Limits...
A case of hypertrophic cranial pachymeningitis presenting with scleritis in a patient with undifferentiated connective tissue disease.

Kim JH, Joo YB, Kim J, Min JK - J. Korean Med. Sci. (2010)

Bottom Line: 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, Behçet's disease, Sjögren syndrome, and temporal arteritis.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Holy Family Hospital, Bucheon, Korea.

ABSTRACT
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, Behçet's disease, Sjögren syndrome, and temporal arteritis. Here, we report a case of HCP initially presented with scleritis and headache in a patient with undifferentiated 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.

Show MeSH

Related in: MedlinePlus

Magnetic resonance image of the brain. (A) On the 3rd hospital day, axial T1-weighted contrast-enhanced magnetic resonance image shows diffuse thick dural enhancement along the left tentorium and left cerebellar surface. (B). In 4 weeks after the initial glucocorticoid treatment, follow-up magnetic resonance image demonstrated nearly complete regression of dural enhancement. Only very thin dural enhancement remained.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC2877233&req=5

Figure 2: Magnetic resonance image of the brain. (A) On the 3rd hospital day, axial T1-weighted contrast-enhanced magnetic resonance image shows diffuse thick dural enhancement along the left tentorium and left cerebellar surface. (B). In 4 weeks after the initial glucocorticoid treatment, follow-up magnetic resonance image demonstrated nearly complete regression of dural enhancement. Only very thin dural enhancement remained.

Mentions: A 37-yr-old man was referred to our emergency room with a provisional diagnosis of a subdural hemorrhage in April 18, 2009. He has complained of history of headache with intermittent nausea and vomiting for two weeks. Brain computed tomography (CT) was done at local clinic to evaluate these symptoms and unenhanced CT scans showed high-density areas along the left tentorium cerebelli. He had been managed with prednisolone (5-30 mg/day) by ophthalmologist in our hospital under the diagnosis of scleritis since January 2008. Thirty milligrams of prednisone was administrated for 7 days initially and then tapered. At the time of symptom developing, the patient was being treated with prednisone 10 mg. The patient has complained of intermittent hand joint pain, swelling, and Raynaud's phenomenon for 3 yr. He had neither other past history nor familial history. He denied a history of head trauma. The initial vital signs were stable. On physical examination, both eyes appeared red. No arthritic pain was observed. The rest of the clinical examination was normal including neurological examination. Complete blood cell counts were within normal limits. Blood analysis showed an erythrocyte sedimentation rate of 48 mm/hr and C-reactive protein of 44.49 mg/L (normal <3.0 mg/L). The values for blood glucose, liver function, renal function, electrolytes, C3, and C4 were their normal ranges. In the tests for autoantibody, the titer of antinuclear antibody was 1:1,600 and anti-ribonucleoprotein (RNP) antibody 63.42 EU. But, antibodies to double stranded DNA, Sm, Ro, La, Scl-7, Jo-1, centromere, nucleosomes, and histone were negative. Rheumatoid factor, anti-CCP antibody, and ANCA were also negative. Tests for syphilis, hepatitis B surface antigen, anti-HBs antibody, anti-HCV antibody, anti-HTLV-1 antibody, and anti-HIV were negative. The level of serum angiotensin-converting enzyme was normal. The urinalysis was normal. CSF examination showed increased opening pressure (250 mmH2O), proteins (99 mg/dL), and cells (28/µL, lymphocyte 98%). CSF cultures and staining for acid-fast bacilli, bacteria and fungi were negative. The patient complained of diplopia on the 2nd hospital day and peripheral type facial palsy on the 10th hospital day. A chest radiography showed calcified nodules in the right upper lobe. Chest CT showed 3.3×1.6 cm sized irregular soft tissue density in the left upper anterior pericardial region (Fig. 1A). Brain MRI showed a left tentorium cerebelli lesion that was iso-intensity on T1 weighted MRI and hypointensity on T2-weighted imaging with marked enhancement after gadolinium (Fig. 2A). On the 19th hospital day, the patient underwent biopsy of left tentorium cerebella. Meningeal biopsy showed fibrosis accompanied by the infiltration of chronic inflammatory cells without evidence of vasculitis or granuloma (Fig. 3). From the 3rd hospital day after meningeal biopsy, 20 mg of intravenous dexamethasone was administered for 6 days, then changed to oral prednisolone (1 mg/kg) with gradual tapering. Follow-up MRI, performed at 4 weeks after the initial glucocorticoid therapy, showed much regression of dural enhancement (Fig. 2B). A follow-up chest CT scan 5 months after the initial glucocorticoid therapy demonstrated a marked decrease of the size of the pericardial mass (Fig. 1B). He recovered from diplopia and facial nerve palsy with glucocorticoid therapy. Currently, after 10 months of follow-up after introduction of glucocorticoid, the patient is doing well with low doses of glucocorticoid.


