Spinal Pachymeningitis Research Articles

Spinal cord compression by cystic IgG4-related spinal pachymeningitis mimicking neurocysticercosis: a case report

BackgroundTo report a case of IgG4-related pachymeningitis presenting with cystic lesions mimicking neurocysticercosis.Case presentationA 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.ConclusionsThis 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.

IgG4-related Spinal Pachymeningitis Causing Spinal Cord Compression

IgG4-related Spinal Pachymeningitis Causing Spinal Cord Compression

Hypertrophic Spinal Pachymeningitis in a Patient with Chronic Antineutrophil Cytoplasmic Antibody–associated Nephritis: A Case Report

Hypertrophic Spinal Pachymeningitis in a Patient with Chronic Antineutrophil Cytoplasmic Antibody–associated Nephritis: A Case Report

Igg4 Related Granulomatous Spinal Pachymeningitis with Cord Compression – A Multisystem Disorder Presenting as Tumor Mimic

IgG4 Related Disease (IgG4 RD) is a comparatively new multisystem disorder associated with elevated levels of IgG4. It includes a number of disorders which were previously thought to be idiopathic or autoimmune. This is a rare condition and can affect any organ system in the body with IgG4 Related pachymeningitis having even lesser incidence. In this case report we present a patient who had chronic low back ache, who on evaluation was found to have a mass compressing on the spinal cord at the thoracic level, requiring surgical decompression. Biopsy and immunological testing were suggestive of IgG4 related pachymeningitis. We describe the diagnosis, medical management, and follow up of this patient, with discussion on how timely diagnosis and management will help in preventing further episodes and help in recovery. This case reiterates about medical conditions presenting as tumor mimics.

Pachymeningitis in Biopsy-Proven Sarcoidosis: Clinical Course, Radiographic Findings, Response to Treatment, and Long-Term Outcomes

Objective To study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time in response to treatment. Background Meningeal inflammation is one of the most common forms of neurosarcoidosis, occurring in 16-69% of affected patients. While the clinical and radiographic features of leptomeningitis in neurosarcoidosis are well known, those of pachymeningitis are far less clear. Design/Methods Patients with a diagnosis of neurosarcoidosis seen at Emory University [01/2011-8/2021] were included if pachymeningeal involvement was evident by MRI and the patient's sarcoidosis was pathologically confirmed (from a neural or extraneural site). Results 26/215 (12.1%) patients with neurosarcoidosis qualified for inclusion. Pathological confirmation came from neural tissue in 50%. Median age of onset was 43.5 years; most were male (16/26, 61.5%). Symptoms were primarily related to pachymeningitis in 20/26 (76.9%). Headache (19/26, 73.1%), visual dysfunction (12/26, 46.2%), and seizures (7/26, 26.9%) were the most common symptoms. All patients had cranial pachymeningitis; only a single patient undergoing spinal imaging (1/11, 9.1%) had spinal pachymeningitis. The falx cerebri (16/26, 61.5%) was the most commonly affected dural structure, but the anterior and middle cranial fossae and tentorium were frequently involved (12/26 each, 46.2%). The pachymeningeal lesions were unifocal (11/26, 42.3%) or multifocal (15/26, 57.7%) in distribution, nodular morphologically (23/25, 92.0%), and homogeneously enhancing (24/25, 96.0%). Symptomatic improvement occurred with steroids initially in 22/25 (88.0%). Ultimately, 23/26 (88.5%) required initiation of steroid-sparing immunosuppressants, including 8/26 (30.8%) eventually undergoing TNF inhibition. Pachymeningeal relapses occurred in 7/26 (26.9%). Median clinical follow-up was 48 months. Median mRS at last follow-up improved to 1.0 from 2.0 at presentation. Conclusions Sarcoid pachymeningitis often presents with headaches and visual dysfunction, usually affects the falx cerebri and anterior and middle cranial fossae, and tends to require steroid-sparing immunosuppressants. It has the potential to relapse, but the prospect for recovery is excellent.

Pachymeningitis in Biopsy-Proven Sarcoidosis: Clinical Course, Radiographic Findings, Response to Treatment, and Long-term Outcomes.

