ABSTRACT
A case of IgG4-related hypertrophic pachymeningitis was reported. The patient was an elderly female, with the course of disease more than 8 years. Clinical manifestations included recurrent headache, vision and hearing loss, exophthalmos and thyroid dysfunction. Finally, she was diagnosed as IgG4-related disease and IgG4-related hypertrophic pachymeningitis by PET-CT and dural biopsy. After treatment with methylprednisolone and mycophenolate mofetil, the patient′s clinical symptoms improved.
ABSTRACT
To study the clinical features of myeloperoxidase(MPO) antineutrophil cytoplasmic antibody (ANCA) associated hypertrophic pachymeningitis (HP). Clinical data of 15 cases diagnosed with MPO-ANCA vasculitis complicated with HP were retrospectively analyzed. Nine cases were males and the other 6 were females, with an average age of (58±8) years. All cases presented with chronic headache. Contrast-enhanced magnetic resonance imaging (MRI) scan showed local or diffused thickening of cerebral and/or spinal dura matter while brain parenchyma were normal. Nine cases developed multiple cranial nerve paralysis, with trigeminal nerve and auditory nerve involved most commonly. The main clinical manifestations were facial pain, hearing loss and tinnitus. Two cases were complicated with hypertrophic spinal pachymeningitis (HSP) and 4 cases were complicated with pulmonary diseases. Positive serum perinuclear pattern ANCA (pANCA) and MPO could be found in all cases, positive serum IgG 4 was seen in two patients. erythrocyte sedimentation rate(ESR;25-116 mm/1h) and C-reactive protein (CRP;29.02-146.00 mg/L) were both elevated in 14 cases. Nine cases had elevated intracranial pressure[180-235 mmH 2O (1 mmH 2O=0.009 8 kPa)] and abnormal protein level (457.6-3710.0 mg/L) in cerebrospinal fluid. Six cases were treated with glucocorticoids (prednisone 20-60 mg/d) and 9 cased with glucocorticoids and immunosuppressants (methotrexate 15 mg/week or cyclophosphamide 100 mg/d po). All patients achieved remission. MPO-ANCA associated HP is a special type of central nervous system involvement in ANCA associated vasculitis (AAV). It rarely involves the lung or kidney. Steroids and immunosuppressive agents are effective. In HP with unknown underlying diseases, it is suggested to screen ANCA and IgG 4 tests for AAV or IgG 4-related disease.
ABSTRACT
Paciente de 65 años, con diagnóstico de Granulomatosis con Poliangeitis (GPA) de 18 años de evolución cuyo debut fue por insuficiencia respiratoria aguda asociado a hemoptisis recibiendo tratamiento con corticoides sistémicos y ciclofosfamida de inducción. Luego recibió mantenimiento con azatioprina 150 mg día, con periodos de recrudecimiento de enfermedad que respondieron al tratamiento con corticoides por períodos cortos. Acude a consulta por cefalea crónica de tres meses de evolución refractaria al tratamiento con antiinflamatorios no esteroides (AINES), asociado a proptosis ocular izquierda y dolor orbitario homolateral, presentando reactantes de fase aguda elevados (eritrosedimentación y Proteína C reactiva). Se evidencia por resonancia magnética nuclear cerebral con gadolinio, realce de la duramadre cerebral y tienda de cerebelo, presentando además una formación orbitaria izquierda.
A 65-year-old patient, with a diagnosis of Granulomatosis with Polyangeitis (GPA) of 18 years of evolution, whose debut was with respiratory failure and hemoptysis, receiving induction treatment with corticosteroids together with cyclophosphamide, and then maintenance treatment with azathioprine 150 mg per day, with periods of flare-up of the disease that responded to treatment with corticosteroids for short periods. He came to the clinic for a 3-month-long chronic headache refractory to treatment with non-steroidal anti-inflammatory drugs (NSAIDs), associated with left ocular proptosis and ipsilateral orbital pain, presenting elevated acute phase reactants (ers and c-reactive protein). It is evidenced by brain magnetic resonance with gadolinium, enhancement of the cerebral dura and cerebellum store, also presenting formation in the left orbit.
