Diffuse Sclerosis Follow-up

  • Author: Robert Stanley Rust Jr, MD, MA; Chief Editor: B Mark Keegan, MD, FRCPC   more...
 
Updated: Feb 3, 2012
 

Further Inpatient Care

The approach to inpatient management is as is indicated in Treatment.

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Inpatient & Outpatient Medications

  • Patients who have received intravenous methylprednisolone should generally receive oral methylprednisolone for a period of 6 weeks, during which time they are tapered from an initial daily dose to every-other-day doses over 3 weeks and then the remaining alternate-day dose is tapered over the ensuing 3 weeks. The initial dose is 2 mg/kg/d or a maximum daily dose of 60-80 mg/kg/d. This taper may be prolonged as needed for patients who demonstrate recurrence of such symptoms and signs of illness as may have resolved during intravenous treatment. The enteral doses may be administered by nasogastric tube or other feeding tubes if the neurologic condition of the patient does not permit the doses to be swallowed. Coadministration of antacids or of histamine (H2) blockers is prudent during the entire course of corticosteroid therapy.
  • Patients must be monitored for such possible complications of corticosteroid therapy as hypertension, gastrointestinal hemorrhage, hypokalemia, hyperglycemia, and opportunistic infection.
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Deterrence/Prevention

No known method of prevention or deterrence exists.

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Complications

Complications include cerebral herniation, inexorable progression of disease to death, development of pneumonia, sepsis, pulmonary embolization, skin breakdown and ulceration in individuals who are moribund, and the various complications due to corticosteroid administration noted above.

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Prognosis

  • Prognosis depends upon definition of illness. In the current state of knowledge, determining the duration of disease, rate of progression, and outcome from the strictly defined category of diffuse sclerosis (Schilder disease 1912 type) that includes approximately 9 cases is impossible. This is also true because potentially valuable therapies such as intravenous high-dose corticosteroids have not been subjected to carefully designed trials in these cases.
  • In those individuals who have been reported as having Schilder disease but whom Poser has placed into the category of transitional sclerosis, the outlook derived from 70 cases Poser reported in 1957 demonstrated mean duration of survival to be 6.2 years after onset (range 3 d to 45 y). Disease duration was less than 1 year in as many as 40% of cases, but survival times of at least 10 years were experienced in more than 23%. These cases are in all likelihood examples of an often aggressive form of multiple sclerosis, hence the survival of these individuals tends on average to be shorter than that experienced by most individuals with multiple sclerosis.
  • In those prepubertal cases with a good response to intravenous corticosteroids, and particularly those who are found to have smaller lesions in locations typical for acute disseminated encephalomyelitis (eg, cortical ribbon, deep gray nuclei, thalamus), a better prognosis may be found in some cases. Information concerning this group and the potential of corticosteroids for remediation of illness is limited. The authors have encountered a small number of patients with large lesions who have had recurrent bouts of illness that have proven difficult to prevent and for whom, despite survival times for as long as 5 years without many cumulative deficits, the prognosis remains guarded.
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Patient Education

For excellent patient education resources, visit eMedicine's Muscle Disorders Center. Also, see eMedicine's patient education article, Multiple Sclerosis.

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Contributor Information and Disclosures
Author

Robert Stanley Rust Jr, MD, MA  Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia School of Medicine; Chair-Elect, Child Neurology Section, American Academy of Neurology

Robert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

William J Nowack, MD  Associate Professor, Epilepsy Center, Department of Neurology, University of Kansas Medical Center

William J Nowack, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Electroencephalographic Association, American Medical Informatics Association, and Biomedical Engineering Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Richard J Caselli, MD  Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale

Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

B Mark Keegan, MD, FRCPC  Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee Consulting; Bionest Consulting fee Consulting

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