Download free adobe acrobat reader dc software for your windows, mac os and android devices to view, print, and comment on pdf documents. It can be caused by a variety of diseases, including sarcoidosis, tuberculosis and syphilis1. We present a 67yearold male with cranial and spinal pachymeningitis and mpoancaassociated vasculitis, which is considered as the cause of pachymeningitis of this patient. There are literally hundreds of things that can cause this including various syndromes, prenatal infections and lack of oxygen during birth just to name a few of the more common causes. Igg4related disease igg4rd is a recently described inflammatory condition known to cause tumefactive lesions at myriad anatomical locations. It is well known but rare especially in pediatric population. Hypertrophic pachymeningitis in rheumatoid arthritis after. We describe the case of an 80yearold man who presented with hypertrophic pachymeningitis hp together with arthritis as the first manifestation of rp. Hypertrophic pachymeningitis pdf pdf pro downloader. For more information on how to use laverne, please read the how to guide. A woman aged 28 years presented with a 1year history of leftsided headache.
It is estimated that igg4rhp may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. This is the first report describing hypertrophic cranial pachymeningitis which developed in association with mixed connective tissue disease mctd. 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. 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 presents with headaches, cranial neuropathies and ataxia occurring alone or in combination. Hypertrophic pachymeningitis hp is a rare clinical entity characterized by diffuse or localized fibrous thickening of the dura mater. 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. Intrathecal administration of rituximaban anticd20 monoclonal antibodyis emerging as a promising therapeutic strategy for b cell lymphomas of the central nervous system cns.
This is composed predominantly of lymphocytes lacking atypical features as well as plasma cell. Similar results from magnetic resonance imaging mri for patients with. Igg4related pachymeningitis is increasingly being recognized as the etiology of hypertrophic pachymeningitis, an entity which until recently was thought to be idiopathic. Aspecific headache during years as the only symptom of. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis, and lyme disease, and autoimmune diseases.
Pdf rituximab treatment for idiopathic hypertrophic. She is headachefree and has not had recurrence of cranial neuropathies on the. Case report pachymeningitis as a manifestation of anca. Intrathecal rituximab for igg4related hypertrophic. Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. Hypertrophic pachymeningitis and tracheobronchial stenosis. There were no giant cells, caseation necrosis, or epitheloid granuloma. Idiopathic hypertrophic cranial pachymeningitis in.
The headache was continuous and interfering with activities of daily living. Hypertrophic pachymeningitis is a rare form of diffuse inflammatory disease that causes thickening of the dura mater. The icd10cm alphabetical index is designed to allow medical coders to look up various medical terms and connect them with the appropriate icd codes. Intraventricular cytarabine in a case of idiopathic. We report a case of pachymeningitis which was manifested primarily by. Ossifying pachymeningitis bioinformatics tool laverne is a handy bioinformatics tool to help facilitate scientific exploration of related genes, diseases and pathways based on cocitations. Hypertrophic pachymeningitis is an uncommon disorder characterized by thickening and fibrosis of the duramater. Pachymeningitis medical definition merriamwebster medical. A diagnosis of granulomatosis with polyangiitis gpa, complicated by hypertrophic cranial pachymeningitis, was established.
Idiopathic cranial hypertrophic pachymeningitis responsive. Autoimmune, inflammatory, and neoplastic testing including csf and serum immunoglobulin g4 levels were. 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. Hypertrophic pachymeningitis karthik s n, bhanu k, velayutham s. Igg4related hypertrophic pachymeningitis is one of the many manifestations of igg4related disease which represents a fibroinflammatory condition that can affect any organ. 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 cranial pachymenlngitis involving dural sinuses. Paraffin sections show dense infiltration of dura by a mixed cellular infiltrate. Hypertrophic pachymeningitis hp is a rare disorder of diverse etiology. 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 hp is a rare, chronic inflammatory condition of the dura.
Optic neuropathy in a case of recurrent idiopathic. Longterm outcome of idiopathic hypertrophic thoracic. Idiopathic hypertrophic pachymeningitis successfully. Tuberculous hypertrophic pachymeningitis presenting as visual blurring and headaches rukhsana tariq, rashid ahmed advanced radiology clinic, off m a jinnah road, karachi.
It is well known but rare especially in pediatric population disease of differing origins. A 28 year old man with craniospinal ihcp and prominent lymphocytic meningitis is reported. Bilateral vision loss secondary to pachymeningitis in a. In both patients, an extensive extramedullary mass of low t2 signal with peripheral contrast enhancement was compressing the spinal cord on mr imaging. Pachymeningitis article about pachymeningitis by the free. Idiopathic hypertrophic pachymeningitis presenting with. Idiopathic hypertrophic cranial pachymeningitis sciencedirect. Pachymeningitis is an inflammatory process of the duramater, which may lead to fibrous thickening. There are 0 terms under the parent term pachymeningitis in the icd10cm alphabetical index. Hypertrophic tumefactive pachymeningitis presenting as.
