Hypertrophic Cranial Pachymeningitis Research Articles

Clinical characteristics of hypertrophic cranial pachymeningitis in granulomatosis with polyangiitis: a retrospective single-center study in China.

Hypertrophic cranial pachymeningitis (HCP) is uncommon but a poorly understood complication of granulomatosis with polyangiitis (GPA). We conducted this retrospective study to elucidate the clinical characteristics and factors independently associated with granulomatosis with polyangiitis (GPA) complicated by hypertrophic cranial pachymeningitis (HCP) in China. We collected the medical records of 78 patients diagnosed with GPA who were admitted to the inpatient department of Peking Union Medical College Hospital between January 2003 and September 2021. Clinical features, laboratory and radiological findings, and Birmingham Vasculitis Activity Scores (excluding meningitis score) were recorded. A binary logistic regression analysis was performed to analyze factors independently associated with GPA-related HCP. Headache (100%) and cranial nerve palsy (61.5%) were common manifestations of HCP. Compared to 52 GPA patients without HCP, 26 patients with HCP required more time from initial symptoms to diagnosis, with a lower ratio of pulmonary and renal involvement, a higher ratio of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity, conductive or sensorineural hearing loss, mastoiditis, and decreased vision or sudden visual loss. Binary logistic regression analysis indicated that proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) negativity (OR 10.698, p = 0.001), conductive or sensorineural hearing loss (OR 10.855, p = 0.005), and decreased vision or sudden visual loss (OR 8.647, p = 0.015) were significantly associated with GPA-related HCP. Of the 26 patients, 18 received methylprednisolone pulse treatment, and 18 received intrathecal injections of dexamethasone and methotrexate. HCP was a severe manifestation of GPA in our study. Independent factors associated with the occurrence of HCP in patients with GPA included PR3-ANCA negativity, conductive or sensorineural hearing loss, and decreased vision or sudden visual loss. Furthermore, GPA-related HCP was associated with higher disease activity, requiring more intensive treatments.

Clinical Observation of Various Types of Idiopathic Hypertrophic Cranial Pachymeningitis

To assist doctors in making better treatment decisions and improve patient prognosis, it is important to determine which therapy modalities are suitable for various forms of idiopathic hypertrophic cranial pachymeningitis (IHCP). All cases were received from the hospital medical record system, and some follow-up information was gathered through telephone follow-up. A total of 26 patients, 14 men and 12 women, with ages ranging from 20 to 73 years and a mean of 47.42 years, were included in the research. Regular types were less likely to recur than irregular and nodular types, focal types were less likely to recur than diffuse types, and corticosteroid-refractory types were more likely to recur than corticosteroid-sensitive types. The extent and shape of the lesion and susceptibility to corticosteroids are potential factors that could influence recurrence. Futhermore, this paper also proposes the fibroblasts as a new therapeutic target which may improve the quality of prognostic survival of patients.

A Hypertrophic Spinal Pachymeningitis Patient With Factor V Leiden (G1691A), MTHFR C677T, MTHFR A1298C, PAI-1 4G-5G, Glycoprotein IIIa L33P Gene Mutations

Hypertrophic pachymeningitis (HP) is a rare clinical entity of diverse etiology, characterized by a chronic inflammation that causes dura thickening. Reports of Idiopathic hypertrophic cranial pachymeningitis (IHCP) were related to infections, trauma, tumors, and rheumatologic conditions. It was first described by Charcot and Joffroy regarding spinal meninges in 1869. HP has three stages; progressive radicular symptoms begin first, then muscle weakness and atrophy start. Findings such as paraplegia, loss of bladder and bowel control, and respiratory distress caused by intercostal and diaphragmatic denervation are considered the third stage of the disease. Especially in the cranial form of the disease, nerve ischemia and various cranial neuropathic findings may occur. Factor V Leiden (G1691A), MTHFR C677T, MTHFR A1298C, and PAI-1 4G-5G gene mutation analysis were measured with an ABI Prism. In this case report, the authors present a case of hypertrophic mutations pachymeningitis with Factor V Leiden (G1691A), MTHFR C677T, MTHFR A1298C, PAI-1 4G-5G, Glycoprotein IIIa L33P gene. In conclusion, we report a case of HP with Factor V Leiden (G1691A), MTHFR C677T, MTHFR A1298C, PAI-1 4G-5G, and Glycoprotein IIIa L33P gene mutations. We emphasize that the identification of pachymeningitis can be easily bypassed with the application of limited laboratory techniques. As in this case report, we think that these mutations should be analyzed in patients diagnosed with pachymeningitis.