Subject(s)
Granulomatosis with Polyangiitis , Respiratory Insufficiency , Therapeutics , Magnetic Resonance SpectroscopyABSTRACT
ABSTRACT Granulomatosis with polyangiitis (GPA) is a vasculitic disease with an infrequent involvement of the central nervous system. This can lead, in rare cases, to hypertrophic pachymeningitis (HP), which is characterized by inflammation and fibrosis that cause a thickening of dura mater. At present, it is crucial to consider GPA in the differential diagnosis of elderly patients with intracranial hypertension. The case is presented of a 60-year-old male with progressive severe headache, vomiting, and wasting syndrome. Physical examination showed pallor, weight loss, and unilateral papilloedema. A gadolinium-enhanced brain MRI scan showed sinusitis, chronic otomastoiditis, and hypertrophic pachymeningitis. Finally, a meningeal biopsy concluded a necrotising granulomatous vasculitis compatible with GPA. However, PR3- and MPO-ANCA were negative. After corticosteroid therapy was initiated, the patient had a favorable outcome during his hospital stay.
RESUMEN La granulomatosis con poliangeítis (GPA) compromete excepcionalmente el sistema nervioso central, conllevando en raras ocasiones a una paquimeningitis hipertrófica (PH), caracterizada por inflamación y fibrosis, que originan un engrosamiento de la duramadre. Actualmente, su consideración es crucial en el diagnóstico diferencial de pacientes ancianos con hipertensión endocraneana. Presentamos el caso de un adulto de 60 anos con cefalea severa progresiva, vómitos, papiledema unilateral y síndrome consuntivo en donde la resonancia magnética cerebral contrastada con gadolinio muestra sinusitis, otomastoiditis y PH. Finalmente, la biopsia de meninges reveló vasculitis granulomatosa necrosante de pequenos y medianos vasos compatible con GPA. Empero, PR3- y MPO-ANCA resultaron negativos. Se inició terapia con corticoides, presentando una evolución clínica favorable durante su hospitalización.
Subject(s)
Humans , Male , Middle Aged , Granulomatosis with Polyangiitis , Meningitis , Central Nervous System , Diagnosis , HeadacheABSTRACT
ABSTRACT Sjögren syndrome (SS) is an autoimmune disease, rarely affecting the central nervous system. The case is reported on a 68-year-old male patient who presented with keratocon-junctivitis sicca and xerostomia, during neuropsychological evaluation showed moderate to severe multidomain cognitive impairment. The magnetic resonance showed evidence of hypertrophic pachymeningitis. The CSF study showed pleocytosis, mild proteinuria, and negative cultures. The immune positive profile, Anti SS-A, SS-B and salivary gland biopsy was compatible with SS. The treatment was started with methylprednisolone pulses at a dose of 1g every 24 h for three days, and controlled with maintenance therapy, showing a favorable response. Chronic meningitis is a diagnostic challenge; among the less common causes is hypertrophic pachymeningitis in SS, and less frequently in male patients (1/9). Non-specific neurological manifestations should be considered, such as headaches or neuropsychiatric symptoms, as on some occasions rare cases may be encountered, such as the one described.
RESUMEN El síndrome de Sjögren (SS) es una enfermedad autoinmune que rara vez afecta al sistema nervioso central. En este informe de caso describimos a un paciente varón, de 68 años, que presentó queratoconjuntivitis seca y xerostomía, y a la evaluación neuropsicológica mostró deterioro cognitivo multidominio en grado de moderado a grave. La resonancia magnética mostró evidencia de paquimeningitis hipertrófica. El estudio del LCR mostró pleocitosis, proteinuria leve y cultivos negativos; el perfil inmunológico positivo anti SS-A, SS-B y la biopsia de la glándula salival compatible con SS. El tratamiento se inició con pulsos de metilprednisolona a una dosis de 1 g/cada 24 h/durante 3 días, control con terapia de mantenimiento, mostrando una respuesta favorable. La meningitis crónica es un desafío para el diagnóstico, entre las causas menos frecuentes está la paquimeningitis hipertrófica en el SS, y con menor frecuencia en los pacientes varones (1/9). Se deben considerar manifestaciones neurológicas inespecíficas, como dolores de cabeza o síntomas neuropsiquiátricos, ya que en algunas ocasiones podemos enfrentarnos a casos raros como el descrito.
