Resultant nerve encasement and ischemia produce multiple cranial neuropathies and headache. The cause of hp was reported to be associated with some disorders such as infectious disease, connective tissue disease ctd and malignant tumor 1. Idiopathic hypertrophic cranial pachymeningitis treated by. Pdf hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or. If the inline pdf is not rendering correctly, you can download the pdf file here.
Objective to investigate the clinical features,pathogenesis,cerebrospinal fluidcsf changes,radiological findings and treatments of hypertrophic pachymeningitis. A 52yearold man presented with refractory generalized tonicclonic seizure. 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. Patients commonly present with longstanding headache andor progressive neurologic deficits, multiple cranial nerve palsies, or blindness.
Prabhakar s, bhatia r, lal v, singh p department of neurology, postgraduate institute of medical education and research, chandigarh, 160 012, india. Full text full text is available as a scanned copy of the original print version. Final diagnosis idiopathic hypertrophic pachymeningitis upmc. We report a case of pachymeningitis which was manifested primarily by.
Hypertrophic pachymeningitis hp is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral andor spinal dura mater. A case of idiopathic hypertrophic pachymeningitis presenting with chronic headache and multiple cranial nerve palsies. Successful treatment of hypertrophic pachymeningitis. Igg4related hypertrophic pachymeningitis radiology. Aug 11, 2012 i am being worked up for pachymeningitis. Clinical image and pathology of hypertrophic cranial pachymeningitis.
Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been. The distinctive signs on brain mris involve strengthening the signal in the cerebral dura. Before ihp can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections syphilis, tuberculosis, and lyme disease, and autoimmune diseases rheumatoid. Hypertrophic pachymeningitis is a rare clinical disorder involving localized or diffuse thickening of the dura mater. New diagnostic techniques such as contrast mri allow one to identify inflammation of the dura mater in the course of gpa more frequently. Pachymeningitis is most often found in infants and in middleaged men. We present a case of a 63yearold woman with cranial variety of that disease. Medline was used to search relevant literatures written in english since 2004.
My neurologist is aggressively looking for the cause. Immunoglobulin g4related disease igg4rd is a group of distinct autoimmune disorders affecting nearly every organ system in the body. Clinically, hp can present as debilitating headaches and cranial nerve defects but in other cases may be completely asymptomatic. Get a printable copy pdf file of the complete article 100k, or click on a page image below to browse page by page. Home about us subject areas contacts about us subject areas contacts. Hypertrophic pachymeningitis is a chronic inflammatory disease, demonstrating local or diffuse thickening of the dura mater. Hypertrophic pachymeningitis, characterised by focal or diffuse thickening of the dura mater, is an uncommon condition. Idiopathic hypertrophic cranial pachymeningitis ihcp causes chronic progressive inflammation and thickening of the basal dura mater 3, 5, 10, 16, 22, 26. Hypertrophic pachymeningitis is a rare disorder of diverse etiology. It is estimated that igg4rhp may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. If no etiology is identified it becomes idiopathic pachymeningitis, a diagnosis of exclusion. Idiopathic hypertrophic cranial pachymeningitis journal of neurology. Nonobstructive idiopathic pachymeningitis cervicalis hypertrophica. We present a patient with severe bilateral vision loss found to have thickening of the dura mater on mri, and subsequently diagnosed.
It is well known but rare especially in pediatric population disease of differing origins. Masson c, henin d, mompoint d, marsault c, nahum h. Idiopathic hypertrophic pachymeningitis ihp consists of an inflammatory process with fibrosis and consequent thickening of dura mater, sometimes with pseudotumoral lesions, without secondary cause. Herein, we report the clinical and histopathological features of a rare case of igg4related intracranial hypertrophic pachymeningitis involving cranial hyperostosis. Hypertrophic pachymeningitis with optic neuropathy.
