Idiopathic hypertrophic cranial pachymeningitis ihcp is a chronic and progressive inflammatory disease that usually results in a diffuse thickening of the dura mater. Clinical image and pathology of hypertrophic cranial. Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. Idiopathic hypertrophic pachymeningitis is a rare entity of chronic in. 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. Hypertrophic pachymeningitis and tracheobronchial stenosis. This is a case series of six canine cases of presumptive or confirmed intracranial idiopathic hypertrophic pachymeningitis.
Idiopathic hypertrophic pachymeningitis request pdf. Although central nervous system manifestations seem. Idiopathic hypertrophic pachymeningitis successfully. It is a fibrosing inflammatory process that involves the dura mater. Hp is characterized by thickening of the cranial dura mater.
A 58yearold male developed left headache after exudative otitis media. Hypertrophic pachymeningitis in rheumatoid arthritis after. 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. 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. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis, and lyme disease, and.
It can cause a localized or diffuse thickening of the dura mater with compression of the spinal canal and possible myelopathic symptoms. Importance igg4related hypertrophic pachymeningitis igg4rhp is an increasingly recognized manifestation of igg4related disease, a fibroinflammatory condition that can affect virtually any organ. Idiopathic hypertrophic pachymeningitis ihp can resemble other. Idiopathic hypertrophic pachymeningitis genetic and rare. A novel model for treatment of hypertrophic pachymeningitis ncbi. Idiopathic hypertrophic pachymeningitis is a chronic, progressive, diffuse, inflammatory fibrosis of the dura mater 15, 22. This entity should be considered when evaluating mri scans obtained in the setting of sudden sensorineural hearing loss. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. Idiopathic hypertrophic pachymeningitis ihp is a very infrequent disorder that causes a localized or diffuse thickening of the dura mater. Myelodysplastic syndromes mds are hematopoietic malignancies characterized by pancytopenia in the peripheral blood and is often complicated by autoimmune diseases 24. Pachymeningitis in mixed connective tissue disease. Idiopathic hypertrophic pachymeningitis with intracranial. Hypertrophic pachymeningitis and tracheobronchial stenosis in igg4related disease.
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. Idiopathic hypertrophic spinal pachymeningitis ihsp is a rare inflammatory disorder that leads to spinal cord compression. Herein are described 14 patients with idiopathic hypertrophic pachymeningitis. Although the firstline therapy for hp is steroids, many hp cases are refractory to steroids or recur when the steroids are ta pered.
Combined hypertrophic pachymeningitis and cerebral venous. Cranial and lumbosacral hypertrophic pachymeningitis associated. In cranial idiopathic hypertrophic pachymeningitis, the maletofemale ratio is 3. Idiopathic hypertrophic pachymeningitis is a diagnosis of exclusion. In both patients, an extensive extramedullary mass of low t2 signal with peripheral contrast enhancement was compressing the spinal cord on mr imaging. Hypertrophic pachymeningitis hp are secondarily caused by various diseases such as infections, malignant tumors, and autoimmune diseases 1. Idiopathic hypertrophic cranial pachymeningitis and dural. In patients with idiopathic hypertrophic pachymeningitis ihp presenting with tumor. Numerous pathological entities produce thickening of the pachymeninges, so that idopathic hypertrophic pachymeningitis is a diagnosis of exclusion. According to the site of involvement, it can be classified into cranial, spinal or craniospinal 1, 4. 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 ihp consists of an inflammatory process with fibrosis and consequent thickening of dura mater, sometimes with pseudotumoral lesions, without secondary cause. Spinal idiopathic hypertrophic pachymeningitis ihp is a rare, chronic, nonspecific, granulomatous inflammatory disorder of the dura with unknown etiology. We describe four patients with idiopathic hypertrophic pachymeningitis. Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening. Abstract hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis. Although the lesion when associated with syphilis most often involves the cervical and higher thoracic cord, lumbar involvement can also occur. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. Hypertrophic pachymeningitis hp is a chronic disease characterized by inflammatory hypertrophy and fibrosis of dura mater. Hypertrophic pachymeningitis hp is a rare inflammatory disorder presenting with thickening of the dura mater. Clinically, hp can present as debilitating headaches and cranial nerve defects but in. Hypertrophic pachymeningitis hp is a rare, chronic and inflammatory disorder.
