Hypertrophic pachymeningitis in rheumatoid arthritis after. Idiopathic hypertrophic pachymeningitis ihp is a rare disorder causing inflammation and thickening of the outer layer dura of the brain andor spinal cord. Although it is reported primarily in adults, there is one previous report describing the condition in a child. This imaging appearance in patients with chronic progressive. In both patients, an extensive extramedullary mass of low t2 signal with peripheral contrast enhancement was compressing the spinal cord on mr imaging.
Igg4related disease and hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis manifesting with. In one case, mr depicted dural involvement as a very. Episcleritis, orbital pseudotumor, and sclerosing cholangitis accompanied a florid intracranial inflammatory pachymeningitis.
We report a case of pachymeningitis which was manifested primarily by. It is estimated that igg4rhp may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. May 31, 2014 hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Antinmdar encephalitis with simultaneous hypertrophic. Hypertrophic pachymeningitis is an uncommon condition presenting as chronic fibrous inflammation of the dural meninx, which was first described by charcot and joffroy in 1869 as a cryptogenic thickening of the dura mater. It was first described by charcot and later by naffziger and stern.
Followup mr imaging after initiation of systemic treatment for hsp, consisting of 40 mg of prednisone twice daily and 20 mg of subcutaneous methotrexate weekly, showed a decrease in lesion size fig 3. Although the firstline therapy for hp is steroids, many hp cases are refractory to steroids or recur when the steroids are tapered. Idiopathic hypertrophic pachymeningitis is a rare form of granulomatous meningitis. We describe a 34yearold man with a 17year history of multifocal fibrosclerosis resulting in unique neurologic complications. Few series of the idiopathic variety have been described, particularly with respect to mri correlation to clinical outcome and treatment. Pachymeningitis is a rare illness which can be shown by magnetic resonance imaging mri to be a thickening of the intracranial dura mater, when associated with an infectious, malignant, or rheumatic systematic disease.
Sir, we report a rare case of compressive optic neuropathy secondary to hypertrophic cranial pachymeningitis. If the inline pdf is not rendering correctly, you can download the pdf file here. On the basis of these findings, the patient was diagnosed with idiopathic hypertrophic spinal pachymeningitis hsp. Igg4related intracranial hypertrophic pachymeningitis. Patients with this condition commonly present with cranial neuropathy accompanied by localized headache. Granulomatosis with polyangiitis gpa is a vasculitic disease with an infrequent involvement of the central nervous system. Pachymeningitis in mixed connective tissue disease. Clinically, hp can present as debilitating headaches and cranial nerve defects but in other cases may be completely asymptomatic. Igg4related hypertrophic pachymeningitis radiology.
Here we are presenting a case of a young female presented with multiple. Case report tuberculous hypertrophic pachymeningitis. Recently, the possibility that igg4related sclerosing disease may underlie some cases of intracranial hypertrophic pachymeningitis has been suggested. It can cause a localized or diffuse thickening of the dura mater with compression of. Ct in all cases and mr imaging in two cases detected isolated thickened dura mater. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis, and lyme disease, and autoimmune diseases rheumatoid. Pachymeningitis in mixed connective tissue disease annals. We herein describe a rare case of a 68yearold man with antinmdar encephalitis presenting with concomitant hypertrophic pachymeningitis. Hypertrophic pachymeningitis radiology reference article. Hypertrophic pachymeningitis is a rare clinical disorder involving localized or diffuse thickening of the dura mater.
The confusion regarding etiology and the inclusion in the literature of conditions which are not primarily inflammatory. Often, the definite diagnosis is made immediately with biopsy and the involved dura is removed surgically. Idiopathic hypertrophic cranial pachymeningitis is a rare inflammatory disease with diffused involvement of the dura. Idiopathic hypertrophic pachymeningitis genetic and rare. Hypertrophic pachymeningitis hp is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although many associated diseases have been suggested, the etiology of ihsp is not well understood. Hypertrophic pachymeningitissyndrome of inappropriate antidiuretic hormone secretionhyponatremia. Pdf idiopathic hypertrophic pachymeningitis is a rare form of granulomatous meningitis. Hypertrophic pachymeningitis hp is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral andor spinal dura mater. Early reports were in relationship to tuberculosis or syphilis. A diagnosis of granulomatosis with polyangiitis gpa, complicated by hypertrophic cranial pachymeningitis, was established.
Clinical and radiographic responses were achieved with steroids. It is a fibrosing inflammatory process that involves the dura mater. A 68yearold woman was referred to our hospital complaining of visual disturbance. Consequently, extensive preoperative imaging studies usually are not available. Two patients with a chronic progressive myelopathy were successfully surgically treated and idiopathic hypertrophic spinal pachymeningitis ihsp was found on histology. The hypertrophic pachymeningitis associated with syphilis has been described by charcot and joffroy 2, gowers 3, and more recently blackwood and corsellis 4. The cause of hp was reported to be associated with some disorders such as infectious disease, connective tissue disease ctd and malignant tumor 1. Hypertrophic pachymeningitis is a condition where there is localized inflammatory thickening of the dura. Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting. Hypertrophic spinal pachymeningitis with special reference to appropriate surgical treatment.