Background and ObjectivesMeningeal inflammation is one of the most common manifestations of neurosarcoidosis, occurring in 16%–69% of affected patients. While the clinical and radiographic features of leptomeningitis in neurosarcoidosis are well known, those of pachymeningitis are far less clear. Our primary aim was to study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time in response to treatment.MethodsPatients with a diagnosis of neurosarcoidosis seen at Emory University (January 2011–August 2021) were included if pachymeningeal involvement was evident by MRI and the patient's sarcoidosis was pathologically confirmed (from a CNS or non-CNS site).ResultsTwenty-six of 215 (12.1%) patients with neurosarcoidosis qualified for inclusion. Pathologic confirmation came from CNS tissue in 50%. The median age of onset was 43.5 years; most were male (16/26, 61.5%). Symptoms were primarily related to pachymeningitis in 20/26 (76.9%). Headache (19/26, 73.1%), visual dysfunction (12/26, 46.2%), and seizures (7/26, 26.9%) were the most common symptoms. All patients had cranial pachymeningitis; only a single patient undergoing spinal imaging (1/11, 9.1%) had spinal pachymeningitis. The falx cerebri (16/26, 61.5%) was the most commonly affected dural structure, but the anterior and middle cranial fossae and tentorium cerebelli were frequently involved (12/26 each, 46.2%). The pachymeningeal lesions were unifocal (11/26, 42.3%) or multifocal (15/26, 57.7%) in distribution, nodular morphologically (23/25, 92.0%), and homogeneously enhancing (24/25, 96.0%). Symptomatic improvement occurred with steroids initially in 22/25 (88.0%). Ultimately, 23/26 (88.5%) required initiation of steroid-sparing immunosuppressants, including 8/26 (30.8%) eventually undergoing TNF inhibition. Pachymeningeal relapses occurred in 7/26 (26.9%). The median clinical follow-up was 48 months. The median modified Rankin scale score at last follow-up improved to 1.0 from 2.0 at presentation.DiscussionPachymeningitis due to sarcoidosis often presents with headaches, visual dysfunction, and seizures; it usually affects the dura of the falx cerebri, anterior and middle cranial fossae, and tentorium cerebelli and tends to require steroid-sparing immunosuppressants. It has the potential to relapse, but the prospect for recovery is good.

Idiopathic hypertrophic spinal pachymeningitis with compressive myelopathy: a case report

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare condition characterized by chronic progressive diffuse inflammatory fibrosis in the dura mater of the spinal cord. Depending on its extent or severity, IHSP may cause symptoms by compressing the spinal cord. We present a case of IHSP across the cervical dura mater in a 78-year-old male patient. The patient visited the emergency department for right shoulder pain a month before admission, weakness in the right upper extremity a week before admission, and sudden quadriparesis on the day of admission. A neurological examination of the motor power of the extremities indicated grade IV quadriparesis and grade I hand grasp power in both hands. Hypoesthesia under the T1 dermatome was evaluated. Cervical magnetic resonance imaging (MRI) with gadolinium enhancement showed diffuse thickening of the anterior and posterior dura through the C2 to C5 levels, causing central canal stenosis and compressive myelopathy. Immediate decompressive laminoplasty was performed. A biopsy specimen of the thickened dura mater was obtained during surgery, and IHSP was diagnosed on the basis of the results of the pathological examination. High-dose steroid therapy was administered after decompressive surgery, and follow-up MRI showed radiological improvement in the lesion area.

Recurrent ‘Occult’ 18F-FDG Uptake in an Atypical Case of Anti-Myeloperoxidase Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

A 72-year-old woman, with anti-myeloperoxidase antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presented with two episodes of spinal pachymeningitis (at two different levels 9 years apart, cervical in 2011 and dorso-lumbar in 2020) associated with aortitis and only demonstrated by F-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT). This association between aortitis and pachymeningitis in AAV appears exceptional. Moreover, the relapse of aortitis and pachymeningitis in 2020 was not accompanied by an increase in ANCA.This case demonstrates the value of 18F-FDG PET/CT in the management of AAV, providing evidence of the recurrence and distribution of lesions in various organs, including those with unexpected involvement.LEARNING POINTSInvolvement of large vessels such as the aorta is rarely associated with anti-myeloperoxidase antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but has been described in a few cases. Possible aortic involvement should always be kept in mind while managing a patient with AAV.Pachymeningitis is rarely associated with AAV, but in case of unexplained and unspecific neurological symptoms in patients with AAV, such involvement should be considered.18F-FDG PET/CT is a promising tool for the management of patients with AAV, allowing unexpected sites, undetected by usual examinations, to be highlighted. In contrast to giant-cell arteritis, this exam has not, until now, been included in the recommended/systematic work-up of AAV.

Clinical analysis of 15 cases with myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis

