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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management

Clinical Management

ADEM is relatively uncommon and in many occasions spontaneously improves and is self-limiting. Therefore, no double-blinded placebo-controlled clinical trial to establish the best treatment or superiority of one treatment over the other exists. Most of existing literature on treatment of ADEM heavily stems from personal observations and experience, small care series, care reports, and certain retrospective studies. The existing therapies for ADEM, heavily, rely on immunosuppression, and once a diagnosis of ADEM is made, a neurologist should treat the patient with intravenous pulse corticosteroid therapy (usually methylprednisolone 10-30 mg/kg/ day for children less than 30 kg and 1000 mg iv daily for 5 days for those heavier than 30 kg). Some neurologists use intravenous dexamethasone (1 mg/kg/day) instead of methylprednisolone. Most neurologists follow this pulse steroid therapy with a tapering oral prednisone regimen.

Axial pre-contrast T1 MRI showing symmetrical hypodense lesion across the basal ganglia, the corpus callosum, and the internal capsule bilaterally

Fig. 7.5 Axial pre-contrast T1 MRI showing symmetrical hypodense lesion across the basal ganglia, the corpus callosum, and the internal capsule bilaterally

While most patients improve with treatment patients who fail to improve, should receive a course of plasma exchange. In a milestone clinical study, Weinshenker et al. [20] examined the efficacy of plasma exchange in treatment of patients with severe demyelinating diseases of CNS unresponsive to corticosteroids in the course of a randomized controlled crossover trial of genuine versus sham plasma exchange. The investigators performed this trial on a cohort of 22 patients who qualified. They detected that 42% of the patients have experienced moderate or significant progress of their neurologic status compared to the 6% of the patients in the sham therapy and the result of this study was statistically significant. This study included one patient with ADEM, and it practically paved the path for more common use of this procedure for treatment of severe demyelinating diseases of CNS. Various experts may do five or seven rounds of plasma exchange. Intravenous immunoglobulin (IVIG) has also been used for treatment of these patients. Plasma exchange has been superior over IVIG in some case reports and small studies. Usually failure of one measure leads to the initiation of the other.

Regarding AHLE, this is considered a medical emergency requiring aggressive immune suppression with combined pulse steroid therapy and plasma exchange or IVIG together with close continuous monitoring and medical and/or surgical treatment of increased intracranial pressure due to edema. AHLE is a grave disease but cases that are treated early and aggressively may recover with minimal deficits.

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