Abstract. Background: Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been. Summary: Idiopathic hypertrophic cranial pachymeningitis is a rare inflammatory disease with diffused involvement of the dura. Often, the definite diagnosis is. Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction.

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One cause of painful ophthalmoplegia. Support Radiopaedia and see fewer ads. Ann Indian Acad Neurol. Pachymeningitis cervicalis hypertrophica syphilitica.

Cases and figures Imaging differential diagnosis. Idiopathic hypertrophic cranial pachymeningitis: Coronal MR imaging showing diffuse dural enhancement and thickening, along with left parieto occipital edema.

Log in Sign up. The main clinical features were headache and cranial nerve palsies. About Blog Go ad-free.

N Engl J Med; A study of seven cases. Hypertropnic meningiomas Multiple meningiomas. Case 2 A year-old male came to ER with headache associated with vomiting since 2 weeks and 1 episode of right focal seizure.

Report of three cases. How to cite this article: Long term improvement of idiopathic hypertrophic cranial pachymeningitis by lymphocytapheresis.


Pathological examination in two autopsied cases revealed diffuse thickening of the dura, especially in the posterior part of the falx cerebri and the tentorium cerebelli. Many patients had mild to moderate elevation of C-reactive protein, and three patients had perinuclear antineutrophil cytoplasmic antibody. Published by Wolters Kluwer – Medknow. Neuroimaging studies revealed diffuse or localized thickening of the dura, and MRI findings were key to diagnosis of this disorder.

Please review our privacy policy. Pachymeningifis pachymeningitis typically cause progressive cranial nerve palsies, headaches, and cerebellar dysfunction.

Hypertrophic pachymeningitis | Radiology Reference Article |

Connective tissue diseases and the nervous system. Erdheim Chester disease Erdheim Chester disease. All patients were treated with corticosteroid and improved intially, but half of them experienced relapses. J Neurol Neurosurg Psychiatry ; Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis.

A case of chronic hypertrophic cranial pachymeningitis causing cranial polyneuropathy and unilateral central retinal vein occlusion.

Exact etiopathogenesis of this entity is still unknown, but pachymeninngitis is speculated to be an autoimmune phenomenon or occur as a direct result of infectious or infiltrative pathology. Churchill livingstone ; New York.

Idiopathic hypertrophic pachymeningitis.

MR imaging of the meninges. Microscopic examination of the dura showed dense pachymeningittis with inflammatory cell infiltration composed mainly of lymphocytes. P-ANCA positive Wegener’s granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: Hypertrophic pachymeningitis is a rare disorder of diverse etiology.

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Clinicoradiological spectrum and therapeutic options. Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. Case Reports Case 1 A year-old lady presented with headache and progressive painless loss of vision in both the eyes over a periods of 6 months.

Idiopathic hypertrophic pachymeningitis.

Discussion Hypertrophic pachymeningitis is a rare disorder of diverse etiology. When no cause is identified it is termed idiopathic hypertrophic pachymeningitis. Hypertrophic cranial pachymeningitis involving the pituitary gland: Presence of associated leptomeningeal enhancement or parenchymal abnormalities with the exception of brain edema should suggest an alternate diagnosis.

A case of posterior fossa hypertrophic pachymeningitis with hydrocephalus. Presse Med ; Neuropathological findings of six cases including hypdrtrophic autopsied cases are also presented.