To study the clinical features of myeloperoxidase(MPO) antineutrophil cytoplasmic antibody (ANCA) associated hypertrophic pachymeningitis (HP). Clinical data of 15 cases diagnosed with MPO-ANCA vasculitis complicated with HP were retrospectively analyzed. Nine cases were males and the other 6 were females, with an average age of (58±8) years. All cases presented with chronic headache. Contrast-enhanced magnetic resonance imaging (MRI) scan showed local or diffused thickening of cerebral and/or spinal dura matter while brain parenchyma were normal. Nine cases developed multiple cranial nerve paralysis, with trigeminal nerve and auditory nerve involved most commonly. The main clinical manifestations were facial pain, hearing loss and tinnitus. Two cases were complicated with hypertrophic spinal pachymeningitis (HSP) and 4 cases were complicated with pulmonary diseases. Positive serum perinuclear pattern ANCA (pANCA) and MPO could be found in all cases, positive serum IgG4 was seen in two patients. erythrocyte sedimentation rate(ESR;25-116 mm/1h) and C-reactive protein (CRP;29.02-146.00 mg/L) were both elevated in 14 cases. Nine cases had elevated intracranial pressure[180-235 mmH2O (1 mmH2O=0.009 8 kPa)] and abnormal protein level (457.6-3710.0 mg/L) in cerebrospinal fluid. Six cases were treated with glucocorticoids (prednisone 20-60 mg/d) and 9 cased with glucocorticoids and immunosuppressants (methotrexate 15 mg/week or cyclophosphamide 100 mg/d po). All patients achieved remission. MPO-ANCA associated HP is a special type of central nervous system involvement in ANCA associated vasculitis (AAV). It rarely involves the lung or kidney. Steroids and immunosuppressive agents are effective. In HP with unknown underlying diseases, it is suggested to screen ANCA and IgG4 tests for AAV or IgG4-related disease.

Idiopathic hypertrophic pachymeningitis as a rare cause of spinal cord compression

Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease characterized by inflammation and hypertrophy of the dura mater. Spinal form of IHP is extremely rare. We report an idiopathic hypertrophic spinal pachymeningitis case, a 67-year-old female who presented with neck and upper back pain with progressive paraparesis and sensory level. Her magnetic resonance imaging cervico-dorsal spine showed abnormal epidural hypointensity on T2-weighted images from C5 to T4 with significant cord compression and intramedullary cord signal changes at T2 and T3 levels. The lesional biopsy was done suggestive of chronic inflammatory changes. She was treated with steroids and was able to walk unaided.

Rare Causes of Hypertrophic Spinal Pachymeningitis Primarily Identified on Spinal MRI.

Rare Causes of Hypertrophic Spinal Pachymeningitis Primarily Identified on Spinal MRI.

Hypertrophic spinal pachymeningitis caused by ANCA-associated vasculitis revealed by 18F-FDG PET/CT

Rationale:Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can involve the central nervous system in estimatedly 15% of patients. Hypertrophic pachymeningitis causes inflammatory hypertrophy of the cranial or spinal dura mater and patients present with various neurological deficits. ANCA-associated hypertrophic spinal pachymeningitis has rarely been reported in literature. We report a case of AAV presenting with hypertrophic spinal pachymeningitis detected by 18F-FDG PET/CT.Patient concerns:A 66-year-old woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis developed back pain, bilateral lower limb weakness, dysuria, and dysporia 1 month ago.Diagnosis:Contrast-enhanced MRI showed thickening and enhancement of the dura mater in the thoracic cord. Intraspinal hypermetabolism in the corresponding region was observed on 18F-FDG PET/CT. The patient was finally diagnosed with ANCA-associated hypertrophic spinal pachymeningitis.Interventions:The patient was treated with a higher dose of prednisone and cyclophosphamide.Outcomes:After 2-week treatment, the patient's neurological symptoms improved rapidly and laboratory findings were ameliorated. A repeated contrast-enhanced MRI showed partial improvement of the disease in the thoracic cord.Lessons:18F-FDG PET/CT and contrast-enhanced MRI can aid in the clinical diagnosis and surveillance in AAV-associated hypertrophic spinal pachymeningitis and potentially facilitate early recognition and intervention to prevent irreversible neurological impairment.

The Idiopathic Hypertrophic Spinal Pachymeningitis

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory condition characterized by chronic inflammatory hypertrophy of the dura mater. It can involve the entire spine. However, most cases are reported in the cervical and thoracic spine. It can progress from local pain to radiculopathy and eventually develop myelopathy. The aetiology of IHSP is not known. However, it has been suggested to be associated with many diseases. Here we report a case of IHSP in 21-year-old female who presented with paraplegia. The diagnosis was made on MRI Spine and histopathological examination. It was treated with surgical decompression, steroid therapy and patient improved gradually.

Myeloperoxidase-Antineutrophil Cytoplasmic Antibody Positive Hypertrophic Spinal Pachymeningitis at the Cervicothoracic Junction: A Case Report.

Myeloperoxidase-Antineutrophil Cytoplasmic Antibody Positive Hypertrophic Spinal Pachymeningitis at the Cervicothoracic Junction: A Case Report.

Hypertrophic Pachymeningitis in Chinese Patients: Presentation, Radiological Findings, and Clinical Course.

