Hypertrophic pachymeningitis hp is a rare inflammatory disorder presenting with thickening of the dura mater. Clinical analysis of hypertrophic pachymeningitis acr. The present report describes the case of a child with this disorder, the course of his. Hypertrophic pachymeningitis and tracheobronchial stenosis. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis. Summary of background data hsp is a rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. The concept of hypertrophic pachymeningitis was introduced in 1869 by charcot et al.
Longterm outcome of idiopathic hypertrophic thoracic. It is estimated that igg4rhp may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. Hypertrophic spinal pachymeningitis with special reference to appropriate surgical treatment. Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. Hypertrophic pachymeningitis hp are secondarily caused by various diseases such as infections, malignant tumors, and autoimmune diseases 1. Only a few clinical cases were reported before the advent of ct. Since 1869 when charcot first reported hypertrophic pachymeningitis hypertrophic cranial pachymeningitis, hcp caplan, 1980, the amount of corresponding literature has reached over 200 articles, most of which are from europe and japan. Although the firstline therapy for hp is steroids, many hp cases are refractory to steroids or recur when the steroids are ta pered. Ct and mr can help differentiate it from these other disorders. The aim of this study was to elucidate the characteristics, pathogenesis and treatment strategy of hypertrophic pachymeningitis that is associated with myeloperoxidase antineutrophil cytoplasmic antibody anca. In both patients, an extensive extramedullary mass of low t2 signal with peripheral contrast enhancement was compressing the spinal cord on mr imaging. Combined hypertrophic pachymeningitis and cerebral venous. Few series of the idiopathic variety have been described, particularly with respect to mri correlation to. The main clinical features of ihp at presentation include headache, cranial nerve involvement, ataxia, transient ischemic attacks tia and seizures.
Neoplastic, infectious, and autoimmune causes must be ruled out. Although the lesion when associated with syphilis most often involves the cervical and higher thoracic cord, lumbar involvement can also occur. Hypertrophic pachymeningitis, characterised by focal or diffuse thickening of the dura mater, is an uncommon condition. In patients with idiopathic hypertrophic pachymeningitis ihp presenting with tumor. Hp is characterized by thickening of the cranial dura mater. Idiopathic hypertrophic pachymeningitis is a rare entity of chronic in. In cranial idiopathic hypertrophic pachymeningitis, the maletofemale ratio is 3.
Idiopathic hypertrophic cranial pachymeningitis and dural. It is a fibrosing inflammatory process which involves the dura mater. A 58yearold male developed left headache after exudative otitis media. Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis, and lyme disease, and. Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. Idiopathic hypertrophic pachymeningitis is a diagnosis of exclusion.
Pdf rituximab treatment for idiopathic hypertrophic. 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. Idiopathic hypertrophic pachymeningitis request pdf. Case of granulomatosis with polyangiitis with pachymeningitis. Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening. Idiopathic hypertrophic pachymeningitis successfully. A novel model for treatment of hypertrophic pachymeningitis ncbi. Idiopathic hypertrophic pachymeningitis with intracranial. 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. Pachymeningitis in mixed connective tissue disease. Hcp is a nonspecific chronic inflammatory response in the dura martin et al.
Numerous pathological entities produce thickening of the pachymeninges, so that idopathic hypertrophic pachymeningitis is a diagnosis of exclusion. Hypertrophic pachymeningitis hp is a chronic disease characterized by inflammatory hypertrophy and fibrosis of dura mater. Idiopathic hypertrophic cranial pachymeningitis ihpm is a clinicopathological entity characterized by thickening and fibrosis of the dura mater, with clinical presentation as headache, cranial neuropathies, cerebellar dysfunction and visual loss occurring either alone or in combination. A 39yearold woman presented with acute, painless left monocular vision loss in the context of 6 months of right peripheral facial weakness and prior right optic neuropathy. Idiopathic hypertrophic pachymeningitis genetic and rare. It is a fibrosing inflammatory process that involves the dura mater. It is a fibrosing inflammatory process which involves the dura mater, including the tentorium. Hypertrophic pachymeningitis and tracheobronchial stenosis in igg4related disease. 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. Idiopathic hypertrophic pachymeningitis successfully treated with intravenous cyclophosphamide chen zhuoyou 1, qian chuanzhong 1, ding xinsheng 2 1 department of neurology, changzhou second peoples hospital, nanjing medical university, changzhou, china 2 department of neurology, the first affiliated hospital with nanjing medical university, nanjing, jiangsu, china.