It can involve the cranial or the spinal dura or both. There are two ical entity characterized by diffuse or localized fibrous thick forms of the disease. The major clinical symptoms were characterized by headache, exophthalmos, and blindness in one eye. The data for differential diagnosis of the disease are given. Igg4related intracranial hypertrophic pachymeningitis with. Hypertrophic pachymeningitis hp is a rare, chronic and inflammatory disorder. The confusion regarding etiology and the inclusion in the literature of. Iodized oils as an aid to the diagnosis of lesions of the spinal cord and a contribution to the knowledge of adhesive. We say erroneously referred to because in most cases reported, the leptomeninges as well as the dura were adherent and involved in the pathological process. Hypertrophic pachymeningitis is a rare form of diffuse inflammatory disease that causes thickening of the dura mater. Although central nervous system involvement is quite rare, it may present as hypertrophic pachymeningitis more frequently affecting the brain than the spine. Pdf file of the complete article 902k, or click on a page image below to browse page by page.
Idiopathic hypertrophic cranial pachymeningitis ihcp is a rare clinical entity, characterized by a chronic inflammation causing thickening of the dura. Mailcap mutt pdf the other is the mailcap file, which specifies the external commands to use for you can view attachments as text, or view them using the mailcap viewer. Hypertrophic pachymeningitis with optic neuropathy heralding systemic vasculitis volume 44 issue 1 adrian budhram, manal y. Clinical image and pathology of hypertrophic cranial. Hp is characterized by thickening of the cranial dura mater. 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 19. Hypertrophic spinal pachymeningitis with special reference to appropriate surgical treatment. Igg4related disease and hypertrophic pachymeningitis. Igg4related intracranial hypertrophic pachymeningitis. Often, the definite diagnosis is made immediately with biopsy and the involved dura is removed surgically. Hypertrophic pachymeningitis in systemic sclerosis sine scleroderma hypertrophic pachymeningitis in systemic sclerosis sine scleroderma kumar, uma. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis. Alright, now in this part of the article, you will be able to access the free pdf download of imaging in spine surgery 1st edition pdf using our direct links mentioned at the end of this article.
Nonobstructive idiopathic pachymeningitis cervicalis. The exact mechanism underlying hp is still undefined. The hypertrophic pachymeningitis associated with syphilis has been described by charcot and joffroy 2, gowers 3, and more recently blackwood and corsellis 4. A case of idiopathic hypertrophic pachymeningitis presenting. Abstract hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. Hypertrophic pachymeningitis hp refers to inflammation leading to a localized or diffuse thickening of the cranial or spinal cord dura mater. Hypertrophic pachymeningitis as an important neurological. Discussion hypertrophic pachymeningitis is a rare disorder that causes localized or diffused thickening of the dura mater 5. Idiopathic hypertrophic cranial pachymeningitis is a rare inflammatory disease with diffused involvement of the dura. Hypertrophic pachymeningitis hp denotes inflammation and thickening of the dura mater that can be idiopathic or secondary to a wide variety of conditions. Hcp is a nonspecific chronic inflammatory response in the dura martin et al. Consequently, extensive preoperative imaging studies usually are not available. Although the lesion when associated with syphilis most often involves the cervical and.
A case of hypertrophic pachymeningitis associated with. Two patients with a chronic progressive myelopathy were successfully surgically treated and idiopathic hypertrophic spinal pachymeningitis ihsp was found on histology. Dec 30, 2019 hypertrophic pachymeningitis hp is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral andor spinal dura mater. 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.
The manifestations of the disease included headaches, paresis of vi, ix, x nerves and cerebellar. 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. Patients with this condition commonly present with cranial neuropathy accompanied by localized headache. 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 has been reported in 28%. Although the firstline therapy for hp is steroids, many hp cases are refractory to steroids or recur when the steroids are tapered. Cranial and lumbosacral hypertrophic pachymeningitis. A nationwide survey of hypertrophic pachymeningitis in. Here we are presenting a case of a young female presented with multiple. We have uploaded a genuine pdf ebook copy of this book to our online file repository so that you can enjoy a blazingfast and safe downloading experience. The patient suffered from progressive mild thoracic backache. Distinguishing magnetic resonance imaging features between. Full text get a printable copy pdf file of the complete article 1.