Idiopathic hypertrophic pachymeningitis ihp can resemble other disorders associated with spinal compression. It often causes headaches and multiple cranial nerve palsies due to fibrous entrapment or ischemic damage. Idiopathic hypertrophic pachymeningitis ihpm is a rare pathological state, with still unclear aetiopathogenesis. Hypertrophic pachymeningitis is an uncommon disorder characterized by thickening and fibrosis of the duramater. 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. Ct and mr can help differentiate it from these other disorders.
A variety of inflammatory and infectious conditions can result in this condition. Idiopathic hypertrophic cranial pachymeningitis is a rare disease. It is a fibrosing inflammatory process which involves the dura mater, including the tentorium. Idiopathic hypertrophic pachymeningitis semantic scholar. 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. The main clinical features of ihp at presentation include headache, cranial nerve involvement, ataxia, transient ischemic attacks tia and seizures. 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. Hypertrophe kraniale pachymeningitis als seltene ursache. The hypertrophic pachymeningitis associated with syphilis has been described by charcot and joffroy 2, gowers 3, and more recently blackwood and corsellis 4. Longterm outcome of idiopathic hypertrophic thoracic. 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. Cranial pachymeningitis is a rarely reported disease that can resemble other disorders associated with tentorial thickening.
Hypertrophic pachymeningitis as the first manifestation of systemic. Only a few clinical cases were reported before the advent of ct. Pachymeningitis is most often found in infants and in middleaged men. Idiopathic hypertrophic pachymeningitis has been described in humans as a rare, chronic progressive non. Pdf idiopathic hypertrophic pachymeningitis manifesting. The concept of hypertrophic pachymeningitis was introduced in 1869 by charcot et al. Pdf rituximab treatment for idiopathic hypertrophic. 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. The prevailing treatment for this condition is highdose corticosteroids. Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. Successful treatment of hypertrophic pachymeningitis. Although many associated diseases have been suggested, the etiology of ihsp is not well understood. Numerous pathological entities produce thickening of the pachymeninges. Idiopathic hypertrophic pachymeningitis in six dogs.
We report a rare case of hypertrophic pachymeningitis hp and cerebral venous thrombosis associated with proteinase3antineutrophil cytoplasmic antibody pr3ancapositive granulomatosis with polyangiitis gpa. Idiopathic hypertrophic cranial pachymeningitis mimicking. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. Autoimmune, inflammatory, and neoplastic testing including csf and serum immunoglobulin g4 levels were. It is a fibrosing inflammatory process which involves the dura mater. We treated a patient with idiopathic cranial hypertrophic pachymeningitis and elevated serum titer of perinuclear anti. Two patients with a chronic progressive myelopathy were successfully surgically treated and idiopathic hypertrophic spinal pachymeningitis ihsp was found on histology. Neoplastic, infectious, and autoimmune causes must be ruled out. Hypertrophic pachymeningitis hp is an intractable neurologic disease. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. Few series of the idiopathic variety have been described, particularly with respect to mri correlation to. Hypertrophic spinal pachymeningitis with special reference to appropriate surgical treatment.
Hypertrophic pachymeningitis hp is a rare disease caused by autoimmunity in the meninx that causes. Idiopathic hypertrophic pachymeningitis in a child with. Hypertrophic pachymeningitis of the internal auditory. Mri showed asymmetric, bilateral smooth pachymeningeal enhancement with involvement of the optic canals figure 1. 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 igg4iggpositive plasma cell ratio was estimated at approximately 32. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The present report describes the case of a child with this disorder, the course of his. Case of granulomatosis with polyangiitis with pachymeningitis. Hcp is a nonspecific chronic inflammatory response in the dura martin et al.
Hypertrophic pachymeningitis hp denotes inflammation and thickening of the dura mater that can be idiopathic or secondary to a wide variety of conditions. 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. The cause of hp was reported to be associated with some disorders such as infectious disease, connective tissue disease ctd and malignant tumor1. Idiopathic hypertrophic pachymeningitis ihp is a rare disorder causing inflammation and thickening of the outer layer dura of the brain andor spinal cord. Clinical analysis of hypertrophic pachymeningitis acr. The condition is labelled as idiopathic hypertrophic pachymeningitis in the absence of any definite inciting factor. Final diagnosis idiopathic hypertrophic pachymeningitis. A novel model for treatment of hypertrophic pachymeningitis cui. Hypertrophic pachymeningitis, characterised by focal or diffuse thickening of the dura mater, is an uncommon condition. The condition is being reported more frequently owing to the use of cranial mri. It is estimated that igg4rhp may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic.
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