Subject(s)
Humans , Male , Aged , Sjogren's Syndrome , Meningitis , Signs and Symptoms , Causality , Diagnosis , Neurologic ManifestationsABSTRACT
La paquimeningitis hipertrófica (PH), es una manifestación poco frecuente de la vasculitis asociada a anticuerpos anti-citoplasma de neutrófilo (ANCA). La literatura describe compromiso de sistema nervioso central (SNC) en 2-8% de los casos en pacientes con vasculitis pauciinmune. Se presenta el caso de un paciente con antecedente de vasculitis anti-mieloperoxidasa (MPO) con un mes de evolución de cefalea hemicraneana izquierda. La resonancia magnética cerebral contrastada evidencia marcado engrosamiento y realce meníngeo dural en el hemicráneo izquierdo, predominante en el tentorio y la fosa posterior. Se descartaron causas infecciosas por lo que se llegó a la conclusión de compromiso meníngeo asociado a vasculitis. Se inició manejo inmunosupresor con mejoría del cuadro clínico. La rápida identificación y manejo de esta entidad puede cambiar su pronóstico sombrío. Se realizó una revisión de la literatura para brindar una herramienta para la toma de decisiones para los médicos que se enfrentan a esta entidad.
Hypertrophic pachymeningitis (PH) is a rare manifestation of vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA). The literature describes central nervous system (CNS) involvement in 2-8% of cases in patients with pauciimmune vasculitis. We present the case of a patient with a history of anti-Myeloperoxidase (MPO) vasculitis with a 1-month history of left-sided headache. Contrast brain magnetic resonance was performed with evidence of marked thickening and dural meningeal enhancement in the left hemicranium, predominantly in the region of the tentorium and posterior fossa. Infectious causes were ruled out and the meningeal compromise associated with vasculitis was concluded. Immunosuppressive management was started with improvement of the clinical picture. Rapid identification and management of this entity can change its bleak outlook. A systematic review of the literature was carried out in order to provide a decision-making tool for physicians facing this entity.
Subject(s)
Humans , Female , Middle Aged , Vasculitis/immunology , Antibodies, Antineutrophil Cytoplasmic/immunology , Meningitis/etiology , Vasculitis/complications , Magnetic Resonance Spectroscopy , Antibodies, Antineutrophil Cytoplasmic/drug effects , Immunosuppressive Agents/therapeutic use , Meningitis/diagnostic imagingABSTRACT
Immunoglobulin G4-related hypertrophic pachymeningitis (IgG4-RHP) is an increasingly recognized manifestation of IgG4-related disease (IgG4-RD), which is a fibroinflammatory condition that can affect virtually any organ. The three hallmark histopathological features of IgG4-RD are lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. We report a case of biopsy-confirmed IgG4-RHP that was initially misdiagnosed as cerebral venous thrombosis.
Subject(s)
Fibrosis , Immunoglobulins , Intracranial Thrombosis , Meningitis , Phlebitis , Plasma Cells , Venous ThrombosisABSTRACT
La paquimeningitis hipertrófica es una enfermedad infrecuente. Puede ser idiopática, secundaria a infección o enfermedad oncológica. Recientemente se la describió asociada a la enfermedad por IgG4, pudiendo ser esta la causa de muchas clasificadas como idiopáticas. Se presenta el caso de un hombre de 60 años de edad con historia de cefalea y epiescleritis, con respuesta parcial a corticoides. La resonancia magnética con contraste evidenciaba refuerzo meníngeo a nivel temporal y occipital izquierdo con extensión a la tienda del cerebelo. La biopsia meníngea demostró fibrosis e infiltrado linfoplasmocitario con más de 10 células plasmáticas IgG4 positivas por campo de gran aumento. El tratamiento con rituximab provocó mejoría clínica y radiológica. La paquimeningitis hipertrófica es una manifestación de la enfermedad por IgG4 y debe ser sospechada con la resonancia magnética y niveles plasmáticos altos de IgG4.
