Cranial pachymeningitis

INTRODUCTION: Pachymeningitis is a fibrous inflammatory process with non-specific symptoms, involving the dura mater. Due to MRI development, diagnosis is both easier and earlier. CURRENT KNOWLEDGE AND KEY POINTS: We report seven cases and review current literature. Clinical features are headaches and cranial nerve palsies. CSF shows inflammatory changes, while MRI evidences thickening of the dura mater. Disease etiologies in the present study were tuberculosis in two cases, sarcoidosis, Lyme disease, lymphoma and dural puncture; in one case only the disease was of unknown origin. The condition of six patients improved with specific treatment. FUTURE PROSPECTS AND PROJECTS: Further MRI development should allow detection of new forms of pachymeningitis and standardization of patients' management through the study of more important series.     

Idiopathic hypertrophic pachymeningitis with anticardiolipin antibody: A case report

Rationale:Idiopathic hypertrophic pachymeningitis (IHP) is a rare neurological disorder without a definite etiology. Diagnosis is mainly based on exclusion of other etiologies.Patient concerns:A 41-year-old male patient presented with insidious onset headache of 3-month duration.Diagnoses:Contrast-enhanced brain magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement over bilateral cerebral hemispheres and the tentorium cerebelli. Lumbar puncture showed increased pressure, lymphocytic pleocytosis, and elevated protein level with normal glucose concentration. Blood tests detected elevated erythrocyte sedimentation rate (ESR) and C-reactive protein. Pathological examination of the dura mater from the right frontal convexity disclosed coarse collagenous deposition with focal lymphoid aggregation. After malignancy and infectious etiologies were excluded, a diagnosis of IHP was made.Interventions:Oral prednisolone and azathioprine followed by methotrexate were administered.Outcomes:During the 7-year follow-up period, although the patient was not totally headache-free, medical therapy significantly reduced the severity of headache. Follow-up MRI studies showed a reduction in meningeal enhancement and serial ESR measurements revealed a trend of improvement.Lessons:Methotrexate therapy may be considered in cases of steroid-resistant IHP. In addition to clinical evaluation, serial ESR testing may be considered to guide the treatment strategy and assess the response to therapy.     

Acute monocular visual loss in carcinomatous hypertrophic pachymeningitis mimicking giant cell arteritis

This report describes a 69-year-old woman who presented with acute monocular visual loss, ipsilateral headache, and elevated sedimentation rate (ESR) and C-reactive protein (CRP). Both temporal artery biopsies were negative. Neuroimaging, dural biopsy, and breast biopsy all confirmed the diagnosis of carcinomatous hypertrophic pachymeningitis associated with metastatic breast carcinoma. After treatment with corticosteroids, her vision improved. Her clinical presentation initially mimicked the symptoms and signs of giant cell arteritis. Acute monocular visual loss without other cranial nerve palsies may be an uncommon presentation of hypertrophic pachymeningitis from metastatic breast carcinoma.     

肥厚性硬脑膜炎3例诊断分析及文献复习

肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)是一种少见的中枢神经系统慢性无菌性炎性疾病,其特点是硬脑膜的纤维性增生,引起神经系统进行性损害.其病因复杂,临床表现多种多样,容易误诊、漏诊.现报道我院收治的3例患者,结合文献分析如下……     

影像呈结节状占位样的特发性肥厚性硬脑膜炎临床分析

 目的 探讨影像呈结节状占位样的特发性肥厚性硬脑膜炎(IHCP)的临床、影像及病理特点。方法 对4例影像呈结节状的IHCP患者的临床资料进行回顾性分析。结果 4例均为男性,平均发病年龄40.25(33～50)岁,病程长(4个月～12年),病情反复发作。主要表现为慢性头痛、多组脑神经麻痹及癫痫发作。头颅影像示局部硬脑膜增厚,呈团块或结节状,与脑膜瘤等肿瘤相似。2例组织病理学检查示硬脑膜结缔组织增生、散在中性粒细胞及浆细胞浸润。甲泼尼龙或联合免疫抑制剂治疗有效。结论 结节状占位样IHCP多以慢性头痛起病,糖皮质激素或联合免疫抑制剂治疗有效,病情易复发。头颅影像可见硬脑膜肥厚,呈团块或结节状占位,易与脑肿瘤、炎性肉芽肿相混淆。
        