A case of hypertrophic cranial pachymeningitis presenting with scleritis in a patient with undifferentiated connective tissue disease.

Kim JH, Joo YB, Kim J, Min JK - J. Korean Med. Sci. (2010)

Magnetic resonance image of the brain. (A) On the 3rd hospital day, axial T1-weighted contrast-enhanced magnetic resonance image shows diffuse thick dural enhancement along the left tentorium and left cerebellar surface. (B). In 4 weeks after the initial glucocorticoid treatment, follow-up magnetic resonance image demonstrated nearly complete regression of dural enhancement. Only very thin dural enhancement remained.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2877233&req=5

Figure 2: Magnetic resonance image of the brain. (A) On the 3rd hospital day, axial T1-weighted contrast-enhanced magnetic resonance image shows diffuse thick dural enhancement along the left tentorium and left cerebellar surface. (B). In 4 weeks after the initial glucocorticoid treatment, follow-up magnetic resonance image demonstrated nearly complete regression of dural enhancement. Only very thin dural enhancement remained.
Mentions: A 37-yr-old man was referred to our emergency room with a provisional diagnosis of a subdural hemorrhage in April 18, 2009. He has complained of history of headache with intermittent nausea and vomiting for two weeks. Brain computed tomography (CT) was done at local clinic to evaluate these symptoms and unenhanced CT scans showed high-density areas along the left tentorium cerebelli. He had been managed with prednisolone (5-30 mg/day) by ophthalmologist in our hospital under the diagnosis of scleritis since January 2008. Thirty milligrams of prednisone was administrated for 7 days initially and then tapered. At the time of symptom developing, the patient was being treated with prednisone 10 mg. The patient has complained of intermittent hand joint pain, swelling, and Raynaud's phenomenon for 3 yr. He had neither other past history nor familial history. He denied a history of head trauma. The initial vital signs were stable. On physical examination, both eyes appeared red. No arthritic pain was observed. The rest of the clinical examination was normal including neurological examination. Complete blood cell counts were within normal limits. Blood analysis showed an erythrocyte sedimentation rate of 48 mm/hr and C-reactive protein of 44.49 mg/L (normal <3.0 mg/L). The values for blood glucose, liver function, renal function, electrolytes, C3, and C4 were their normal ranges. In the tests for autoantibody, the titer of antinuclear antibody was 1:1,600 and anti-ribonucleoprotein (RNP) antibody 63.42 EU. But, antibodies to double stranded DNA, Sm, Ro, La, Scl-7, Jo-1, centromere, nucleosomes, and histone were negative. Rheumatoid factor, anti-CCP antibody, and ANCA were also negative. Tests for syphilis, hepatitis B surface antigen, anti-HBs antibody, anti-HCV antibody, anti-HTLV-1 antibody, and anti-HIV were negative. The level of serum angiotensin-converting enzyme was normal. The urinalysis was normal. CSF examination showed increased opening pressure (250 mmH2O), proteins (99 mg/dL), and cells (28/µL, lymphocyte 98%). CSF cultures and staining for acid-fast bacilli, bacteria and fungi were negative. The patient complained of diplopia on the 2nd hospital day and peripheral type facial palsy on the 10th hospital day. A chest radiography showed calcified nodules in the right upper lobe. Chest CT showed 3.3×1.6 cm sized irregular soft tissue density in the left upper anterior pericardial region (Fig. 1A). Brain MRI showed a left tentorium cerebelli lesion that was iso-intensity on T1 weighted MRI and hypointensity on T2-weighted imaging with marked enhancement after gadolinium (Fig. 2A). On the 19th hospital day, the patient underwent biopsy of left tentorium cerebella. Meningeal biopsy showed fibrosis accompanied by the infiltration of chronic inflammatory cells without evidence of vasculitis or granuloma (Fig. 3). From the 3rd hospital day after meningeal biopsy, 20 mg of intravenous dexamethasone was administered for 6 days, then changed to oral prednisolone (1 mg/kg) with gradual tapering. Follow-up MRI, performed at 4 weeks after the initial glucocorticoid therapy, showed much regression of dural enhancement (Fig. 2B). A follow-up chest CT scan 5 months after the initial glucocorticoid therapy demonstrated a marked decrease of the size of the pericardial mass (Fig. 1B). He recovered from diplopia and facial nerve palsy with glucocorticoid therapy. Currently, after 10 months of follow-up after introduction of glucocorticoid, the patient is doing well with low doses of glucocorticoid.

Bottom Line: 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, Behçet's disease, Sjögren syndrome, and temporal arteritis.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Holy Family Hospital, Bucheon, Korea.

ABSTRACT
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, Behçet's disease, Sjögren syndrome, and temporal arteritis. Here, we report a case of HCP initially presented with scleritis and headache in a patient with undifferentiated 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.

Show MeSH
Related in: MedlinePlus