Cyc is the backbone of all treatment regimes for induction of remission. 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. There was associated headache, periorbital discomfort and diplopia on left gaze. Fiftyfive years later, charcot and joffroy 1873 background hypertrophic pachymeningitis hp is a rare clin published a clinical picture of the disease 1. Methods the study began with a preliminary survey to determine the approximate number of hp patients diagnosed from 1 january 2005 to 31 december 2009, and was followed by a questionnaire survey for clinical. Hypertrophic pachymeningitis, childs nervous system 10. Exhaustive bacteriological and histopathological studies failed to identify a specific cause for this hypertrophic cranial pachymeningitis. Immunoglobulin g4 igg4related disease is a systemic syndrome, characterized by sclerosing. Idiopathic hypertrophic pachymeningitis is a rare form of granulomatous meningitis. Pdf we report the treatment and followup, including magnetic resonance imaging mri of a patient with idiopathic hypertrophic. Idiopathic hypertrophic pachymeningitis genetic and rare. It could be idiopathic or due to variety of inflammatory and infectious conditions.
This imaging appearance in patients with chronic progressive. 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. Idiopathic hypertrophic pachymeningitis ihp is a very infrequent disorder that causes a localized or diffuse thickening of the dura mater. A proportion of idiopathic hypertrophic pachymeningitis cases may involve a component of meningeal igg4rd. It occurs in either of these areas alone or as a craniospinal form. 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. The case about to be reported belongs to the group of disorders erroneously referred to as chronic hypertrophic spinal pachymeningitis. Although the lesion when associated with syphilis most often involves the cervical and higher thoracic cord, lumbar involvement can also occur. Igg4related hypertrophic pachymeningitis is one of the many manifestations of igg4related disease which represents a fibroinflammatory condition that can affect any organ. Headache, cranial nerve palsy, and ataxia are the most common clinical manifestations. Hypertrophic granulomatous cranial pachymeningitis causing. We present a patient with an ihcp, nonresponsive to corticotherapy. Idiopathic hypertrophic pachymeningitis ihpm is a rare pathological state, with still unclear aetiopathogenesis. Hypertrophic pachymeningitis hp is characterized by inflammation of the dura mater.
Few series of the idiopathic variety have been described, particularly with respect to mri correlation to clinical outcome and treatment. Corticosteroid therapy has a definite but transient effect in alleviating manifestations of ihcp. Hypertrophic spinal pachymeningitis with special reference to. Idiopathic hypertrophic pachymeningitis with intracranial. Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Hypertrophic pachymeningitis as the first manifestation of systemic.
Pachymeningitis associated with a pulmonary nodule. Idiopathic hypertrophic pachymeningitis ihp is a rare disorder causing inflammation and thickening of the outer layer dura of the brain andor spinal cord. Iodized oils as an aid to the diagnosis of lesions of the spinal cord and a contribution to the knowledge of adhesive circumscribed meningitis. A novel model for treatment of hypertrophic pachymeningitis. It has been described in the setting of numerous systemic inflammatory diseases including immunoglobulin g4 igg4related disease as well as granulomatosis with polyangiitis gpa.
A 44yearold malay lady presented with drooping of the right eyelid and worsening of left eye vision for one week duration. Adequate therapeutic management is still a matter of debate. 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. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. The main clinical features of ihp at presentation include headache, cranial nerve involvement, ataxia, transient ischemic attacks tia and seizures. Numerous clinicopathological entities cause thickening of the pachymeninges, thus idiopathic hypertrophic pachy meningitis is diagnosed by exclusion. He has had one other patient who unlike me was asymptomatic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum.
