Robert D. Tien

Magnetic resonance imaging evidence of hippocampal sclerosis in progression: a case report.

Summary: A 32‐month‐old child presented in status epilepticus (SE) involving the left side of the body. Fast spin‐echo magnetic resonance imaging (FSE‐MRI) with hippocampal volumetry performed ≤24 h after the seizure showed increased T2 signal of the right hippocampus, but no atrophy. Complex partial seizures (CPS) appeared at age 33 months, and three more episodes of SE occurred between 33 and 37 months of age. Follow‐up FSE‐MRI at 34 and at 45 months of age demonstrated progressive hippocampal atrophy with resolution of the increased T2 signal. Her CPS became intractable and, at age 51 months, she underwent right temporal lobectomy. In the ensuing 5 months, she has had only one major motor seizure. This case demonstrates that acute increased hippocampal T2 signal intensity can occur soon after SE and hippocampal sclerosis (HS) may become evident within months in the setting of recurrent early childhood SE. This observation may support the hypothesis that early childhood SE can lead to HS. Furthermore, this case suggests that years of temporal lobe CPS may not be necessary for development of HS.

Fast Spin-Echo, Magnetic Resonance Imaging-Measured Hippocampal Volume: Correlation with Neuronal Density in Anterior Temporal Lobectomy Patients

Summary: To assess the value of magnetic resonance imaging (MRI)‐measured hippocampal volume in the detection of hippocampal sclerosis, we studied 28 patients undergoing anterior temporal lobectomy for medically intractable mesial temporal lobe epilepsy. Hippocampal volumetry and visual analysis of T2 signal change were performed using fast spin‐echo T2‐weighted MRI. Quantitative neuronal density measurements were performed in the resected hippocampal specimens. There was a significant correlation between MRI‐measured absolute hippocampal volume (AHV) and neuronal density in CA1, CA2, and CA3 subfields (p<0. 0001, p < 0.01, and p < 0.05, respectively). Differential hippocampal volume (side‐to‐side volume difference) failed to detect bilateral atrophy in three patients, but the bilateral hippocampal atrophy was recognized by considering AHV in these patients. This study suggests that MRI‐measured AHV can be of value in evaluating patients with mesial temporal lobe epilepsy, especially when there is no side‐to‐side difference in hippocampal volumetry.

Dural tail sign : a specific MR sign for meningioma?

Somewhat conflicting reports have appeared about the significance of linear meningeal thickening and enhancement adjacent to peripherally located cranial mass lesions on contrast enhanced magnetic resonance images. Some authors consider this finding nearly diagnostic of meningioma. In an attempt to determine the specificity of this so-called tail sign, particularly with respect to meningioma, we retrospectively reviewed 16 cases from institutional records. From our results, the tail sign appears to be highly suggestive but not specific for meningioma.

Successful treatment of childhood pilocytic astrocytomas metastatic to the leptomeninges with high‐dose Cyclophosphamide

Leptomeningeal dissemination of childhood pilocytic astrocytoma (PA) is a rare event with little information available regarding therapy. We report here four children with disseminated PA whom we treated with high doses of cyclophosphamide with clinical benefit. The patients were aged 2.5 to 8 years. Three patients presented with PA localized in the posterior fossa, initially treated with surgical resection (n = 3) and radiotherapy (n = 1). Leptomeningeal dissemination occurred at 32, 44, and 8 months from diagnosis, respectively. The fourth patient presented with an optic pathway tumor with leptomeningeal dissemination at diagnosis. At commencement of cyclophosphamide therapy, disease was present in the subarachnoid space (intracranial, n = 2; spinal, n = 4), cerebral ventricles (n = 2), and primary site (n = 3). Histology was identical at diagnosis and recurrence in the two biopsied cases and cerebrospinal fluid was negative in all cases. Treatment was with cyclophosphamide 4-5 g/m2/cycle given every 4 weeks for a total of two cycles (n = 1) and four cycles (n = 3). One patient achieved disease stabilization (duration 27 months at the time of publication) and three patients experienced significant reductions in tumor burden. Subsequent intrathecal therapy was administered to two patients. Two patients developed disease progression at 10 and 9 months from cessation of chemotherapy. The one re-treated patient responded to further, lower dose, cyclophosphamide. This is the first report of the use of high dose cyclophosphamide for disseminated PA. The recurrence of disease in two cases with a further response to lower dose cyclophosphamide has implications for the optimal duration of therapy for these low grade, aggressive tumors.