Hypertrophic cranial pachymeningitis in countries endemic for. Hypertrophic pachymeningitis with optic neuropathy heralding systemic vasculitis volume 44 issue 1 adrian budhram, manal y. Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. The inflammation usually involves the cranial or spinal dura mater, with resultant neurologic deficits. Progressively increasing headaches were the usual symptoms, along with ataxia and various cranial nerve palsies. Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been associated with rheumatoid arthritis, syphilis, wegeners granulomatosis, tuberculosis, and cancer. Hypertrophic pachymeningitis is a rare disorder of diverse etiology. Hypertrophic pachymeningitis associated with cerebral spinal fluid. Idiopathic hypertrophic cranial pachymeningitis sciencedirect.
Carruthers and arezou khosroshahi and robert carruthers and shweta shinagare and anat stemmerrachamimov and vikram deshpande and john h stone, booktitle. Distinguishing magnetic resonance imaging features between. Hypertrophic cranial pachymeningitis and granulomatosis. A variety of inflammatory and infectious conditions can result in this condition. The condition is labelled as idiopathic hypertrophic pachymeningitis in the absence of any definite inciting factor. We live in tennessee and my husband has been dia, with a very rare disease called pachy meningitis he is being treated in nash. Pachymeningitis is most often found in infants and in middleaged men. Patients commonly present with longstanding headache andor progressive neurologic deficits, multiple cranial nerve palsies, or blindness.
Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. High doses of intravenous methylprednisolone were administrated during his hospitalization. The cause of hp was reported to be associated with some disorders such as infectious disease, connective tissue disease ctd and malignant tumor1. Hypertrophic pachymeningitis and the syndrome of inappropriate. Case report with 7 years of imaging followup yu chang lee, yao chung chueng, shin wei hsu, and chun chung lui summary. Hypertrophic pachymeningitis and cerebral infarction resulting from. Longterm outcome of idiopathic hypertrophic thoracic. Propionibacterium acnes associated chronic hypertrophic. Article information, pdf download for hypertrophic pachymeningitis associated with cerebral spinal fluid hypovolemia as initial presentation of. The igg4iggpositive plasma cell ratio was estimated at approximately 32. The differential diagnosis of hp includes immunemediated conditions. Discussion hypertrophic pachymeningitis is an unusual rare inflammatory process involving the duramater, leptomeninges, and the tentorium. The latter was associated with blindness, multiple cranial neuropathies, pseudotumor cerebri, and seizures. Pdf hypertrophic pachymeningitis case report researchgate.
Compressive optic neuropathy in fungal hypertrophic. Outbreaks of the disease are diagnosed mainly in the springautumn period, which may be due to sudden temperature fluctuations, epidemics of infectious diseases, a decrease in immune protection in the population and seasonal vitamin deficiency. Dec 19, 2007 idiopathic hypertrophic cranial pachymeningitis ihcp is a rare clinical entity, characterized by a chronic inflammation causing thickening of the dura. Spinal idiopathic hypertrophic pachymeningitis ihp is a rare, chronic, nonspecific, granulomatous inflammatory disorder of the dura with unknown etiology. Tuberculous hypertrophic pachymeningitis involving cranio cervical region is rarely reported. The concept of hypertrophic pachymeningitis was introduced in 1869 by charcot et al. Keywords pachymeningitis, pediatric, antitubercular. It could be idiopathic or due to variety of inflammatory and infectious conditions. Idiopathic hypertrophic pachymeningitis in a child with. Herein are described 14 patients with idiopathic hypertrophic pachymeningitis.
Recently, some reports showed that some patients with hypertrophic pachymeningitis. We treated a patient with idiopathic cranial hypertrophic pachymeningitis and elevated serum titer of perinuclear anti. Idiopathic hypertrophic spinal pachymeningitis ihsp is a rare inflammatory disorder that leads to spinal cord compression. Dural biopsy revealed chronic lymphohistiocytic pachymeningitis without granulomatous inflammation, consistent with idiopathic hypertrophic pachymeningitis ihp. Hypertrophic pachymeningitis hp is a rare, chronic and inflammatory disorder. Pdf idiopathic hypertrophic pachymeningitis manifesting. Idiopathic hypertrophic cranial pachymeningitis treated by. Idiopathic hypertrophic pachymeningitis is a chronic, progressive, diffuse, inflammatory fibrosis of the dura mater 15, 22. Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis. In the present case the pathologic process not only involved the larger portion of the cerebral dura and its falx, but extended to. Pdf hypertrophic pachymeningitis as an important neurological. On imaging, it presents as a localized, multiple, or diffuse enhancing dural thickening commonly forming masslike lesions.
