Parainflammator Leukoencephalomyelitis: Clinical and Magnetic Resonance Imaging Findings

Abstract

Parainflammatory leukoencephalomyelitis is a broad term used to include the spectrum of disorders that affect the central nervous system following infection, immunization, or other noxious stimuli. There is a wide range of clinical and pathologic severity, ranging from acute cerebellar ataxia to acute hemorrhagic leukoencephalopathy. With the improved survival of these patients, magnetic resonance imaging provides a window to the pathologic process, which can aid in the long-term management of these patients. Although lesions of the brainstem and spinal cord correlate well to clinical symptoms, multiple cortical lesions may be present without specific localizing signs. The distribution of magnetic resonance lesions is different from that commonly seen in multiple sclerosis. In some cases, prolonged immunosuppression may be required to prevent recrudescence of the inflammatory response.

Acute Cerebellitis Caused by Herpes Simplex Virus Type 1

“…Etiological diagnosis was made by PCR detection of HSV-1 DNA in CSF. The clinical courses and the MRI findings were similar for our patients but quite different from those described in cases of acute cerebellitis due to other causes, in which foci of edema or demyelination in cerebellar white matter were observed [7,24,25].…”

Treatment Leading to Dramatic Recovery in Acute Hemorrhagic Leukoencephalitis

“…Survival from the disease has been reported rarely. 1,2,4,5 Among those patients who have survived, high-dose corticosteroid therapy was universally used, and at least 1 case required decompressive craniectomy. 4 Surgical management and corticosteroids are not always successful, as evidenced by a recent report of a 16year-old girl with Epstein-Barr virus-associated acute hemorrhagic leukoencephalitis who died despite corticosteroid intervention and ventricular drainage.…”

Acute Cerebellitis in Children: A Variable Clinical Entity

“…Interestingly, both chickenpox and vaccination, both common causes of acute cerebellar ataxia, were rare causes of acute cerebellitis: only 2 children had chicken pox prior to acute cerebellitis and 3 cases were attributable to a recent vaccination. [17][18][19][20][21][22][23] Four cases were attributable to drug ingestions (3 cases of opiate toxicity and 1 case of ingestion of the tricyclic antidepressant amitriptylline). [24][25][26][27] When a prodromal illness was reported, the average time from prodromal illness to development of acute cerebellitis was 8.4 days (range 1-43).…”