Idiopathic hypertrophic pachymeningitis, a case report

Idiopathic hypertrophic cranial pachymeningitis (ICHP) is a rare entity characterized by chronic inflammation resulting in the thickening of the dura mater. The first cases of IIHCP were related to tuberculosis or syphilis. Other causes are rheumatologic diseases, solid neoplasms or lymphoproliferative processes. After extensive evaluation by neuroimaging, lumbar puncture and serum markers (immunological-infectious-granulomatous) and no cause is founded, it is considered idiopathic in origin. Due to the lack of pathophysiological knowledge (whose hypotésis is possibly inflammatory), the appropriate therapeutic management is still a matter of debate.

Idiopathic hypertrophic cranial pachymeningitis successfully treated with dexamethasone – a case report

Introduction : Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare pathological entity which is diagnosed after the exclusion of secondary reasons. Most frequently, IHCP is related to rheumatological diseases such as rheumatic arthritis, sarcoidosis, anti-neutrophil cytoplasmic antibody-associated vasculitis, and IgG4-related disorders. The inflammatory process concerns dura mater and manifests with symptoms of cranial nerve damage or compression. Case description : A 59-year-old Caucasian man presented with transitional episodes of tinnitus and an unspecific headache. Imaging studies (magnetic resonance imaging – MRI) demonstrated a thickened dura mater. Lymphoma was suspected due to an abnormal flow cytometric analysis of a cerebrospinal fluid (CSF) sample. Hematological diagnostics including bone marrow biopsy, whole body computed tomography (CT) and digestion tract examination were performed leading to the exclusion of hematological malignancy. The patient underwent surgery, and a specimen from the lesion was obtained. On this basis, intracranial idiopathic hypertrophic pachymeningitis was diagnosed. First line treatment strategy was introduced with corticosteroids with a gradual dose reduction. After the 48-month follow-up, we observed an alleviation of symptoms with a gradual significant decrease in the thickening of dura matter with almost complete resolution. Conclusions : Idiopathic hypertrophic pachymeningitis should be considered in the differential diagnosis of a thickened dura mater. Administration of steroids appears reasonable as a first line treatment of IHCP.

A case of idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa–Hunt syndrome in an elderly female

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an extremely rare clinical entity of unknown etiology, characterized by a chronic inflammation causing thickening of the dura mater.[1] ICHP can produce similar presentation to Tolosa–Hunt syndrome (THS) if it involves cavernous sinus.[2] THS might represent a focal manifestation of IHCP.[2] The THS is a rare syndrome with an estimated annual incidence of one case per million per year.[3] It typically presents with orbital pain associated with palsy of the third, fourth, or sixth cranial nerve. We present an interesting case of a patient with IHCP associated with THS in an elderly female who responded well to high-dose steroids and in a few days had significant improvement in her retro-orbital pain and ocular movements. The importance of appropriate neuroimaging and serological investigation in patients with suspected THS, role of erythrocyte sedimentation rate in follow-up of these patients, and the relationship between IHCP and THS are discussed.

Hypertrophic cranial pachymeningitis coinfection with tuberculosis and actinomycosis.

This is a rare case report about hypertrophic cranial pachymeningitis coinfection with tuberculosis and actinomycosis in a 35-year-old male. The patient presented with progressive headache, paraesthesia, and blurred vision. Dural biopsy, histology, and cultures are imperative in pachymeningitis for establishing the diagnosis and guiding treatment.

Magnetic resonance imaging indicator of the causes of optic neuropathy in IgG4-related ophthalmic disease

BackgroundThe following study investigates the involvement of optic neuropathy in IgG4-related ophthalmic diseases (IgG4-ROD) based on the magnetic resonance imaging (MRI) data, and different imaging features of IgG4-ROD related optic neuropathy related to other orbital diseases.MethodsThis retrospective study included 225 patients with IgG4-RD admitted at two ophthalmology centers between January 2014 and December 2017. Twenty-six patients had both pre-therapeutic orbital MRI and optic never injury. The causes of optic neuropathy were analyzed, and the special sign in MRI to diagnose IgG4-ROD was also evaluated.ResultsTwelve cases had inflammation of the optic nerve sheath, while 14 cases had compression due to extraocular muscles and pseudo tumor masses. Two cases had hypertrophic cranial pachymeningitis, while one case had hypophysis involving optic chiasma.ConclusionThe most common causes of optic nerve injury in IgG-4 ROD are inflammation of optic nerve sheath, compression of extraocular muscles, pseudo tumor mass and hypertrophic cranial pachymeningitis, and hypophysis involving optic chiasma.

