Hypertrophic pachymeningitis: varied manifestations of a single disease entity.
ABSTRACT Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Three cases of this entity are described. Presenting features were headaches and cranial neuropathies in two patients and predominantly cerebellar dysfunction in the third. One of the patients also had evidence of spinal involvement. Lower cranial nerves were chiefly involved in two patients whereas optic nerve was the predominantly affected nerve in one. Except for the presence of rheumatoid arthritis in one of the patients, we could not document clinical or biochemical evidence of any predisposing infective, inflammatory or infiltrative condition in the other two. All three patients had characteristic changes on imaging suggestive of thickened and enhancing duramater. Although variable steroid responsiveness was seen in all the three patients, tendency towards steroid dependence was evident. The clinical presentations, causes, radiological features, management options and differential diagnosis of this unique clinical syndrome have been discussed.
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ABSTRACT: Idiopathic hypertrophic pachymeningitis (IHP) is a chronic progressive diffuse inflammatory fibrosis of the dura-mater, leading to its diffuse enlargement. The following describes a case of IHP presenting with a superficial soft tissue mass. A 40-year-old female came to hospital with a subcutaneous lump over the left face and frontal headache for 6 months. An excision biopsy revealed chronic inflammation. Magnetic resonance imaging (MRI) of the brain showed left mastoiditis and early dural inflammation of the left temporal region. A few months later, she developed diplopia, complex partial seizures, and retrobulbar neuritis of the left optic nerve. Repeat MRI brain demonstrated meningeal thickening on both sides of the tentorium cerebelli extending to the left tempero-parietal meninges. The meningeal biopsy revealed markedly thickened fibro-connective dural tissue with infiltration of chronic inflammatory cells. There was no evidence of bacterial, fungal, tuberculous or neoplastic infiltration. IHP was diagnosed and steroid therapy initiated. Within weeks, she showed marked clinical improvement. IHP is a diagnosis of exclusion. The absence of underlying infective, neoplastic, or systemic autoimmune disease favors IHP. The above patient had headache, neuro-ophthalmic signs, seizures, which are features of IHP. However, superficial soft tissue involvement is rare.Journal of neurosciences in rural practice. 05/2012; 3(2):193-5.
Chapter: Central Nervous System Tuberculosis03/2012; , ISBN: 978-953-51-0200-7
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ABSTRACT: Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder characterized by localized or diffuse thickening of the dura mater, and it usually presents with multiple cranial neurophaties. It has been associated with a variety of inflammatory, infectious, traumatic, toxic and neoplasic diseases, when no specific cause is found the process is called idiopathic. The infectious cases occur in patients under systemic immunosuppression, which have an evident contiguous source or those who have undergone neurosurgical procedures. We describe a case of a 62-year-old immunosuppressed woman with diabetes and rheumatoid arthritis, which had HCP and osteomyelitis of the skull base caused by pseudomonas aeruginosa, presenting with headache and diplopia. We believe this is the second documented case of pachymeningitis secondary to this microorganism. As a multifactorial disease, it is essencial to determine the specific causative agent of HCP before making treatment decisions, and great care is needed with immunocompromised patients. KEYWORDS: Pseudomonas aeruginosa; Hypertrophic pachymeningitis; Ophtalmoplegia, optical neuropathy; Osteomyelitis; Skull base.Journal of Clinical Medicine Research 04/2012; 4(2):138-44.