Aug 11, 2012 we live in tennessee and my husband has been dia, with a very rare disease called pachy meningitis he is being treated in nash. Pdfreader, pdfviewer kostenlos adobe acrobat reader dc. Introduction hypertrophic pachymeningitis hp refers to inflammation leading to a localized or diffuse thickening of the cranial or spinal cord dura mater. Hypertrophic pachymeningitis hp is an inflammatory con dition in which the dura. Hypertrophic pachymeningitis hp denotes inflammation and thickening of the 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 mass effect, nerve. Igg4 igg4related disease pachymeningitis rituximab intrathecal. In some cases, even after extensive investigation, no specific cause is found and the process is called idiopathic intracranial pachymeningitis2. The condition is labelled as idiopathic hypertrophic pachymeningitis in the absence of any definite inciting factor.
Mar 17, 20 idiopathic hypertrophic pachymeningitis first description in 1869 by charcot and jovroy association with neurosyhpillis or tuberculosis navziger in 1949 described first case of idiopathic hypertrophic cranial pachymeningitis ihcp clinical symptoms results with compression of anatomical structures by thickened meninges headache 88% cranial. Cyclophosphamide was contraindicated due to high risk of. Autoimmune, inflammatory, and neoplastic testing including csf and serum immunoglobulin g4. Distinguishing magnetic resonance imaging features between. Here we are presenting a case of a young female presented with multiple. It is a rare inflammatory fibrosing disease of the dura of unidentified etiology and. T1 idiopathic cranial hypertrophic pachymeningitis responsive to antituberculous therapy. Often, the definite diagnosis is made immediately with biopsy and the involved dura is removed surgically. Igg4related intracranial hypertrophic pachymeningitis. Download fulltext pdf idiopathic hypertrophic pachymeningitis manifesting with frontal brain edema article pdf available in revista neurociencias 193.
Pdf hypertrophic pachymeningitis in sjogrens syndrome. Recently, there has been an increasing number of hcp reported, probably because of improvements in imaging studies. Hypertrophic cranial pachymeningitis is a rare, idiopathic form of granulomatous pachymeningitis. We report here a rare case of igg4related disease with intracranial hypertrophic pachymeningitis and irregular tracheobronchial stenosis. In contrast, frequencies were lower with idiopathic pachymeningitis than. A proportion of idiopathic hypertrophic pachymeningitis cases may involve a component of meningeal igg4rd. Hypertrophic pachymeningitis as an early manifestation of. A 38yearold female patient with bilateral papilledema who presented with. Idiopathic hypertrophic cranial pachymeningitis is a rare inflammatory disease with diffused involvement of the dura. Hp hypertrophic pachymeningitis, hpf highpower field, igg4rd igg4related disease, mri magnetic resonance imaging, wbc white blood cell.
Although the firstline therapy for hp is steroids, many hp cases are refractory to steroids or recur when the steroids are tapered. Case report tuberculous hypertrophic pachymeningitis. May 25, 2017 definition from wiktionary, the free dictionary. The latter was associated with blindness, multiple cranial neuropathies, pseudotumor cerebri, and seizures. 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.
Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis, and lyme disease, and autoimmune diseases rheumatoid. All three patients were recurrencefree of neurologic symptoms and exhibited prominent decreases in the dural thickness after rtx treatment. Hypertrophic pachymeningitis is a rare disorder of diverse etiology. Rituximab treatment for idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis mimicking hemicrania. If no etiology is identified it becomes idiopathic pachymeningitis, a diagnosis of exclusion. However, the longterm evolution of this form of pachymeningitis is unclear. Two patients with a chronic progressive myelopathy were successfully surgically treated and idiopathic hypertrophic spinal pachymeningitis ihsp was found on histology.
Relapsefree survival in antineutrophil cytoplasmic antibody. Hypertrophic pachymeningitis is a condition where there is localized inflammatory thickening of the dura. This report describes three cases of hypertrophic cranial pachymeningitis and discusses the clinical, radiographic, and pathological findings in these and other reported cases. Infectious etiologies include tuberculosis, syphilis, and lyme disease. Idiopathic hypertrophic pachymeningitis first description in 1869 by charcot and jovroy association with neurosyhpillis or tuberculosis navziger in 1949 described first case of idiopathic hypertrophic cranial pachymeningitis ihcp clinical symptoms results with compression of anatomical structures by thickened meninges headache 88% cranial.
Hypertrophic cranial pachymeningitis and granulomatosis. Herein are described 14 patients with idiopathic hypertrophic pachymeningitis. Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. Idiopathic hypertrophic spinal pachymeningitis hsp is one such progressive. Hypertrophic pachymeningitis with optic neuropathy. The primary idiopathic form is diagnosed after excluding other possible etiologies. Both igg4rd and idiopathic hp share similar demographics, histopathology, and natural history. Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory process of unknown origin that can cause neurological deficits owing to thickening of the dura.