Mantle cell lymphoma manifesting neurological symptoms similar to those of hypertrophic cranial pachymeningitis

The patient was a 58-year-old man, who presented with headache and myodesopsia. He demonstrated papilledema and hemorrhage in the fundus of the left eye and MRI findings showed localized hypertrophic dura mater. He was diagnosed as having hypertrophic cranial pachymeningitis and treated with antibiotics and prednisolone. However, the patient complained of persistent headache. Therefore, CT scans of the chest and abdomen were obtained. These images demonstrated superficial and intraabdominal lymphadenopathy and a histological diagnosis of mantle cell lymphoma was made on biopsy of an inguinal mass. Specimen obtained at craniotomy also showed the same lymphoma cells diffusely infiltrating the dura mater. Complete remission of the lymphoma including disappearance of hypertrophic dura mater was obtained after 4 courses of rituximab plus hyper-CVAD alternating with high-dose methotrexate and cytarabine, and the neurological manifestation improved thereafter. Subsequently, he underwent autologous peripheral blood stem cell transplantation. Hypertrophic pachymeningitis is a chronic progressive inflammatory disorder with hypertrophic dura mater of brain and spinal cord, caused by diverse illnesses. Mantle cell lymphoma infiltrating the dura mater is extremely rare, and has not been reported previously. In some cases of hematological malignancy with hypertrophic pachymeningitis, dural biopsy is required to differentiate the etiology.     

Cerebrospinal fluid biomarkers implicated in the pathogenesis of anti-neutrophil cytoplasmic antibody-related hypertrophic pachymeningitis

Clinical Rheumatology - Hypertrophic pachymeningitis (HP) related to anti-neutrophil cytoplasmic antibody (ANCA) is the most frequently seen immune-mediated HP. We investigated cerebrospinal fluid...     

以肥厚性硬脑膜炎为主要表现的肉芽肿性多血管炎临床病例分析

目的探讨肉芽肿性多血管炎(GPA)继发肥厚性硬脑膜炎(HCP)的临床特点。方法回顾性分析北京协和医院2004—2018年收治的GPA继发HCP病例资料的特点。结果①GPA患者315例,19例继发HCP,占6.0%;②男性12例,女性7例;年龄19~64岁,中位年龄57岁。③神经系统表现:19例均有头痛,16例颅神经受累。受累部位:额部8例,颞部8例,颅底8例(鞍旁4例,其中海绵窦3例,眶尖2例),小脑幕6例,大脑镰2例,顶部1例,枕部1例,1例合并硬脊膜炎。④系统表现:发热10例,体质量下降8例,肺部受累4例,肾脏受累3例,16例鼻窦炎,10例中耳炎,16例局限型GPA。⑤15例ANCA抗体阳性,8例蛋白酶3(PR3)-ANCA阳性,6例髓过氧化物酶(MPO)-ANCA阳性。⑥16例行腰椎穿刺检查:脑脊液压力9例升高、5例正常、2例降低;脑脊液蛋白升高10例。⑦15例(78.9%)伯明翰系统性血管炎评分(BVAS)>15分。⑧19例均使用糖皮质激素、免疫抑制剂治疗,其中12例行甲泼尼龙冲击治疗,12例鞘内注射地塞米松(或+甲氨蝶呤),19例病情均缓解。结论HCP是GPA少见且严重的表现,主要表现为颅高压和颅神经受累,多见于局限型GPA患者,常伴有全身疾病的活动,需积极治疗。     

Rheumatoid factor positive hypertrophic cranial pachymeningitis in association with hypopituitarism and multiple cranial nerve palsies

