Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings.

Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment. Regarding the diagnosis workup, enhanced MRI is the most useful imaging method to evaluate dural thickening. This article addresses the MR imaging patterns of immune-mediated HP, including immunoglobulin G4-related disease, neurosarcoidosis, granulomatosis with polyangiitis, rheumatoid pachymeningitis, and idiopathic HP. The main infectious and neoplastic mimicking entities are also discussed with reference to conventional and advanced MR sequences.

A clinical approach to hypertrophic pachymeningitis.

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.

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.

Anterior Transpetrosal Approach and Microsurgical Resection of a Petrous Tentorial Meningioma: 3-Dimensional Operative Video.

Tentorial meningiomas are challenging tumors because of its complex relationship with vital neurovascular structures. We present the case of a 41-yr-old female with a history of right-sided facial numbness associated with pain around the ear. Magnetic resonance imaging demonstrated a lesion in the right tentorium edge closely related with the porus trigeminus, suggestive of a meningioma. Because of worsening of symptoms the patient underwent surgery for tumor removal. A standard temporo-zygomatic craniotomy was performed, followed by an extradural peeling of the middle fossa; the petrous apex was drilled allowing access to the posterior fossa dura. Dural opening was carried connecting the temporal and posterior fossa, and the tentorium was then cut to the incisura. The tumor was identified and completely removed reaching Simpson grade I resection. Postoperatively, the patient presented a right dry eye in the first days that fully improved, and also a right-sided facial paralysis (House-Brackmann grade IV) and diplopia, both recovered completely after 4 mo. We believe that facial paralysis was the result of an undesired traction of the geniculate ganglion, or upon the nerve itself. To avoid such complication, dissection over the GSPN must be carried parallel to that nerve. Facial numbness and pain improved with no neurological other deficits. Tentorial meningiomas are complex deep-seated lesions that can be successfully approached through an anterior transpetrosal route in selected cases. Informed consent was obtained from the patient for publication of this operative video. Anatomical images were a courtesy of the Rhoton Collection, American Association of Neurological Surgeons/Neurosurgical Research and Education Foundation.

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

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.

Craniocervical decompression with duraplasty and cerebellar tonsillectomy as treatment for Chiari malformation-I complicated with syringomyelia.

This study aimed to investigate the therapeutic effects of craniocervical decompression with duraplasty and cerebellar tonsillectomy for the treatment of Chiari malformation-I with syringomyelia (CM I-SM). From January 2005 to December 2011, 127 patients with CM I-SM underwent craniocervical decompression with duraplasty and cerebellar tonsillectomy and the therapeutic effects of these surgeries were evaluated using Tator scores. No patient in this study died or showed disease deterioration after the surgery. Re-examination by magnetic resonance imaging (MRI) showed that the cisterna magna was obviously larger after the operation in all but one patient. Moreover, syringomyelia (SM) was reduced in 76 patients. CM I-SM symptoms disappeared or decreased in 112 patients after following discharge. Follow-up was conducted in 84 of the patients and 79 of these patients exhibited improved symptoms. A second MRI re-examination showed that the cisterna magna was successfully constructed in 44 patients; 42 of these patients showed further eliminated or obviously reduced SM. Craniocervical decompression with duraplasty and cerebellar tonsillectomy achieved favorable therapeutic effects. Thus, craniocervical decompression with duraplasty and cerebellar tonsillectomy is a rational surgical approach with beneficial clinical effects. The proposed approach may have useful applications in the treatment of CM I-SM.

Clinical image and pathology of hypertrophic cranial pachymeningitis.

The objective of this study was to examine the clinical findings, magnetic resonance imaging (MRI), pathological features, and treatment experiments of patients with hypertrophic cranial pachymeningitis (HCP). The clinical findings, MRI, and pathological appearances of 9 patients with HCP were analyzed retrospectively. The thickened dura mater was markedly enhanced after contrast media injection. The lesion near the brain hemisphere presented long regions of T1- and T2-weighted abnormal signal intensities. The abnormal signal intensities of the brain tissue were decreased significantly. Pathological examination demonstrated chronic inflammation changes, with cerebral dura mater fibrous tissue showing obvious hyperplasia, and the periphery of the blood vessel showing a great quantity of infiltrating phlegmonosis cells. HCP mainly presents headache and paralysis of multiple cranial nerves. The distinctive signs on brain MRIs involve strengthening the signal in the cerebral dura.

Duropleural fistula revealed by neurological manifestations: an unusual cause of pleural effusion.

Symptomatic pleural collection of cerebrospinal fluid is a rare but accepted complication in hydrocephalic paediatric patients treated with ventriculopleural shunts. Few cases have been described in adults, usually as complication of trauma, tumours or spinal surgery, particularly post-laminectomy. It should be considered in the differential diagnosis of pleural effusion after neurosurgical procedures involving the spine. We describe two patients with large cerebrospinal fluid collections in the pleural cavity caused by postoperative duropleural fistula, who presented with neurological symptoms, cerebrospinal fluid pressure headache and meningitis.

Dural ectasia: a likely cause of inadequate spinal anaesthesia in two parturients with Marfan's syndrome.

We report two cases of Caesarean section in patients with Marfan's syndrome where continuous subarachnoid anaesthesia failed to provide an adequate surgical block. This was possibly because of dural ectasia, which was confirmed by a computed tomography scan in both cases.