Distinguishing magnetic resonance imaging features between idiopathic hypertrophic pachymeningitis and secondary hypertrophic pachymeningitis. Igg4related pachymeningitis is increasingly being recognized as the etiology of hypertrophic pachymeningitis, an entity which until recently was thought to be idiopathic. Hypertrophic cranial pachymeningitis in countries endemic. Hp is characterized by thickening of the cranial dura mater. Hypertrophic pachymeningitis hp is an inflammatory condition in which the dura mater of the cranium or spine becomes thickened, leading to symptoms that result from. Pdf hypertrophic pachymeningitis in sjogrens syndrome. Hypertrophic pachymeningitis is a unique clinical entity characterized by fibrosis and thickening of dura mater resulting in neurological dysfunction. Multiple pathologic entities can produce pachymeningeal thickening. To our knowledge, there are few reports of immunoglobulin g4 igg4related hypertrophic pachymeningitis. Hypertrophic pachymeningitis hp is a rare disorder that involves localized or diffuse thickening of the dura mater. Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. A nationwide survey of hypertrophic pachymeningitis in japan. Hypertrophic pachymeningitis with optic neuropathy.
Mar 30, 2017 idiopathic hypertrophic pachymeningitis ihp is a rare disorder causing inflammation and thickening of the outer layer dura of the brain andor spinal cord. Hypertrophic pachymeningitis and cerebral infarction resulting from ancaassociated vasculitis. Importance igg4related hypertrophic pachymeningitis igg4rhp is an increasingly recognized manifestation of igg4related disease, a fibroinflammatory condition that can affect virtually any organ. Idiopathic hypertrophic cranial pachymeningitis request pdf. Hypertrophic pachymeningitis presents as nodular or linear thickening with intense meningeal enhancement. This can lead, in rare cases, to hypertrophic pachymeningitis hp, which is characterized by inflammation and fibrosis that cause a thickening of dura mater. Treatment was commenced with three pulses of 1 g intravenous methylprednisolone, followed by 60 mg oral prednisolone on a slow weaning course, and four cycles of rituximab 375 mgm 2 week. Nov 29, 2016 autoimmune, inflammatory, and neoplastic testing including csf and serum immunoglobulin g4 levels were unremarkable.
Three patients with diffuse idiopathic cranial pachymeningitis with predominant involvement of the tentorium and falx are reported. Oct 31, 2011 idiopathic hypertrophic spinal pachymeningitis ihsp is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Cyclophosphamide was contraindicated due to high risk of. Clinical analysis of hypertrophic pachymeningitis acr. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. Hypertrophic pachymeningitis is a rare disorder in which intracranial dura mater thickens focally or diffusely. Abstract hypertrophic pachymeningitis hp is an inflammatory condition in which the dura mater of the cranium or spine becomes thickened, leading to symptoms that result from mass effect, nerve compression, or vascular compromise. Hypertrophic pachymeningitis as an important neurological. Chronic pachymeningitis involving the dura of the posterior fossa and upper cervical regions and simulating intracranial tumor is of such rare occurrence as to prompt this report. The exact mechanism underlying hp is still undefined. Hypertrophic pachymeningitis, cerebral or spinal, is a much rarer type of neurosyphilis than the vascular or leptomeningeal forms. Hypertrophic pachymeningitis is an uncommon disorder characterized by thickening and fibrosis of the duramater.
Igg4related hypertrophic pachymeningitis is one of the many manifestations of igg4related disease which represents a fibroinflammatory condition that can affect any organ. Most patients with hypertrophic pachymeningitis have chronic headaches with or without neurological manifestations including cranial neuropathies, pituitary dysfunction, cerebellar ataxia, seizures, myelopathy and neuroophthalmic complications that include. Hypertrophic pachymeningitis hp is a rare inflammatory disease that results in thickening of the dura mater. Adequate therapeutic management is still a matter of debate. The objective of this article is to characterize hypertrophic pachymeningitis hp. Hypertrophic cranial pachymeningitis and granulomatosis with. Idiopathic hypertrophic pachymeningitis is a rare entity, posing challenge to the radiologist for a confident.
New diagnostic techniques such as contrast mri allow one to identify inflammation of the dura mater in the course of gpa more frequently. Cranio cervical tuberculous hypertrophic pachymeningitis. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis. Hypertrophic pachymeningitis hp is a rare chronic inflammatory disorder characterized by marked. Hypertrophic pachymeningitis in a patient with anca negative. Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Pdf idiopathic hypertrophic pachymeningitis researchgate. Abstract abstract hypertrophic pachymeningitis hp is an inflammatory condition in which the dura mater of the cranium or spine becomes thickened, leading to symptoms that result from mass effect, nerve compression, or vascular compromise. Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been. We present a patient with an ihcp, nonresponsive to corticotherapy. Research paper a nationwide survey of hypertrophic pachymeningitis in japan tomomi yonekawa,1 hiroyuki murai,1 satoshi utsuki,2 takuya matsushita,1 katsuhisa masaki,1 noriko isobe,1 ryo yamasaki,3 mari yoshida,4 susumu kusunoki,5 kiyomi sakata,6 kiyotaka fujii,2. Idiopathic hypertrophic pachymeningitis is a rare but increasingly recognized disorder characterized by diffuse thickening of the dura mater of unknown etiology. Fungal aetiology is rarely reported in the literature and can be life. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis, and lyme disease, and autoimmune diseases.
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