Background Hypertrophic pachymeningitis (HP) is generally regarded as a rare inflammatory disease, which results in a diffuse thickening of the dura mater. We retrospectively collected data from patients with HP. Methods A total of 16 patients with HP were included in our study. The clinical features, laboratory evaluation, imaging findings, treatment, and outcome were reviewed. Results Of the 16 cases, half were male, with a mean age of 52.6 ± 13.2 years. The mean duration from onset to diagnosis was 8.6 months. The most frequent presenting symptoms in HP cases were a recurrently chronic headache (81.3%) and multiple cranial nerve injury (50%). Antineutrophil cytoplasmic antibody- (ANCA-) related HP was found in 5 cases and IgG4-related HP in 1 case. The intracranial pressure was elevated in 4 cases. The cerebrospinal fluid (CSF) had lymphocytosis in 5 cases and increased protein in 12 cases. Immunoglobulins (IgG, IgA, and IgM) and protein showed linear relationships in the CSF. On magnetic resonance imaging (MRI), localized or diffuse dura maters were thickened in all cases. HP combined with subacute subdural hemorrhage or hypertrophic spinal pachymeningitis was also observed in individual cases. Biopsy of the dura mater in one case showed amounts of inflammatory cells infiltrating, with an increased percentage of IgG4-positive plasma cells. Of all cases referring to glucocorticoid treatment, the symptoms have improved significantly in 10 cases. In other 6 cases, mycophenolate mofetil or azathioprine was added. All patients showed clinical improvement at the follow-up visits. Conclusion The clinical characters of HP are chronic onset, recurrently chronic headache, and multiple cranial nerves paralysis. Inflammatory changes in CSF caused by intrathecal synthesis of immunoglobulin, characteristic dural enhancement on MRI, and pathologic biopsy are all helpful for diagnosis. The addition of immunosuppressant, especially mycophenolate mofetil, is a good choice for steroid-resistance HP.

ANCA-Associated Hypertrophic Spinal Pachymeningitis Presenting with Longitudinally Extensive Transverse Myelitis: A Case Report.

ANCA-Associated Hypertrophic Spinal Pachymeningitis Presenting with Longitudinally Extensive Transverse Myelitis: A Case Report.

Idiopathic Hypertrophic Dorsal Pachymeningitis - Imaging Features

Idiopathic Hypertrophic Spinal Pachymeningitis (IHSP) is a rare disease causing chronic inflammatory hypertrophy of the dura mater. It may be idiopathic or secondary to other diseases. It is an extremely rare cause of spinal cord compression. This process can be present throughout the entire spine but is commonly reported in the cervical and dorsal spine. IHSP is a diagnosis of exclusion since it is associated with trauma, infection, and autoimmune diseases. We describe a rare case of Idiopathic Hypertrophic Dorsal Pachymeningitis (IHDP) in a 42 -year -old woman presenting with progressive weakness of both lower limbs for the past 2 years and outline the important clinical and radiographic features of the disease.

An orphan disease: IgG4-related spinal pachymeningitis: report of 2 cases

IgG4-related disease is relatively new disease entity and a rare one, and our knowledge of this entity continues to evolve. It was first described in the pancreas and since then has been described in virtually every organ. Spinal involvement resulting in pachymeningitis is rare, and there are only 8 reported cases of the same to date, with the cervicothoracic spine being the most commonly affected region. The authors describe 2 cases in which the patients presented with spinal compression resulting in myeloradiculopathy (Case 1) and radiculopathy (Case 2). Imaging of spine in both cases revealed an ill-defined contrast-enhancing lesion at the lumbar level. Preoperatively, a diagnosis of spinal tumor was made, but intraoperatively no spinal tumor was found. The diagnosis was established histopathologically. The disease has no particular defining features clinically or radiologically and can mimic common spinal tumors. It is important to accurately diagnose this rare entity because of its multisystem involvement and progressive course. Strict treatment guidelines have yet to be formulated. Although histologically this disease can mimic other inflammatory conditions, the presence of storiform fibrosis and an increased number of IgG4-positive plasma cells can help in clarifying the diagnosis.

Comparative analysis between hypertrophic spinal pachymeningitis and epidural meningioma

Comparative analysis between hypertrophic spinal pachymeningitis and epidural meningioma

Effective Response of Methotrexate for Recurrent Idiopathic Hypertrophic Spinal Pachymeningitis.

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic progressive and diffuse inflammatory fibrosis of the spinal dura mater. Though treatment of IHSP is surgical decompression with steroid therapy, treatment for recurrent IHSP is controversial. Our patient was diagnosed with IHSP based on magnetic resonance imaging (MRI) and underwent laminectomy for decompression following steroid pulse therapy. Despite maintenance of steroid therapy, the patient experienced 3 recurrences. As an alternative immunosuppressant medication, methotrexate was introduced with low-dose steroid. Fortunately, the symptom was resolved, and a decrease of dura thickening was revealed on MRI. We present the case and suggest that methotrexate might be an effective treatment modality for recurrent IHSP.