Acute disseminated encephalomyelitis presenting as multiple cystic lesions

Abstract

A 23 year old right handed man presented with a left parietal syndrome two weeks after a non-specific upper respiratory tract infection. Other clinical examination was normal. Cranial MRI disclosed multiple supratentorial lesions predominating in white matter. T1 weighted images with Ga-DTPA contrast disclosed ring shaped enhancing hypointense lesions (figure, A, B). In axial T2 weighted images these lesions were hyperintense with slight surrounding oedema (figure, C). Antibody tests for HIV, cysticercosis, and toxoplasma were negative. Thoracic and abdominal CT was normal. Because of rapid clinical deterioration, steroid treatment was begun and surgical biopsy carried out. Histology showed a subacute myelonoclastic process with many macrophages and relative sparing of axons. Three weeks after admission, MRI showed regression of the T2 lesions ( figure, D) with almost total disappearance of T1 contrast enhancement and hypointensities ( figure, E). No additional lesions were seen one month and one year later and there was total disappearance of contrast enhancement and supplementary regression of hyperintense lesions. Clinically he had improved. One year after onset, he had a mild memory disorder, episodic dysarthria, and some sensory loss in his right hand. A DE RECONDO

Progressive cavitating leukoencephalopathy: A novel childhood disease

“…11 In our patients, the episodic nature of acute deteriorations, without evidence of preceding brain infections or obvious metabolic stress, differs from the chronic progressive course seen in the above conditions, with the exception of Leigh's disease, in which episodic deterioration also is common. The episodic clinical regression, increased lactate, and related biochemical abnormalities in several patients suggest a mt disorder.…”

Acute disseminated encephalomyelitis: A review of 18 cases in childhood

“…Well‐defined multifocal hyperintensities on T2‐weighted images throughout the white matter, spinal cord and grey matter are considered to be diagnostic of ADEM 3,4 . Large solitary white matter demyelinating lesions and multiple cystic lesions have also been described in the published literature 19,20 . Other techniques not widely available, but that are helpful in supporting the diagnosis of ADEM are gadolinium diethylenetriamine penta‐acetic acid (Gd‐DTPA) enhancement of the lesions and areas of increased activity on single photon emission computerized tomography 21,22 .…”

“…3,4 Large solitary white matter demyelinating lesions and multiple cystic lesions have also been described in the published literature. 19,20 Other techniques not widely available, but that are helpful in supporting the diagnosis of ADEM are gadolinium diethylenetriamine penta-acetic acid (Gd-DTPA) enhancement of the lesions and areas of increased activity on single photon emission computerized tomography. 21,22 Two children in the present series had abnormal CT scans, however, MRI of the brain was needed to confirm the diagnosis.…”

Postinfectious encephalomyelitis

“…Numerous reports followed, each highlighting an unusual imaging characteristic such as normal initial brain scan [23], normal scan followed by the development of lesions during clinical recovery [24], lesions in the deep gray matter [25] or thalami [26,27], a solitary lesion restricted to an area such as the brainstem [28], or the presence of multiple cystic lesions [29] or ring-enhancing lesions with mass effect [30].…”