A clinical approach to hypertrophic pachymeningitis / Abordagem diagnóstica para paquimeningite hipertrófica

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.

Ectasia dural e hipotensión endocraneal en síndrome de Marfán / Dural ectasia and intracranial hypotension in Marfan syndrome

INTRODUCCIÓN: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE). OBJETIVO: Pre sentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán. CASO CLÍNICO: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica. CONCLUSIONES: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Resonancia magnética de columna cervical con secuencia en flexión para el diagnóstico de la enfermedad de Hirayama / Magnetic resonance of the cervical spine with sequence in flexion for the diagnosis of Hirayama disease

La enfermedad de Hirayama o atrofia muscular espinal juvenil no progresiva de las extremidades superiores es una clase de mielopatía relacionada con la flexión del cuello. Afecta principalmente a hombres jóvenes (entre 15 y 25 años) y se caracteriza por una debilidad muscular asimétrica y unilateral de miembros superiores con atrofia. Suele presentarse de manera insidiosa, con curso progresivo y autolimitado a los 3-4 años del inicio del cuadro. Se cree que es producida por trastornos isquémicos en la microcirculación de las astas anteriores del segmento medular cervical entre C8 y T1 por la compresión en el segmento medular anterior debido al desplazamiento anterior de la duramadre al flexionar el cuello. Si bien existen varias teorías sobre la causa de este deslizamiento, la más aceptada se relaciona con la falta de crecimiento de la duramadre con respecto a la columna durante la pubertad. Esto provocaría un aumento de la tensión de la dura posteriory, como consecuencia, el desplazamiento anterior durante la flexión. Dado su excelente contraste tisular y la posibilidad de realizar adquisiciones en distintos planos, la resonancia magnética es el estudio de elección. Las imágenes deben ser obtenidas en posición neutra y en flexión cervical máxima para poner de manifiesto el desplazamiento de la dura, con el consiguiente aumento de la sensibilidad y especificidad de la prueba. Así, se logra mayor confianza en el diagnóstico y menor cantidad de falsos positivos, en comparación con la posición neutra como única adquisición.

Hirayama disease is a type of myelopathy related to neck flexion. It affects young male adults between 15 and 25 years, and is characterised by unilateral and asymmetric upper limb muscle weakness with atrophy. It usually presents insidiously, with a progressive course and self-limits in 3-4 years. It is believed that it could be produced by ischaemic disorders in the microcirculation of the anterior horns of the cervical spine segment C8 and T1 due to anterior displacement of the dura. There are several theories for the cause of this displacement, with the most accepted being the relationship between the lack of growth of the dura mater and the spine during puberty. This increases the tension of the posterior dura mater and consequently the anterior displacement during flexion. Due to its excellent tissue contrast and the possibility of acquisitions in different planes, magnetic resonance imaging is the study of choice. Images must be obtained in both neutral and cervical flexion to highlight the displacement of the dura mater. This increases the sensitivity and specificity of the test, giving greater confidence in the diagnosis, and reducing false positives compared to neutral as a single acquisition.

Massive falx cerebri empyema.

A rare case of otogenic massive intra-falx empyema is reported. The patient presented with headache, vomiting and left lower limb weakness of 8 months duration. C.T. scan revealed two cavities in the falx. Right fronto-parietal craniectomy and drainage of massive empyema was undertaken through interhemispheric approach with wide opening of the falx, evacuation of voluminous amount of thick pus and marsupalization of flax with dura. The patient showed complete recovery. The relevant literature is briefly reviewed.