Rohana Naqi

Meningioma-like lesions in Erdheim Chester disease

A 55-year-old female patient, a hypertensive, was admitted with generalized tonic clonic seizures, fever, cough, and drowsiness. Her symptoms had started 1 month back. Detailed work-up was done, and she was diagnosed to have diabetes insipidus. Her laboratory investigations showed hypernatremia with serum sodium level of 170, WBC count was 21.2, ESR was 94, and C-reactive protein was 20.5. Her chest X-ray showed osteopenia of visualized bone with patchy bony density at lateral ends of both clavicles. Radionuclide skeletal scintigraphy revealed diffusely increased tracer uptake over appendicular skeleton and left hemipelvis, with evidence of bony expansion and photon deficiencies more marked over the proximal one third of both femur and left Sacroiliac joint. She underwent brain MRI with gadolinium, which revealed multiple extra-axial masses along the bilateral cerebral convexities, intraventricular region involving occipital horn of left lateral ventricle, and along tentorium and falx. These were associated with mild surrounding edema causing effacement of adjacent cortical sulci and pressure on body of right lateral ventricle. These showed isointense signals on T1-weighted image and hypointense signals on T2-weighted images. Post contrast images showed intense homogenous enhancement of masses (Fig. 1). Based on these findings, a most probable diagnosis of meningiomatosis was suggested. To confirm the diagnosis of meningiomas, the patient was subjected to biopsy of lesion along the right cerebral convexity. A diagnosis of xanthogranulomatous lesion was made. Correlating with the clinical and radiographic features, these lesions were consistent with manifestations of ECD. She was put on azathioprine, thyroxine, desmopressin, esomeprazole. After a week, the patient was clinically stable, oriented, tolerating oral well, and her fever settled.

Posterior reversible encephalopathy syndrome.

OBJECTIVE To evaluate the Magnetic Resonauce Imaging (MRI) features in patients having Posterior Reversible Encephalopathy Syndrome. METHODS This is a retrospective study from 8th June 2005 to 26th July 2009. Twelve patients were included who were confirmed to have Posterior Reversible Encephalopathy Syndrome, per imaging and clinical follow-up. Two neuro-radiologists blinded to the clinical condition retrospectively reviewed each image. Standard sequences were unenhanced Fluid Attenuated Inversion Recovery (FLAIR), T1-weighted, T2- weighted images followed by diffusion-weighted imaging and contrast-enhanced T1-weighted imaging. The regions involved were recorded on the basis of these sequences. RESULTS Abnormal T2-weighted hyperintense signals (indicating vasogenic oedema) were consistently present in the parietal or occipital regions in 5 (41.6%), but other locations were also involved, including the deep white matter in 3 (25%), frontal lobes in 1, inferior temporal lobes in 1, cerebellar hemispheres in 1, and basal ganglia in 1 (8.3% each). On follow-up examination after 5-7 weeks, the patients showed marked improvement clinically and on neuro-imaging, and were discharged in a stable condition. After administration of gadolinium contrast, there was no area of abnormal enhancement in 11 cases and minimal enhancement was seen in 1 case. In our series, 3 patients had follow-up MRI examination which revealed the resolution of previously seen changes as well as the resolution of clinical symptoms. However, the diagnosis of Posterior Reversible Encephalopathy Syndrome was established in 9 other patients by resolution of clinical symptoms alone in 2-3 weeks. CONCLUSION Awareness of diverse clinical and radiographic presentation of acute Posterior Reversible Encephalopathy Syndrome is essential to avoid misdiagnosis and treatment delay. Moreover, the syndrome is reversible with prompt treatment and has good outcome. This case series confirmed clinical improvement and recovery in most patients within weeks.