mass in the cervical spine showing hypointensity on T2-weighted images and peripheral enhancement, causing compression of the spinal cord. Histological analysis showed a nonspecific chronic inflammatory process in dense fibrous tissue. The patient had a good outcome after therapy with steroids.

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La paquimeningitis hipertrófica idiopática crónica y la estenosis traqueal subglótica idiopática son raras condiciones de origen desconocido. En ambas existen un proceso inflamatorio fibrótico que afecta respectivamente, la duramadre y la traquea. La paquimeningitis hipertrófica idiopática crónica en forma típica, causa parálisis progresiva de nervios craneales, cefaleas, hipertensión intracraneal o disfunción cerebelosa. En la estenosis traqueal subglótica idiopática, usualmente limitada a la región subglótica y los dos primeros anillos traqueales, los sintomas son variables incluyendo disnea progresiva, sibilancias y estribor notables sobre el cuello. La paquimeningitis hipertrófica idiopática crónica ocurre en pacientes de todas las edades y el examen de elección para detectarla es la resonancia magnética cerebral, en tanto que la estenosis traqueal subglótica idiopática suele afectar mujeres jóvenes o maduras y es mejor evidenciada mediante tomografía computarizada. El diagnóstico es uno de exclusión. Deben descartarse enfermedades infecciosas como tuberculosis y lúes, y otras como sarcoidosis, carcinomatosis meníngea o vasculitis. Suelen responder inicialmente a los corticosteroides pero puede haber recurrencia al suspenderlos. La evolución a largo plazo es incierta. Este trabajo informa acerca de una paciente en quien ambas condiciones se dieron cita, discutiéndose sus manifestaciones clínicas, radiológicas y patológicas. Aunque considerada esencial para el diagnóstico, en nuestro caso no se realizó una biopsia meníngea. Planteamos la posibilidad de una asociación entre ambas condiciones, por su inicio simultáneo con recaídas, por su condición de inflamación crónica y recurrente y su respuesta a los corticosteroides.

Chronic idiopathic hypertrophic paquimeningitis and chronic subglottic tracheal stenosis are rare conditions of unknown origin. In both there is a fibrotic inflammatory process affecting, respectively the dura mater and the trachea. The chronic idiopathic hypertrophic paquimeningitis in its typical presentation causes progressive paralysis of cranial nerves, headaches, intracranial hypertensión or cerebellar dysfunction. In the chronic subglottic tracheal stenosis, usually limited to the subglottic region and the two first traqueal rings, its symptoms are variable including progressive shortness of breath, wheezing and stridor notable on the neck. The chronic idiopathic hypertrophic paquimeningitis occurs in patients of all ages and the test of choice for diagnosis it is the brain magnetic resonance image, while the chronic subglottic tracheal stenosis usually affect young or mature women and is best evidenced by computerizide tomography scan. The diagnósis is one of exclusion. Infectious diseases such as tuberculosis and lues, and others as sarcoidosis, meningeal carcinomatosis or vasculitides should be discarded. Often initially respond to corticosteroids but may have recurrences when stopped. The evolution in the long term is uncertain. This paper reports on a patient with both conditions, discussing their clinical, radiological and pathological manifestation. Although considered essential for the diagnosis, in our case was not performed a meningeal biopsy. We postulate the possibility of association between these two conditions, based in their simultaneous onset, the occurence of relapses in both diseases, its condition of recurrent and chronic inflammation, and its response to corticosteroids.

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La paquimeningitis hipertrófica es una enfermedad poco frecuente caracterizada por engrosamiento de la duramadre. Presentamos una paciente con esta enfermedad que se manifestó con cefalea crónica y en la que concomitantemente se evidenció una glomerulonefritis necrotizante extracapilar pauciinmune asociada a anticuerpos anticitoplasma de neutrófilos de patrón perinuclear (ANCA-P). El diagnóstico se estableció por resonancia nuclear magnética. Recibió tratamiento inmunosupresor con prednisona y ciclofosfamida con evolución favorable.

Hypertrophic pachymeningitis is a very unusual disease, the main characteristic of which is thickening of the dura mater. We describe a patient who started this illness showing chronic headache and pauci-immune necrotizing extracapillary perinuclear antineutrophil cytoplasmic antibody (P-ANCA) associated glomerulonephritis. The diagnosis was made by brain magnetic resonance image. She received immunosuppressant therapy with prednisonel and cyclophosphamide with clinical improvement.

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RESUMO

La paquimeningitis craneal hipertrófica idiopática es un raro proceso inflamatorio crónico de etiología desconocida que produce engrosamiento de la duramadre y alteraciones neurológicas progresivas debido a la compresión de estructuras vecinas. Se presenta el caso de una mujer adulta con un cuadro clínico crónico de cefalea, fiebre, pérdida progresiva de la visión y neuropatía óptica bilateral. El diagnóstico se basó en la visualización de la duramadre engrosada en las neuroimágenes y en la exclusión de causas conocidas mediante estudios histopatológicos. La terapia con corticoesteroides mejoró la cefalea pero no el déficit visual. El diagnóstico y el seguimiento de esta entidad se han facilitado con la utilización de la resonancia magnética cerebral con medio de contraste. La biopsia de duramadre sigue siendo la regla de oro para el diagnóstico definitivo de la enfermedad. La terapia con esteroides produce mejoría en un buen número de pacientes; sin embargo, son frecuentes las recaídas por lo que es necesario el uso concomitante de otro inmunosupresor como la ciclofosfamida o la azatioprina. La mortalidad es baja, pero son comunes las secuelas neurológicas definitivas.

Idiopathic hypertrophic cranial pachymeningitis is an infrequent chronic inflammatory process of unknown etiology which causes thickening of the dura mater and progressive neurologic alterations due to the compression of adjacent structures. A case is presented of an adult woman with a clinical syndrome consisting of headache, progressive visual loss and bilateral optic neuropathy. The diagnosis was based upon visualization of the thickened dura mater in neuroimaging studies and the exclusion of known causes by histopathological examination. Diagnosis and follow-up of this condition are currently easier with the use of nuclear magnetic resonance with contrast medium. Biopsy of the dura mater continues to be the gold standard for the definitive diagnosis of this disease. Steroid therapy causes clinical improvement in most of the patients; however, relapses are frequent, making necessary the concomitant use of other immunosuppressive agents such as cyclophosphamide or azathioprine. Mortality is low but definitive neurologic sequelae are common.

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