Igg4related pachymeningitis is increasingly being recognized as the etiology of hypertrophic pachymeningitis, an entity which until recently was thought to be idiopathic. Objectives to clarify the prevalence, frequent causes and distinct features of hypertrophic pachymeningitis hp according to background conditions in a nationwide survey in japan. Secondary could be due to granulomatous, paraneoplastic and autoimmune etiologies. Pdf hypertrophic pachymeningitis case report researchgate. Hypertrophic pachymeningitis in rheumatoid arthritis after. Hypertrophic pachymeningitis karthik s n, bhanu k, velayutham. Hcp mainly presents headache and paralysis of multiple cranial nerves. It was diagnosed by mri while looking for the cause of hearing loss. Neurosurgical considerations for treating igg4related. An electronic family health history tool to identify and manage patients at increased risk for colorectal cancer. Chronic idiopathic constipation disorder 82934008, snomedct chronic idiopathic monocytosis disorder 2232, snomedct chronic idiopathic pain syndrome disorder 736464002, snomedct chronic left idiopathic vestibulopathy disorder 762307002, snomedct chronic right idiopathic vestibulopathy disorder 762308007, snomedct congenital erosive and vesicular dermatosis.
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. Intracranial hypotension ich and distension of anterior internal vertebral venous plexus ivvp have. Case report idiopathic hypertrophic pachymeningitis case. Igg4related disease igg4rd is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges. Mar 15, 2015 hypertrophic pachymeningitis hp is a rare clinical entity characterized by diffuse or localized fibrous thickening of the dura mater.
Importance igg4related hypertrophic pachymeningitis igg4rhp is an increasingly recognized manifestation of igg4related disease, a fibroinflammatory condition that can affect virtually any organ. Tuberculous hypertrophic pachymeningitis involving cranio cervical region is rarely reported. Hypertrophic spinal pachymeningitis with special reference. Histogenesis of cerebral hypertrophic pachymeningitis and its. Oral as well as two to threeweekly pulses of intravenous cyclophosphamide have been recommended for remission induction in systemic disease 25. 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. 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.
Results the symptom onset in the 8 patients was ranged from 43 to 78 years old. Get a printable copy pdf file of the complete article 948k. 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. It was first described by charcot and later by naffziger and stern. Hypertrophic pachymeningitis is an extremely rare fibrosing inflammatory process involving the dura mater.
Idiopathic hypertrophic cranial pachymeningitis ihcp is a rare disease with pain and compression. Hypertrophic pachymeningitis is a clinical disorder due to localized or diffuse thickening of the dura mater, with or without an associated inflammation. Download imaging in spine surgery 1st edition pdf free. Clinical analysis of hypertrophic pachymeningitis acr. The leading diagnostic finding of ihcp consists of diffuse or localised thickening of the dura, which demands appropriate imaging and image interpretation. This article is from clinical and experimental otorhinolaryngology, volume 4. Hypertrophic pachymeningitis is a unique clinical entity characterized by fibrosis and thickening of dura mater resulting in neurological dysfunction. Early reports were in relationship to tuberculosis or syphilis. Hypertrophic pachymeningitis in systemic sclerosis sine. In both patients, an extensive extramedullary mass of low t2 signal with peripheral contrast enhancement was compressing the spinal cord on mr imaging. It is complicated by also having rheumtoid arthritis.
On imaging, it presents as a localized, multiple, or diffuse enhancing dural thickening commonly forming masslike lesions. Idiopathic hypertrophic cranial pachymeningitis sciencedirect. Abstracthypertrophic pachymeningitis is a progressive disease resulting in a. Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. Optic neuropathy associated with hypertrophic cranial. Idiopathic hypertrophic pachymeningitis successfully treated. Available formats pdf please select a format to send. Hypertrophic pachymeningitis is a condition where there is localized inflammatory thickening of the dura. Bilateral vision loss secondary to pachymeningitis in a. Cranio cervical tuberculous hypertrophic pachymeningitis.
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