Idiopathic Hypertrophic Cranial Pachymeningitis Research Articles

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.

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

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

Idiopathic hypertrophic cranial pachymeningitis presenting as acute painless visual loss

Idiopathic hypertrophic cranial pachymeningitis presenting as acute painless visual loss

Usefulness of L-[Methyl-<sup>11</sup>C]Methionine Positron Emission Tomography in the Treatment of Idiopathic Hypertrophic Cranial Pachymeningitis

A 53-year-old man suffered from pulsating headache for 2 months. Magnetic resonance imaging with gadolinium revealed a linear or nodular mass along the left fronto-parietal convexity. Positron emission tomography (PET) with L-[methyl-11C]methionine (11C-MET) demonstrated increased uptake in the enhanced lesion. Biopsy, obtained by craniotomy, demonstrated granulation with lymphocyte and plasma cell infiltration, suggesting inflammatory changes, and a diagnosis of idiopathic hypertrophic cranial pachymeningitis (IHCP) was made. Steroid therapy resulted in improvement of the clinical symptoms and shrinkage of the enhanced lesion in a week. Follow-up 11C-MET PET study, after 18 months of steroid therapy, demonstrated significantly decreased uptake in the lesion, so the steroid therapy was discontinued. Neither clinical nor radiological recurrence was observed one year after discontinuation of the steroid therapy. This case of IHCP with increased 11C-MET uptake, which then decreased after steroid therapy suggests that 11C-MET PET is a useful monitoring modality for therapeutic efficacy against IHCP, and can indicate the appropriate timing of therapy discontinuation.

Long-term lack of progression after initial treatment of idiopathic hypertrophic pachymeningitis

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare inflammatory disease which is sometimes difficult to diagnose and can lead to misinterpretations of the clinical and imaging findings. The main clinical manifestations are headache, ataxia and cranial nerve palsy. In most of the reported patients continuous medication is needed to avoid disease recurrence. We present a female patient with an 8-year follow-up, no clinical regression and no need for any further medical treatment. Even though most patients with IHCP experience recurrence after diagnosis and initial treatment there were no clinical or imaging signs of relapse in our patient. Our patient is still not under any medical or surgical treatment due to the lack of any significant symptoms.

Short‐Lasting Unilateral Neuralgiform Headache With Autonomic Symptoms Syndrome as the Initial Manifestation of Idiopathic Hypertrophic Cranial Pachymeningitis

This is the first report of 2 patients presenting with short-lasting unilateral neuralgiform headache with autonomic symptoms as the initial manifestation of idiopathic hypertrophic cranial pachymeningitis. They both had acute retro-orbital pain ipsilateral to the dural thickening on magnetic resonance imaging of brain, and one had transient miosis as an additional parasympathetic feature. Short-lasting unilateral neuralgiform headache with autonomic symptoms syndrome may be associated with secondary central nervous system pathology, and neuroimaging should be considered in all patients with trigeminal autonomic cephalalgia.

Idiopathic hypertrophic cranial pachymeningitis: Three biopsy-proven cases including one case with abdominal pseudotumor and review of the literature

Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. It presents with headaches, cranial neuropathies and ataxia occurring alone or in combination. Dural biopsy is essential to exclude secondary causes of pachymeningitis. There is paucity of data on biopsied cases of HP. We report three biopsy-proven cases of idiopathic hypertrophic cranial pachymeningitis. All our patients had headaches and multiple cranial neuropathies; ataxia was seen in one patient. One patient had recurrent anterior and posterior cranial neuropathies, while one each had recurrent anterior and posterior cranial neuropathies. Two patients had profound irreversible mono-ocular visual loss. All of them showed prominent pachymeningeal thickening on imaging. Infarcts were seen in one patient, which have rarely been documented. All patients showed biopsy evidence of meningeal thickening and nonspecific chronic inflammation of the dura. The disease may have a remitting and relapsing course, and usually responds to steroids. Clinical improvement was excellent in two patients and modest in one on steroid therapy. All our patients required azathioprine during the course of therapy. Early institution and long-term maintenance of steroid therapy prevents neurologic sequelae. Occurrence of abdominal inflammatory pseudotumor in a patient of HP possibly as part of multifocal fibrosclerosis has not been described earlier.

Primary Diffuse Meningeal Melanomatosis

Primary diffuse meningeal melanomatosis can clinically mimic a wide variety of other conditions, including lymphoma, leukemia, neurosarcoidosis, metastatic carcinoma, acute disseminated encephalomyelitis, subacute meningitis, viral encephalitis, and idiopathic hypertrophic cranial pachymeningitis. We report on a young patient with primary diffuse meningeal melanomatosis who presented with papilledema, flaccid paraparesis, and cognitive impairment. The importance of imaging of the whole central nervous system, cerebrospinal fluid analysis, and pathohistological examination is emphasized in making the appropriate diagnosis.

Idiopathic Hypertrophic Cranial Pachymeningitis Misdiagnosed as Acute Subtentorial Hematoma

A case of idiopathic hypertrophic cranial pachymeningitis (IHCP) misdiagnosed as an acute subdural hematoma is reported. A 37-year-old male patient presented with headache following head trauma 2 weeks earlier. Computerized tomography showed a diffuse high-density lesion along the left tentorium and falx cerebri. Initial chest X-rays revealed a small mass in the right upper lobe with right lower pleural thickening, which suggested lung cancer, such as an adenoma or mediastinal metastasis. During conservative treatment under the diagnosis of a subdural hematoma, left cranial nerve palsies were developed (3rd and 6th), followed by scleritis and uveitis involving both eyes. Magnetic resonance imaging (MRI) revealed an unusual tentorium-falx enhancement on gadolinium-enhanced T1-weighted images. Non-specific chronic inflammation of the pachymeninges was noticed on histopathologic examination following an open biopsy. Systemic steroid treatment was initiated, resulting in dramatic improvement of symptoms. A follow-up brain MRI showed total resolution of the lesion 2 months after steroid treatment. IHCP should be included in the differential diagnosis of subtentorial-enhancing lesions.

Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: two patients with long-term follow up

Idiopathic hypertrophic cranial pachymeningitis (IHPM) is a clinicopathological entity characterized by thickening and fibrosis of the dura mater with resultant clinical symptoms. It is generally steroid responsive and has a tendency to remit and relapse. We present here two patients with IHPM with associated dural sinus occlusion and describe their clinicoradiological features and long-term course and outcome.

Idiopathic Hypertrophic Cranial Pachymeningitis Presenting With Headache

Idiopathic hypertrophic cranial pachymeningitis is a rare inflammatory disease, usually involving the dura mater of skull base, tentorium, and falx. Chronic headache is the most common clinical manifestations. We report a case of idiopathic hypertrophic cranial pachymeningitis that was, initially, misdiagnosed as acute subdural hematoma along the tentorium cerebelli and posterior falx on the brain precontrast CT scan of a patient with severe headache. Correct diagnosis of pachymeningitis was made only after brain magnetic resonance imaging and meningeal biopsy.