Character changes from idiopathic cranial pachymeningoencephalitis.

A 66-year-old man with idiopathic cranial pachymeningoencephalitis was described. He suffered from left orbital pain, and character changes. He became short tempered, and was very attached to trifles. Two years prior to these symptoms, he had developed transient left abducent nerve palsy. Head MRI showed a thickening and enhancement of the dura mater on gadolinium-enhanced T1-weighted images, and high signal intensity lesions at bilateral frontal lobes predominantly in the white matter on T2-weighted images. Biopsies revealed microglial proliferation in the cerebral parenchyma, and mild lymphocytic perivascular infiltration. No evidence of intracranial infection was detected. We therefore treated him with methylprednisolone pulse therapy followed by oral prednisolone. His character became gradually normalized, and bilateral frontal lobe lesions seen on MRI disappeared. This is the first case to describe recurrent pachymeningoencephalitis with character changes, and symptoms were probably due to frontal lobe dysfunction.

Persistent primitive hypoglossal artery associated with proximal posterior inferior cerebellar artery aneurysm.

BACKGROUND: A persistent primitive hypoglossal artery (PPHA) is a rare anomaly. The association of PPHA with intracranial aneurysms of the artery has also been rarely reported. We surgically treated a case of PPHA associated with a ruptured saccular aneurysm at the proximal posterior inferior cerebellar artery (PICA). CASE DESCRIPTION: The patient was admitted because of subarachnoid hemorrhaging. Angiography and three-dimensional computed tomography (CT) angiography (3D-CTA) demonstrated a left PPHA entering the posterior fossa through the left large hypoglossal canal. The left vertebral artery was absent. A saccular aneurysm was found at the junction of the PPHA and the proximal PICA. 3D-CTA showed not only the aneurysm itself but also the anatomical relationship between the aneurysm and the surrounding structures. Therefore, 3D-CTA was very useful in planning the surgery. The neck of the aneurysm was clipped through a far lateral approach associated with a C1 laminectomy, because this case had a large posterior condylar emissary vein and the aneurysm was located just posteroinferior to the hypoglossal canal. CONCLUSIONS: A case of PPHA associated with an aneurysm at the proximal PICA is reported. This case not only had a large hypoglossal canal but also had a huge posterior condylar emissary vein in the large posterior condylar canal. Anomalous structures associated with PPHA are also discussed. Finally, 3D-CTA proved to be very useful in planning the optimal surgical modality around the lateral portion of the foramen magnum.