Possible induction of acute disseminated encephalomyelitis (ADEM)-like demyelinating illness by intrathecal mesenchymal stem cell injection.

We report a 27-year-old woman with an episode of encephalitis and optic neuritis, followed by autologous bone marrow mesenchymal stem cell transplants and possible induction of acute disseminated encephalomyelitis-like demyelinating illness.

Life-saving hemicraniectomy for fulminant acute disseminated encephalomyelitis.

Fulminant acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disorder, which most often occurs after an infection or vaccination. It frequently presents with focal neurologic signs and an altered sensorium. Patients often require critical care for airway management but are typically treated with medical therapy alone, including intravenous steroids and other immunotherapies. We present a case of dominant hemisphere fulminant ADEM in a patient who required neurosurgical intervention and a life-saving hemicraniectomy despite maximum medical therapy.

Decompressive craniectomy for acute disseminated encephalomyelitis.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare, acute demyelinating condition. Although it usually presents in an acute or subacute manner over days, its clinical course may be rapid with symptoms and signs of severe intracerebral mass effect secondary to cerebral oedema. METHODS: Case report and literature review. RESULTS: We report a case of a patient presenting with a hyperacute course manifested by rapid loss of consciousness and focal neurological signs. Management with emergency hemicraniectomy and steroids resulted in rapid neurological improvement and minimal long-term deficit. CONCLUSIONS: We believe that only surgical decompression is likely to be life saving in similar cases of hyperacute cerebral oedema due to ADEM. The wide decompression performed was concordant with that indicated for traumatic brain swelling. Such aggressive management is vindicated by the rapid recovery shown by our patient within days of surgery and the finding of minimal neurological sequelae at 3 months.

An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children.

PURPOSE: This study determined the statewide incidence and prevalence of acute disseminated encephalomyelitis (ADEM) and examined the course of three pediatric patients treated for tumefactive demyelination (TD) at the Blair E. Batson Children's Hospital. METHODS: Analyses of ICD-9-CM code hospital records and clinical database were conducted. RESULTS: From 2001 through 2007 the incidence in pediatric patients under 20 years was 0.4/100,000/year, with a prevalence of 8.6/100,000 during 2008. Three patients presented with TD. Case 1 had a 3-week history of ataxia and diplopia; case 2 presented with a sudden onset of coma, while the third child had a 4-month history of increasing lethargy and clumsiness in all extremities. Cerebrospinal fluid examinations were nondiagnostic. MRI examinations revealed asymmetric T2/fluid-attenuated inversion recovery hyperintensity within the pons (case 1), a large heterogenously enhancing temporal lobe mass, with extensive edema (case 2), and multiple small brain lesions with occasional ring enhancement (case 3). In case 1, intralesional MR spectroscopy demonstrated changes consistent with ADEM. Case 2 required intracranial monitoring, and medical treatment to control elevated ICP. Cases 2 and 3 underwent cortical biopsies that revealed ADEM. All three patients improved with corticosteroid therapy. At a minimum of 15 months follow-up, cases 1 and 2 showed resolution of deficits and MRI lesions, while the third patient demonstrated additional MRI lesions and increasing paraparesis. CONCLUSIONS: These cases demonstrate that appropriate neuroradiological evaluation, treatment of acutely elevated ICP, and brain biopsy can play critical roles in the management of children with undiagnosed ADEM and TD.

Decompressive hemicraniectomy for acute disseminated encephalomyelitis: case report.

OBJECTIVE AND IMPORTANCE: Acute disseminated encephalomyelitis (ADEM) varies widely in symptoms and severity. Some cases are associated with massive life-threatening cerebral edema refractory to conventional medical management. CLINICAL PRESENTATION: A 51-year-old woman with ADEM who developed severe brain swelling and herniation despite aggressive medical management is described. INTERVENTION: A decompressive hemicraniectomy and durotomy led to rapid improvement and an excellent outcome. CONCLUSION: This case report reinforces the place of this procedure in the armamentarium of treatment options for patients with medically refractory brain swelling and elevated intracranial pressure caused by ADEM. The potential for an increase in the incidence of ADEM with more frequent smallpox vaccinations emphasizes the significance of redefining the full range of management options for this treatable disease.