Hypertrophic pachymeningitis is an infrequent disorder. It can be idiopathic or secondary to infectious, autoimmune or neoplastic disease. The recently described ‹IgG4-related disease› could be the origin of many cases considered cryptogenic. We present the case of a 60-year-old man, with a history of headache and episcleritis in both eyes, with partial response to corticoid therapy. The brain MR study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left temporal and occipital lobes to ipsilateral tentorium. Meningeal biopsy showed fibrosis and lymphoplasmacytic infiltrate, with more than 10 IgG4+ plasma cells per high power field. After treatment with rituximab there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging. Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be based on MRI findings if plasma IgG4 are elevated.
Subject(s)
Humans , Male , Middle Aged , Immunoglobulin G , Autoimmune Diseases of the Nervous System/diagnosis , Autoimmune Diseases of the Nervous System/etiology , Meningitis/diagnosis , Meningitis/etiology , Biopsy , Magnetic Resonance Imaging , Tomography, X-Ray ComputedABSTRACT
BACKGROUND AND PURPOSE: 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 the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are tapered. Here we report three HP cases that were successfully treated with rituximab (RTX). METHODS: From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m²). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI. RESULTS: All three patients were recurrence-free of neurologic symptoms and exhibited prominent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients. CONCLUSIONS: We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.
Subject(s)
Humans , Autoimmunity , Cohort Studies , Cranial Nerve Diseases , Headache , Magnetic Resonance Imaging , Meningitis , Neurologic Manifestations , Paresthesia , Rare Diseases , Recurrence , Rituximab , Seizures , SteroidsABSTRACT
Objective To investigate the diagnostic value of MRI in hypertrophic pachymeningitis (HP). Methods MRI images of 11 patients with HP were analyzed retrospectively. The characteristic signs of the MRI images, and the relationship between the MRI features and pathology of HP were reviewed. Results In the 11 cases of HP, involvement of cerebral dura mater accounted for 8 cases, with spinal dura mater involvement in 2, both cerebral and spinal dura mater involvement in the other one case. The main sign of HP in MRI was an increased thickness of the dura mater, which showed iso-or hypointensity on T1WI without significant difference as compared with the brain parenchyma and spinal cord. The thickened dura mater showed obviously low signal on T2WI, and it was recognized easily as compared with the brain parenchyma and the spinal cord. On the DWI of 9 cases who had cerebral dura mater involvement, increased dura mater showed lower signal, but it was not so distinct as shown on T2WI. On the post-contrast T1WI, the thickened dura mater showed obvious enhancement, displaying better images than that of preenhancement image. Conclusion Among the different MRI techniques, T2-weighted imaging and post-enhanced T1WI have their advantages in the diagnosis of HP, and other MR imaging techniques could be complementary in increasing diagnostic accuracy and comprehensiveness.
ABSTRACT
A 25-year-old man presented with blurred vision and chronic headache. His brain MRI revealed bilateral frontal pachymeningeal enhancement with leptomeningeal enhancement. The patient had experienced recurrent oral ulcer and had anterior uveitis and papulopustules skin lesion. We diagnosed him with hypertrophic pachymeningitis (HP) associated with neuro-Behcet's disease (NBD). There have been few reports describing HP in patients with NBD. We report a case of NBD presenting as HP.
Subject(s)
Adult , Humans , Brain , Headache Disorders , Magnetic Resonance Imaging , Meningitis , Oral Ulcer , Skin , Uveitis, AnteriorABSTRACT
Idiopathic hypertrophic pachymeningitis (IHP) is a rare disease, and it is characterized by chronic progressive inflammatory fibrosis and thickening of the dura mater with resultant compression of the spinal cord or neural structure without any identifiable cause. It can occur in the intracranial or spinal dura mater alone or as a craniospinal form. The spinal form is rarer than the cranial form and the craniospinal form is extremely rare. We report a rare case of IHP in the craniocervical junction involving both the cranial and spinal dura mater and discuss the diagnosis and management of the disease.
