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| Discussion |
CLINICAL
PROFILE:
A 21-year-old male presented with convulsions and headaches for one year; giddiness and ptosis since six months; decreased hearing on the right side for three months and hypoesthesia of right half of the face for the same period. There was right sided sensory neural hearing loss and papilledema.
Blood and CSF studies were unremarkable.
Imaging: Plain and contrast enhanced MRI of the brain showed thickened dura as hypointense signal and vasogenic edema as hyperintense signals in paritooccipital region with loss of flow void of posterior part of sagittal sinus
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Fig. 1 |
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Post contrast images (T1 axial, T1 coronal and T1sagital) showed enhanced thickened dura
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Fig. 2 |
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MR venogram showed absence of filling of posterior part of sagittal sinus, torcula and right transverses sinus
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Fig. 3 |
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Right and left carotid angiograms showed thrombosis of posterior sagittal sinus, both transverses sinuses and the right internal jugular vein
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Fig. 4 |
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The diagnosis of hypertrophic pachymeningitis was arrived at after out the other causes such tuberculosis, Wegners granulomatosis, syphilis, rheumatoid arthritis
Hypertrophic pachymeningitis is a rare inflammatory disease but frequency of detection is increases with magnetic resonance imaging (MRI).
The diseases those are infrequently associated with infectious, neoplastic and autoimmune inflammatory disorders are:
Idiopathic cranial or spinal pachymeningitis
Intracranial hypotension
Spontaneous hypotension
Hypotension occurring after spinal fluid drainage
Infection
Lyme disease
Syphilis
Mycobacterium tuberculosis
Fungal infection
Cysticercosis
Human T-cell lymphotrophic virus I
Malignant external necrotizing otitis caused by
Pseudomonas species
Systemic autoimmune diseases and vasculitides
Wegener granulomatosis
Rheumatoid arthritis
Sarcoidosis
Sjo¨gren syndrome
Temporal arteritis
Malignancy
Dural carcinomatosis
Metastatic disease in adjacent skull
Meningioma
DIAGNOSIS:
Diagnosis is made by excluding all other causes (as mentioned in table) of hypertrophic pachymeningitis.
Patients with idiopathic hypertrophic pachymeningitis ( IHP) often present with chronic headaches, which can resemble chronic migraine with or without other neurological manifestations. Other neurologic symptoms of IHP include cranial nerve palsies (cranial nerves IV–VIII), cerebellar ataxia, seizures, and neuro-ophthalmic symptoms such as visual field loss, complete blindness, optic neuropathy, and increased intracranial pressure with papilledema
The neurologic manifestations are attributed to compression of cranial nerves at the skull base by enlarged dura mater resulting from the chronic inflammatory process. The ensuing headache may be due to focal meningeal irritation or, possibly, to localized arachnoiditis. Further documented neurologic complications of IHP include venous sinus thrombosis, obstructive hydrocephalus, and cerebral edema (as seen in our case) . The literature suggests an association of IHP with many interesting syndromes including Tolosa-Hunt syndrome (glaucomatous inflammation of the wall of the cavernous sinus resulting in painful ophthalmoplegia), cranial polyneuritis (especially with involvement of the 7th–12th cranial nerves), multifocal fibrosclerosis (chronic glaucomatous inflammation consisting of retroperitoneal fibrosis, Riedel thyroiditis, sclerosing cholangitis, and pseudo tumor oculi) and diabetes insipidus. Iso to hypointense on T1-weighted MR imagesand hypointense on T2-weighted MR images with without a hyperintense border Linear thickening of the falx and tentorium is the most common finding, and the next most common finding is focal nodular thickening that simulates a dural mass. Other associated findings include mastoid effusion, sinus abnormality, cavernous sinus involvement, white matter edema, and hydrocephalus. As in our case white matter edema is seen involving paritooccipital region.
Histopathological examination shows thickening of dura and pia matter due to the proliferation of the fibrous tissue.
Treatmant:
The current standard of treatment is intended to curb the inflammatory response that causes much of the morbidity associated with the disease. Although there
have been few verified clinical trials, the administration of systemic corticosteroids and immunomodulating agents such as azathioprine and cyclophosphamide
has been somewhat successful in the treatment of IHP. A recent study documented the successful use of intraventricular cytarabine in a patient who did not respond
to steroids or immunomodulating agents.
For a focal lesion which is causing significant mass effect surgical resection should be done but the recurrence rates are high.