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.
Article: Hypertrophic pachymeningitis.Annals of Indian Academy of Neurology 07/2011; 14(3):203-4. · 0.93 Impact Factor
Conference Paper: Value-Driven Coordination Process Design Using Physical Delivery Models.[Show abstract] [Hide abstract]
ABSTRACT: Current e-business technology enables the execution of increasingly complex coordination processes that link IT services of different companies. Successful design of cross-organizational coordination processes requires the mutual alignment of the coordination process with a commercial business case. There is however a large conceptual gap between a commercial business case and a coordination process. The business case is stated in terms of commercial transactions, but the coordination process consists of sequences, choices and iterations of actions of people and machines that are absent from a business case model; also, the cardinality of the connections and the frequency and duration of activities are different in both models. This paper proposes a coordination process design method that focusses on the the shared physical world underlying the business case and coordination process. In this physical world, physical deliveries take place that realize commercial transactions and that must be coordinated by a coordination process. Physical delivery models allow us to identify the relevant cardinality, frequency and duration properties so that we can design the coordination process to respect these properties. In the case studies we have done so far, a physical delivery model is the greatest common denominator that we needed to verify consistency between a business case and a coordination process model.Conceptual Modeling - ER 2008, 27th International Conference on Conceptual Modeling, Barcelona, Spain, October 20-24, 2008. Proceedings; 01/2008
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ABSTRACT: Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. It presents with headaches, cranial neuropathies and ataxia occurring alone or in combination. Dural biopsy is essential to exclude secondary causes of pachymeningitis. There is paucity of data on biopsied cases of HP. We report three biopsy-proven cases of idiopathic hypertrophic cranial pachymeningitis. All our patients had headaches and multiple cranial neuropathies; ataxia was seen in one patient. One patient had recurrent anterior and posterior cranial neuropathies, while one each had recurrent anterior and posterior cranial neuropathies. Two patients had profound irreversible mono-ocular visual loss. All of them showed prominent pachymeningeal thickening on imaging. Infarcts were seen in one patient, which have rarely been documented. All patients showed biopsy evidence of meningeal thickening and nonspecific chronic inflammation of the dura. The disease may have a remitting and relapsing course, and usually responds to steroids. Clinical improvement was excellent in two patients and modest in one on steroid therapy. All our patients required azathioprine during the course of therapy. Early institution and long-term maintenance of steroid therapy prevents neurologic sequelae. Occurrence of abdominal inflammatory pseudotumor in a patient of HP possibly as part of multifocal fibrosclerosis has not been described earlier.Annals of Indian Academy of Neurology 07/2011; 14(3):189-93. · 0.93 Impact Factor