Subject(s)
Diagnosis , Dura Mater , Fibrosis , Meningitis , Rare Diseases , Spinal CordABSTRACT
Hypertrophic pachymeningitis is a progressive disease resulting in a diffuse thickening of dura mater due to inflammation, tumor or autoimmune diseases, but most cases are idiopathic. It is seldom reported to be related to sensorineural hearing loss, but it can cause sensorineural hearing loss which can be potentially reversed through treatment. Here, we report the case of a 54-year-old woman who had progressive, bilateral, worse in the left, sensorineural hearing loss and visual disturbance with an accompanying headache over several months. Brain MRI showed diffusely thickened dura mater, highly enhanced after gadolinium administration, which was consistent with pachymeningitis. It was assumed to be related to autoimmune pathogenesis on the basis of elevated serum myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titers. After empirical steroid and cyclophosphamide therapy, auditory impairment improved, especially in the high frequency region of the pure tone audiogram, and significant improvement in the word recognition test. Moreover, a follow-up MRI revealed much decreased enhancement of the dura mater, and the MPO-ANCA titer decreased to within the normal range. In the case of rapidly progressive sensorineural hearing loss or hearing impairment accompanying other cranial neuropathy, pachymeningitis should be taken into consideration, and brain MRI with gadolinium enhancement is the best method of detecting it. Also, to ensure proper treatment, a cautious evaluation including an ANCA work-up should be performed.
Subject(s)
Female , Humans , Middle Aged , Antibodies, Antineutrophil Cytoplasmic , Autoimmune Diseases , Brain , Cranial Nerve Diseases , Cyclophosphamide , Cytoplasm , Dietary Sucrose , Dura Mater , Follow-Up Studies , Gadolinium , Headache , Hearing Loss , Hearing Loss, Sensorineural , Inflammation , Meningitis , Reference Values , Granulomatosis with PolyangiitisABSTRACT
PURPOSE: To report a case of bilateral optic neuropathy related with idiopathic hypertropic pachymeningitis. CASE SUMMARY: A 66-year old woman presented with acute visual loss that developed 6 days previously in the right eye and 3 days prior in the left eye. During the initial evaluation, her visual acuity was light perception in the right eye and counting fingers at 30 cm in the left eye. A relative afferent papillary defect (RAPD) was noted in the patient's right eye. On brain MRI, the duramater was thickened and enhanced by contrast. The erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP) level were increased and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) as positive. Other neurological and medical abnormalities were not found. Under the suspicion of bilateral optic neuropathy due to idiopathic hypertrophic pachymeningitis, the patient was treated with methylprednisolone pulse therapy. Two days after the treatment, the visual acuity was hand movements at 20 cm in the right eye and 0.6 in the left eye. Three months after the treatment, the visual acuity was counting fingers at 20 cm in the right eye, and 0.7 in the left eye. The right eye showed optic disc pallor and diffuse retinal nerve fiber layer (RNFL) defect. The left eye showed a suspicious RNFL defect, and was otherwise normal. CONCLUSIONS: The authors report a rare case of optic neuropathy related with idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis should be considered as one of the various causes of optic neuropathy.
Subject(s)
Female , Humans , Blood Sedimentation , Brain , C-Reactive Protein , Cytoplasm , Eye , Fingers , Hand , Light , Meningitis , Methylprednisolone , Nerve Fibers , Optic Nerve Diseases , Pallor , Patient Rights , Retinaldehyde , Visual AcuityABSTRACT
Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been reported to be infrequently associated with systemic autoimmune disorders such as Wegener's granulomatosis, rheumatoid arthritis, sarcoidosis, Behcet's disease, Sjogren syndrome, and temporal arteritis. Here, we report a case of HCP initially presented with scleritis and headache in a patient with undifferenciated connective tissue disease (UCTD). HCP was initially suspected on brain magnetic resonance imaging and defined pathologically on meningial biopsy. Immunologic studies showed the presence of anti-RNP antibody. After high dose corticosteroid therapy, the patient's symptoms and radiologic abnormalities of brain were improved. Our case suggested that HCP should be considered in the differential diagnosis of headache in a patient with UCTD presenting with scleritis.
