3-T magnetic resonance imaging simultaneous automated multimodal approach improves detection of ambiguous visual hippocampal sclerosis.

BACKGROUND AND PURPOSE: To evaluate if an automatic magnetic resonance imaging (MRI) processing system may improve detection of hippocampal sclerosis (Hs) in patients with mesial temporal lobe epilepsy (MTLE). METHODS: Eighty consecutive patients with a diagnosis of MTLE and 20 age- and sex-matched controls were prospectively recruited and included in our study. The entire group had 3-T MRI visual assessment of Hs analysed by two blinded imaging epilepsy experts. Logistic regression was used to evaluate the performances of neuroradiologists and multimodal analysis. RESULTS: The multimodal automated tool gave no evidence of Hs in all 20 controls and classified the 80 MTLE patients as follows: normal MRI (54/80), left Hs (14/80), right Hs (11/80) and bilateral Hs (1/80). Of note, this multimodal automated tool was always concordant with the side of MTLE, as determined by a comprehensive electroclinical evaluation. In comparison with standard visual assessment, the multimodal automated tool resolved five ambiguous cases, being able to lateralize Hs in four patients and detecting one case of bilateral Hs. Moreover, comparing the performances of the three logistic regression models, the multimodal approach overcame performances obtained with a single image modality for both the hemispheres, reaching a global accuracy value of 0.97 for the right and 0.98 for the left hemisphere. CONCLUSIONS: Multimodal quantitative automated MRI is a reliable and useful tool to depict and lateralize Hs in patients with MTLE, and may help to lateralize the side of MTLE especially in subtle and uncertain cases.

A novel Notch3 gene mutation not involving a cysteine residue in an Italian family with CADASIL.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary cerebrovascular disease leading to accumulating neurologic deficits and dementia. CADASIL has been linked to nucleotide substitutions and deletions in the Notch3 gene. All the mutations described until now lead to unpaired cysteine residue in the epidermal growth factor-like repeats. The authors report a family with CADASIL carrying a deletion in the Notch3 gene that did not involve a cysteine residue.

Cervical neuroma presenting as a subarachnoid hemorrhage: case report.

OBJECTIVE AND IMPORTANCE: The association of subarachnoid hemorrhage (SAH) with spinal lesions is well known, but hemorrhage from a cervical schwannoma is exceedingly rare. The histopathology and the mechanism of bleeding are discussed. CLINICAL PRESENTATION: We report a healthy 37-year-old man presenting with SAH after intense physical stress caused by bleeding of a cervical neuroma. INTERVENTION: A C6-T1 laminectomy disclosed an ovoid lesion, 4 cm in diameter; extremely dilated veins originated from the tumor. Removal of the spinal lesion resulted in immediate decongestion of the related venous network. The histopathological examination confirmed that the lesion was a telangiectatic schwannoma. The mechanism of bleeding of the intraforaminal cervical schwannoma is discussed. CONCLUSION: Telangiectatic neuromas may be a cause of occult SAH. The importance of magnetic resonance imaging of the cervical spine is emphasized to explain SAH with negative findings on four-vessel angiography in patients whose SAH may have a surgically correctable cause distant from the intracranial compartment.