Gd-DPTA enhanced MRI in Bell's palsy and herpes zoster oticus: an overview and implications for future studies.

Magnetic resonance imaging (MRI) is a new and important tool for use in diagnosing and investigating diseases affecting the facial nerve. In recent gadolinium-DTPA enhanced MRI (Gd-MRI) studies it has unequivocally been demonstrated that ipsilateral facial nerve contrast enhancement, predominantly in the meatal portion, is present in both Bells palsy and herpes zoster oticus. In this overview, the results of MRI studies performed on patients with acute peripheral facial palsy, especially Bells palsy and herpes zoster oticus, are discussed. The Gd-MRI pattern in Bells palsy is very similar to that seen in herpes zoster oticus, and the findings reported so far support the theory that an inflammation may be the cause of the nerve injury in both cases. So far, however, Gd-MRI has not been helpful in evaluating the severity and/or prognosis of the facial palsy. Further studies employing improved techniques, including three-dimensional fast (or turbo) spin echo (3DFSE) MRI with heavily T2-weighted sections and high resolution three-dimensional Fourier transform (3DFT) MRI, need to be conducted in order to determine whether it is possible to follow the course of the disease and whether MRI and/or Gd-MRI are useful prognostic tools in the early stages of palsy.

Magnetic resonance imaging of AIDS‐related polyradiculopathy

AIDS-related polyradiculopathy is a syndrome associated with cytomegalovirus infection. We report two cases of AIDS-related polyradiculopathy in which spinal T1-weighted MRI with gadolinium-DTPA showed enhancement of the pial lining of the conus medullaris, cauda equina, and lumbar nerve roots. Both patients clinically improved with ganciclovir treatment.

MR Fat Suppression Combined with Gd-DTPA Enhancement in Optic Neuritis and Perineuritis

A fat suppression MR technique used in combination with Gd-DTPA enhancement was investigated to determine its value in cases of inflammatory optic nerve lesions. This technique, the so-called hybrid method, is a derivative of the chopper fat suppression technique and provides water-only images without increasing the imaging or postprocessing time. The study group consisted of four patients with acute visual loss, all of whom received Gd-DTPA. Conventional T2-weighted and fat suppression post-Gd-DTPA T1-weighted images were obtained in all patients; in addition, in one patient a post-Gd-DTPA T1-weighted image without fat suppression was obtained. In three patients, the conventional T2-weighted images failed to reveal any abnormality. In contrast, the enhanced optic nerve and enhanced perineural inflammatory infiltrate were easily identified on T1-weighted images after administration of Gd-DTPA and application of fat suppression technique. The lesions in inflammatory optic neuritis or perineuritis were easily distinguished from the surrounding fat, which had been suppressed. This combined technique resulted in significantly improved definition of normal anatomic structures and made the enhancing lesions more conspicuous, especially in areas with large amounts of fat such as the retrobulbar orbit.

Meningioangiomatosis: CT and MR findings.

Meningioangiomatosis (MA) is a rare hamartomatous lesion of the cerebral cortex; to date only 18 cases with imaging findings have been reported in the English literature. The origin of MA is probably malformative, with possible association with neurofibromatosis. These lesions frequently cause seizures in young patients. We report two new cases seen at our institution and present their CT and MR findings clearly illustrating MA cortex infiltration. Gd-DTPA used in one of the two cases failed to cause enhancement.

Spinal Dural Arteriovenous Fistula: Demonstration Using Phase Contrast MRA

Contrast angiography is the standard means of diagnosing spinal dural arteriovenous fistulas. We present a patient in whom a spinal dural arteriovenous fistula was demonstrated on phase contrast MR angiography.

MR findings in a patient with Ramsay-Hunt syndrome.

A case of Ramsay Hunt syndrome (herpes zoster oticus) was studied with Gd diethylenetriamine pentaacetic acid enhanced magnetic resonance imaging using a surface coil rather than a conventional head coil. This allowed us to demonstrate in detail the inflammatory changes of the multiple structures, involved.