ABSTRACT
A 59-year-old woman presented with a 2-month history of headache with left eyelid swelling, and erythema in her lower extremities. She also had persistent low-grade fever and weight loss of 3 kg over 2 months. Blood biochemistry tests performed elsewhere indicated an inflammatory reaction. One week before hospitalization, she was treated at our hospital using the Japanese herbal medicines, keishito and shosaikoto. On hospitalization, her headache and eyelid swelling had slightly decreased ; therefore, she was advised to continue the Japanese herbal medicine, shosaikoto. Contrast-enhanced brain MRI showed meningeal thickening extending from the right anterior cranial fossa to the left cavernous sinus, suggesting a diagnosis of hypertrophic pachymeningitis. Cerebrospinal fluid examination revealed mild pleocytosis with mononuclear leukocyte predominance. Biopsy of the erythematous lesion on her leg was carried out, and analysis of the obtained specimen led to the diagnosis of erythema nodosum. In this case, exhaustive efforts to identify the cause of the combined hypertrophic pachymeningitis and erythema nodosum were unsuccessful. However, the symptoms and signs were alleviated after treatment with the Japanese herbal medicine, shosaikoto. We did not administer steroid therapy, which is generally prescribed for such an immune-inflammatory disease, because of the possible presence of an infectious disease. The Japanese herbal medicine was found to be a candidate therapeutic agent in this case.
ABSTRACT
Idiopathic hypertrophic pachymeningitis is a rare inflammatory disorder of the dura mater. Spinal involvement is extremely rare and there are few case reports. We present a 36 year-old female of idiopathic hypertrophic spinal pachymeningitis compressing thoracic spinal cord which showed rapid recurrence.
Subject(s)
Female , Humans , Dura Mater , Meningitis , Recurrence , Spinal Cord , SpineABSTRACT
Idiopathic hypertrophic pachymeningitis is a clinical disorder caused by a localized or diffuse thickening of the dura mater, with an associated chronic inflammation. This can be diagnosed when there is no evidence of other etiologies such as trauma, infection, tumors, and Wegener's disease. Clinical manifestations are chronic headache with or without neurological manifestations such as cranial nerve palsies, cerebellar ataxia, neuro-ophthalmologic complications, and rarely clinical seizures. We described a patient with simple partial seizures with focal sensory and motor symptoms in the right hand as an initial and the only clinical manifestation, accompanied by a tumor-like lesion in the left parietal convexity on brain MRI. The patient underwent a lesionectomy, and the seizures have been well controlled so far without immunosuppressant treatment.
Subject(s)
Humans , Brain , Cerebellar Ataxia , Cranial Nerve Diseases , Dura Mater , Epilepsies, Partial , Hand , Headache Disorders , Inflammation , Magnetic Resonance Imaging , Meningitis , Neurologic Manifestations , Rabeprazole , SeizuresABSTRACT
Idiopathic hypertrohpic cranial pachymeningitis is rare, and is essentially a diagnosis of exclusion. A 53-year-old man presented with headache and visual loss in the right eye, first experienced a month earlier. MR images depicted a mass in the right cavernous sinus. At T1-weighted imaging, both the mass and the thickened dura mater present in both fromted lobes were isointense, while at while T2-weighted imaging, the signal intensity of both the mass and the dura mater was low. After the injection of contrast medium, pachymeningeal enhancement was observed. We report the radiologic findings in a case of idiopathic hypertrophic cranial pachymeningitis, confirmed surgically and pathologically.
Subject(s)
Humans , Middle Aged , Cavernous Sinus , Diagnosis , Dura Mater , Headache , MeningitisABSTRACT
Idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic nonspecific and granulomatous inflammatory disorder of the dura with unknown etiology. The diagnosis can be established by open biopsy and exclusion of all other specific granulomatous and infectious diseases. We report a typical case of spinal IHP occurring in a long segment of cervical and thoracic dura from C6 to T8. The patient was 56-yr-old female, who had been suffered from pain on her upper back and both arms for 3 months and recent onset motor weakness of both legs. During the 9 months of follow-up period, she experienced the improvement of her neurologic symptoms with combined therapy of partial excision and corticosteroid medication. Since early surgical intervention and subsequent pulse ste-roid therapy are mandatory for this disease to avoid irreversible damage of nervous system, the identification of this unique disease entity is essential on frozen diagnosis. A few cases have been reported in Korean literature.