Pituitary involvement in patients with granulomatosis with polyangiitis: case series and literature review

GPA with pituitary involvement is a rare condition which is prone to be misdiagnosed. The aim of this study was to summarize clinical features of pituitary involvement in GPA and facilitate early diagnosis. Twelve GPA patients were retrospectively analyzed at a single hospital between 2000 and 2017. A literature review was conducted to compare previous findings with our clinical results. The incidence rate of pituitary involvement in GPA was 3.9% (12/304) without sexual predilection. Other impairments included ear, nose and throat (n = 12), oculi (n = 10), lung (n = 6), meninges (n = 4), kidney (n = 3), and skin (n = 2). Antineutrophil cytoplasmic antibodies (ANCA) were positive in all patients with lung or kidney involvement (n = 6/6), while ANCA were negative in almost all patients without lung or kidney involvement (n = 5/6). Endocrine abnormalities included central diabetes insipidus (CDI, n = 11/12) hypogonadotropic hypogonadism (n = 6/11), adrenocorticotropic hormone deficiency (n = 4/7), thyroid-stimulating hormone deficiency (n = 5/11), and growth hormone deficiency (n = 3/9). Enlarged pituitary gland (n = 6), absence of posterior hyperintense signal on T1-weighed images (n = 11) and hypertrophic cranial pachymeningitis (n = 4) were common radiological manifestations. After treatment, nine patients experienced remission but one died. Pituitary images of 3/4 patients showed size of pituitary lesions decreased. CDI was not alleviated and hypopituitarism remained in two patients. Pituitary involvement in GPA can occur at any time throughout the course of disease, including at the initial presentation. GPA could not be excluded based on negative-ANCA in patients with pituitary abnormality alone. CDI and hypogonadotropic hypogonadism are dominant endocrine abnormalities. Systemic diseases may alleviate and pituitary images may improve after treatment, though the recovery of pituitary function is rare.

The clinical and imaging features of hypertrophic pachymeningitis: a clinical analysis on 22 patients.

To explore the clinical and imaging features of patients with hypertrophic cranial pachymeningitis (HCP). A retrospective study was performed on 22 patients with HCP diagnosed at the Affiliated Hospital of Xuzhou Medical University from February 2014 to September 2017. A headache was present as an initial symptom in 18 patients. The headache was associated with the loss of vision (2 cases), facial pain (1 case), and unsteady walking (1 case). Other symptoms included cranial nerve dysfunction (15 cases), cerebellar ataxia (4 cases), and sinus thrombosis (3 cases). In the laboratory tests, 7 patients showed an increased number of white blood cells, higher levels of C-reaction protein (CRP), and erythrocyte sedimentation rate (ESR). An elevated level of immunoglobulin G4 (IgG4) and the presence of the anti-neutrophil cytoplasmic antibody (ANCA) were found in 3 and 2 patients respectively. There were 17 patients who had abnormalities in their cerebrospinal fluid (CSF) on lumbar puncture. On magnetic resonance imaging (MRI), a local or generalized thickening was observed in the cerebral falx, the tentorium of the cerebellum, the fronto-parietal lobe, the occipito-parietal lobe, and the dura of skull base. A dural biopsy obtained in one case showed a variety of inflammatory changes. An immunohistochemical analysis revealed the positivity of CD138, IgG, and IgG4 in some cells. All 22 patients had a good response to corticosteroids. HCP mainly leads to a headache and the paralysis of multiple cranial nerves. A biopsy and MRI are often required and serve as the basis for the diagnosis and effective therapy.

Idiopathic Hypertrophic Cranial Pachymeningitis misdiagnosed as venous sinus thrombosis. (P6.198)

Idiopathic Hypertrophic Cranial Pachymeningitis misdiagnosed as venous sinus thrombosis. (P6.198)

Focal status epilepticus as a manifestation of idiopathic hypertrophic cranial pachymeningitis

BackgroundIdiopathic hypertrophic cranial pachymeningitis (IHCP) is an uncommon disease of unknown etiology characterized by thickening of the cerebral dura mater with possible associated inflammation. The most frequently described clinical symptoms include headache, cranial nerve palsy, and cerebellar dysfunction. Epilepsy and/or status epilepticus as main presentation is very uncommon. Case presentationTwo consecutive cases are presented of patients manifesting focal status epilepticus secondary to IHCP, with clinical, laboratory [blood test and cerebrospinal fluid (CSF) analysis], neuroradiologic [magnetic resonance imaging (MRI) at 3 Tesla and digital subtraction angiography (DSA)], and therapeutic data. One patient underwent meningeal biopsy; pathology findings are also included. Corticosteroid therapy resulted in clinical improvement in both cases, and neuroimaging showed decreased abnormal morphology, compared to initial findings. ConclusionIn the diagnostic approach to focal status epilepticus or epilepsy, IHCP must be considered a potential, although extremely infrequent, cause. Anti-inflammatory treatment is an effective addition to antiepileptic drug therapy in patients with IHCP.