This is the first report of a patient presenting with rheumatoid factor (RF) positive hypertrophic cranial pachymeningitis (HCP) in association with hypopituitarism and multiple cranial nerve palsies. Our patient developed palsies of the left II and III, bilateral VI and VII, and right IX, X, and XII cranial nerves. A stimulation test showed hypopituitarism due to hypothalamic failure. The patient was seropositive for RF but had no multiple joint pain or deformities. Magnetic resonance imaging (MRI) showed thickened dura of the sellar and parasellar region, hypothalamus, bilateral cavernous sinuses and the tentorium all of which were enhanced by gadolinium (Gd). Treatment with prednisone improved clinical symptoms and MRI findings concomitant with reduction of RF titer. Although the exact mechanism of HCP has not been clearly elucidated, the present case suggests an autoimmune mechanism associated with RF.     

Hypopituitarism and diabetes insipidus with localized hypertrophic pachymeningitis (Tolosa-Hunt syndrome) associated with Hashimoto thyroiditis

We report a 69-year-old woman with intracranial pachymeningitis showing hypopituitarism, diabetes insipidus, and Tolosa-Hunt syndrome associated with Hashimoto thyroiditis confirmed by autopsy. A large tumorous lesion of the hypothalamo-pituitary gland was revealed on magnetic resonance imaging, after the patient complained of gait and visual field disturbance. These symptoms subsided after thyroid hormone supplementation. Hypopituitarism and diabetes insipidus were diagnosed after cessation of the treatment by the patient herself. Multiple cranial nerve palsies and orbito-frontalgia appeared. Methylprednisolone pulse therapy improved the symptoms, but they recurred when the dose of glucocorticoid was decreased. The patient died of brain thrombosis. Autopsy revealed typical findings of Hashimoto thyroiditis and intracranial pachymeningitis involving the cranial base and pituitary gland. The high titer of rheumatoid factor and Hashimoto thyroiditis in this patient suggest an immunological role in the pathogenesis of pachymeningitis.     

Idiopathic hypertrophic cranial pachymeningitis treated by oral methotrexate: a case report and review of literature

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare clinical entity, characterized by a chronic inflammation causing thickening of the dura. Adequate therapeutic management is still a matter of debate. We present a patient with an IHCP, non-responsive to corticotherapy. Oral methotrexate was introduced (12.5 mg weekly) and total remission was observed after 6 weeks, both clinically and after neuro-imaging. We conclude that methotrexate can be effective and a therapeutical option in patients with IHCP who are resistant to corticotherapy or present major side-effects of chronic corticosteroids use.     

A case of MPO- and PR3-ANCA-positive hypertrophic cranial pachymeningitis with elevated serum IgG4

We report a case of orbital tumor and hypertrophic cranial pachymeningitis in a 64-year-old woman, who was initially suspected to have IgG4-related disease because of an elevated level of serum IgG4 at onset. However, her condition was resistant to glucocorticoid therapy, and additional cyclophosphamide was necessary to control the disease activity. Additional features included elevated levels of serum myeloperoxidase (MPO) and proteinase-3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and biopsy specimens from the orbital mass revealed very few infiltrating IgG4-positive cells. Instead, rupture of the elastic layer of the arterial walls with neovascularization and a small number of giant cells were observed. Considering these findings and the clinical course, the disease was considered more likely to be ANCA-associated pachymeningitis with elevation of the serum IgG4 level.     

Intravenous immunoglobulin-associated cranial pachymeningitis

To date, intravenous immunoglobulin (IvIg) has more often been considered as a safe medication. However, with the wider use of IvIg, severe side effects have also been reported to occur in IvIg-treated patients, notably aseptic meningitis. Other neurological complications have more rarely been described in patients receiving IvIg therapy, e.g. stroke or acute encephalopathy. We recently observed a case which is of particular interest, as the patient with steroid-refractory polyarteritis nodosa developed cranial pachymeningitis related to IvIg therapy. To our knowledge, this is the first reported case of cranial pachymeningitis complicating IvIg therapy. Our findings emphasize the importance of recognizing IvIg-related neurological complications in IvIg-treated patients. As cranial pachymeningitis is a fibrosing process, both recognition and management at an early stage are required to prevent definite neurological impairment in patients.