Idiopathic hypertrophic pachymeningitis ihp is a very infrequent disorder that causes a localized or diffuse thickening of the dura mater. According to the site of involvement, it can be classified into cranial, spinal or craniospinal 1, 4. Idiopathic hypertrophic pachymeningitis ihp can resemble other disorders associated with spinal compression. Hypertrophic pachymeningitis of the internal auditory. Hypertrophic pachymeningitis is an uncommon disorder characterized by thickening and fibrosis of the duramater. Herein are described 14 patients with idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis semantic scholar. Numerous pathological entities produce thickening of the pachymeninges. Pdf idiopathic hypertrophic pachymeningitis manifesting. Hypertrophic pachymeningitis hp is a rare, chronic and inflammatory disorder. Mri showed asymmetric, bilateral smooth pachymeningeal enhancement with involvement of the optic canals figure 1. 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 as the first manifestation of systemic.
The igg4iggpositive plasma cell ratio was estimated at approximately 32. The hypertrophic pachymeningitis associated with syphilis has been described by charcot and joffroy 2, gowers 3, and more recently blackwood and corsellis 4. The condition is being reported more frequently owing to the use of cranial mri. The condition is labelled as idiopathic hypertrophic pachymeningitis in the absence of any definite inciting factor. A cerebral mri scan showed initially and after 3 months clear thickening and enrichment of contrast dye in all meningeal structures pachymeningitis, as well as widened ventricles iiii, with periventricular capping in the form of a hydrocephalus fig 1. We describe four patients with idiopathic hypertrophic pachymeningitis. Abstract hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases.
The prevailing treatment for this condition is highdose corticosteroids. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. Importance igg4related hypertrophic pachymeningitis igg4rhp is an increasingly recognized manifestation of igg4related disease, a fibroinflammatory condition that can affect virtually any organ. We live in tennessee and my husband has been dia, with a very rare disease called pachy meningitis he is being treated in nash. Idiopathic hypertrophic pachymeningitis has been described in humans as a rare, chronic progressive non. Idiopathic hypertrophic pachymeningitis ihpm is a rare pathological state, with still unclear aetiopathogenesis. Two patients with a chronic progressive myelopathy were successfully surgically treated and idiopathic hypertrophic spinal pachymeningitis ihsp was found on histology. Idiopathic hypertrophic cranial pachymeningitis ihcp is a chronic and progressive inflammatory disease that usually results in a diffuse thickening of the dura mater. Idiopathic hypertrophic pachymeningitis in a child with.
These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis. Hypertrophe kraniale pachymeningitis als seltene ursache. Although central nervous system manifestations seem. The cause of hp was reported to be associated with some disorders such as infectious disease, connective tissue disease ctd and malignant tumor1.
Myelodysplastic syndromes mds are hematopoietic malignancies characterized by pancytopenia in the peripheral blood and is often complicated by autoimmune diseases 24. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. Hypertrophic pachymeningitis in rheumatoid arthritis after. Cranial pachymeningitis is a rarely reported disease that can resemble other disorders associated with tentorial thickening. Idiopathic hypertrophic pachymeningitis ihp can resemble other. Pachymeningitis is most often found in infants and in middleaged men. Final diagnosis idiopathic hypertrophic pachymeningitis. Hypertrophic pachymeningitis hp denotes inflammation and thickening of the dura mater that can be idiopathic or secondary to a wide variety of conditions. This is a case series of six canine cases of presumptive or confirmed intracranial idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis ihp consists of an inflammatory process with fibrosis and consequent thickening of dura mater, sometimes with pseudotumoral lesions, without secondary cause. This entity should be considered when evaluating mri scans obtained in the setting of sudden sensorineural hearing loss. Although many associated diseases have been suggested, the etiology of ihsp is not well understood. Idiopathic hypertrophic spinal pachymeningitis ihsp is a rare inflammatory disorder that leads to spinal cord compression.
We report a rare case of hypertrophic pachymeningitis hp and cerebral venous thrombosis associated with proteinase3antineutrophil cytoplasmic antibody pr3ancapositive granulomatosis with polyangiitis gpa. Hypertrophic pachymeningitis hp is a rare disease caused by autoimmunity in the meninx that causes. Spinal idiopathic hypertrophic pachymeningitis ihp is a rare, chronic, nonspecific, granulomatous inflammatory disorder of the dura with unknown etiology. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. 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. We treated a patient with idiopathic cranial hypertrophic pachymeningitis and elevated serum titer of perinuclear anti. Idiopathic hypertrophic cranial pachymeningitis mimicking. Cranial and lumbosacral hypertrophic pachymeningitis associated. Idiopathic hypertrophic pachymeningitis is a chronic, progressive, diffuse, inflammatory fibrosis of the dura mater 15, 22. Clinical image and pathology of hypertrophic cranial.
Clinically, hp can present as debilitating headaches and cranial nerve defects but in. Successful treatment of hypertrophic pachymeningitis. It often causes headaches and multiple cranial nerve palsies due to fibrous entrapment or ischemic damage. A variety of inflammatory and infectious conditions can result in this condition.
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