Wasilewski and samkoff,1 immunoglobulin g4 igg4related disease rd is an increasingly recognized cause of hypertrophic pachymeningitis hp and likely accounts for a substantial subset of cases previously thought to be idiopathic. A 56yearold man with a twoyear history of mctdgradually developed symptoms of headache and blurred vision. Hypertrophic pachymeningitis with optic neuropathy heralding systemic vasculitis volume 44 issue 1 adrian budhram, manal y. Hipertrophic cranial pachymeningitis hcp is a rare clinical entity that is characterized by difuse or localized chronic inflammation and hypertrophy of the dura mater 1, 2. 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. Hypertrophic pachymeningitis hp is an inflammatory condition in which the. We describe a 34yearold man with a 17year history of multifocal fibrosclerosis resulting in unique neurologic complications. After revision of other 32 reported cases, our study. 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.
On clinical evaluation, she had leftsided sensorineural deafness. Hypertrophic cranial pachymeningitis associated with mixed. On imaging, it presents as a localized, multiple, or diffuse enhancing dural thickening commonly forming masslike lesions. The patient has been headache free and off of prednisone. Hypertrophic pachymeningitis radiology reference article. Pachymeningitis in mixed connective tissue disease. It presents like tumours of the craniocervical junction, which have an insidious onset and cause symptoms of cord and spinal nerve compression when these progress to occupy the subarachnoid space. Pdf idiopathic hypertrophic pachymeningitis manifesting. Patients with this condition commonly present with cranial neuropathy accompanied by localized headache. Cases in which the pachymeningitis has no known etiology are termed idiopathic hp. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. Pachymeningitis definition of pachymeningitis by the free. Secondary could be due to granulomatous, paraneoplastic and autoimmune etiologies.
She developed leftsided sixth nerve palsy and facial numbness and was referred to us. Mri showed asymmetric, bilateral smooth pachymeningeal enhancement with involvement of the optic canals figure 1. Article information, pdf download for idiopathic hypertrophic pachymeningitis. Hypertrophic pachymeningitis hp is a rare chronic inflammatory disorder characterized by marked. The igg4iggpositive plasma cell ratio was estimated at approximately 32. Few series of the idiopathic variety have been described, particularly with respect to mri correlation to clinical outcome and treatment. Idiopathic hypertrophic pachymeningitis is a rare form of granulomatous meningitis. This imaging appearance in patients with chronic progressive.
Igg4related hypertrophic pachymeningitis radiology. Pachymeningitis in granulomatosis with polyangiitis. Idiopathic hypertrophic pachymeningitis is a chronic, progressive, diffuse, inflammatory fibrosis of the dura mater 15, 22. Episcleritis, orbital pseudotumor, and sclerosing cholangitis accompanied a florid intracranial inflammatory pachymeningitis. Pachymeningitis is a rare cause of headache characterized by dura mater thickening with various origins. The precise diagnosis in pachymeningitis often remains elusive despite these thorough investigations. The spinal form is further uncommon and presents as a chronic progressive. Idiopathic hypertrophic pachymeningitis successfully treated. Immunoglobulin g4 igg4related disease is a systemic syndrome, characterized by sclerosing. We present a case similar to previously described cases of idiopathic hypertrophic cranial pachymeningitis. Consequently, extensive preoperative imaging studies usually are not available. Multifocal fibrosclerosis with hypertrophic intracranial.
Pachymeningitis definition of pachymeningitis by medical. Importance igg4related hypertrophic pachymeningitis igg4rhp is an increasingly recognized manifestation of igg4related disease, a fibroinflammatory condition that can affect virtually any organ. We present a patient with severe bilateral vision loss found to have thickening of the dura mater on mri, and subsequently diagnosed with igg4rd after dural biopsy. Idiopathic hypertrophic chronic pachymeningitis ihcp is characterised by inflammatory fibrotic thickening of the dura mater. Characteristics, outcome and treatments with cranial pachymeningitis. Hypertrophic pachymeningitis is an extremely rare fibrosing inflammatory process involving the dura mater.
Idiopathic hypertrophic spinal pachymeningitis ihsp is a rare inflammatory disorder that leads to spinal cord compression. Idiopathic hypertrophic pachymeningitis ihp is a rare disorder causing inflammation and thickening of the outer layer dura of the brain andor spinal cord. Meningeal involvement, with persistent and severe headache as main manifestation and abnormal thickening and enhancement of the dural mater on postcontrast magnetic resonance imaging, is extremely rare. We currently lack international standards for the diagnosis of. Idiopathic hypertrophic pachymeningitis request pdf. Regarding cranial pachymeningitis due to any aetiology, clinical manifestations vary depending on the location of the pathology, and the affected patients commonly present with manifestations of increased intracranial pressure, such as headache, or progressive neurologic deficits arising due to compression of neural structures by the thickened dura mater such as cranial neuropathies including.
Hypertrophic cranial pachymeningitis and skull base. Pachymeningitis is most often found in infants and in middleaged men. Among the causes of hp, vasculitis and autoimmune disease should be considered. Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. A variety of inflammatory and infectious conditions can result in this condition. It is a fibrosing inflammatory process that involves the dura mater. Dural biopsy is essential to exclude secondary causes of pachymeningitis. 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 can resemble other disorders associated with spinal compression. Idiopathic cranial hypertrophic pachymeningitis ichp consists of meningeal thickening due to chronic inflammation for which no cause such as infection, specific granulomatous disease, or malignancy is demonstrable. Adobe acrobat reader dcdownload kostenloser pdfviewer fur.
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