A case of idiopathic hypertrophic cranial pachymeningitis presenting as chronic subdural hematoma

A 26-year-old male presented with a 6-day history of paroxysmal headache which was worsen with nausea and vomiting for 1 day. Head CT on admission revealed left chronic subdural hematoma with midline shift. An emergency Burr hole drainage for hematoma was performed. Headache recurred 6 days later. MRI of the brain revealed a diffuse thickening and a gadolinium-enhancement of the falx, cranial dura mater and tentorium cerebelli on the left side with pia mater involved. Lumber puncture showed increased intracranial pressure and elevated IgG level in cerebrospinal fluid. Histological examination of the biopsy specimen showed thickened, fibrotic dura with a sterile chronic inflammation. According to pathological examination, idiopathic hypertrophic cranial pachymeningitis was considered as the final diagnosis. Symptoms were improved with steroid pulse therapy.

Idiopathic Hypertrophic Cranial Pachymeningitis Presenting as Recurrent Multiple Cranial Nerve Palsies: Case Report and Review of Literature

Recurrent ophthalmoplegia poses a diagnostic challenge. A less commonly reported etiology is Idiopathic hypertrophic cranial pachymeningitis (IHCP), characterized by thickening of the dura mater and widespread CNS dysfunction depending of the location. The diagnosis is one of exclusion. Patients have a recurrent course and need immunosuppression. The authors’ report here in diagnostic and management dilemma in a patient with IHCP

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.

Immunoglobulin G4-Related Hypertrophic Pachymeningitis with Skull Involvement

Immunoglobulin G4 (IgG4)-related hypertrophic pachymeningitis, defined as focally or diffusely thickened dura mater and lymphoplasmacytic infiltration with increased IgG4 bearing plasma cells, is a rare disease. Moreover, cases involving bone are even rarer. In this report, the authors describe a case of IgG4-related hypertrophic pachymeningitis involving the skull in a 65-year-old man presenting with generalized tonic seizures. There is a 2.4 cm diameter extra-axial mass at the vertex of the left frontal convexity and thickened dura mater with contrast enhancement on magnetic resonance (MR) imaging. In addition, the skull adjacent to the mass was focally enhanced. He underwent surgical resection of the enhanced mass and skull. Histopathological findings revealed chronic inflammation with fibrosis, and idiopathic hypertrophic intracranial pachymeningitis was considered. However, eight months after surgery, partial seizures developed and brain MR imaging revealed a recurrence adjacent to the previous mass. We decided to perform additional immunohistochemical staining of the previous specimen, instead of a re-excision. Immunohistochemical staining showed markedly increased IgG4 (+) plasma cells. Consequently, IgG4-related hypertrophic meningitis was confirmed. Since then, steroids and immunosuppressant medications were started. Follow-up MR imaging at 3 months after medication initiation demonstrated complete remission. In conclusion, IgG4-related hypertrophic pachymeningitis should be considered in the differential diagnosis of hypertrophic cranial pachymeningitis.

Somatosensory reflex seizures in a patient with hypertrophic cranial pachymeningitis

Somatosensory-evoked reflex epilepsy is characterized by seizures in response to specific stimuli. It is highly uncommon for somatosensory-evoked focal seizures to be caused by movement or a change in posture. Reflex epilepsy induced by both somatosensory and proprioceptive stimulations has not been previously reported. In this study, we present a case of reflex epilepsy evoked by somatosensory and proprioceptive stimulation in a patient with hypertrophic cranial pachymeningitis. After comparing our patient with other cases of previously reported somatosensory-evoked reflex epilepsy, we determined that our patient had an unusual cause of reflex epilepsy.

Idiopathic hypertrophic cranial pachymeningitis: A rare cause of chronic headache and progressive cranial neuropathies: A case report

Idiopathic hypertrophic cranial pachymeningitis: A rare cause of chronic headache and progressive cranial neuropathies: A case report

Idiopathic hypertrophic cranial pachymeningitis: A rare cause of chronic headache and progressive cranial neuropathies: A case report

Idiopathic hypertrophic cranial pachymeningitis: A rare cause of chronic headache and progressive cranial neuropathies: A case report

HYPERTROPHIC CRANIAL PACHYMENINGITIS IN MPO-ANCA-RELATED VASCULITIS: A CASE REPORT AND LITERATURE REVIEW

A 75-year-old woman presented with rapidly progressive glomerulonephritis with positive results for anti-myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Corticosteroid therapy was successfully introduced. However, 7 months later, magnetic resonance imaging revealed marked swelling in the falx cerebri and high density regions were apparent on gallium scintigraphy, leading to diagnosis of hypertrophic cranial pachymeningitis (HCP). Symptoms improved with intensified corticosteroid therapy, but radiological examination 9 months later revealed right nasal sinus inflammation accompanied by osteolytic change. Granulomatosis with polyangiitis (Wegener's) was finally diagnosed. HCP is an important complication in MPO-ANCA-related vasculitis, and needs to be considered during the clinical course.