Critical Care Medicine-Neurologic Disorders>>>>>Inflammatory and Demyelinating
Question 4#

A 22-year-old Scandinavian female presents with acute symptoms of vision loss, headaches, fatigue, and leg weakness. She reports that her mother has a history of multiple sclerosis (MS). MRI demonstrates inflammatory lesions of the same age found in multiple areas. Despite various treatment modalities, the patient progressed to death 6 months later. 

Which of the following MS variant diseases is MOST likely?

a. Balo concentric sclerosis
b. Marburg variant
c. Schilder disease
d. Devic disease

Correct Answer is B

Comment:

Correct Answer: B

MS is an autoimmune central nervous system (CNS) demyelinating disease with a prevalence of 15 to 250 per 100 000 people. It is more common in females, especially of Scandinavian descent, with increased prevalence associated with geographical latitude extremes. MS typically has a relapsing and remitting course separated in time and CNS location. A number of MS variant diseases has been described. The Marburg variant is a fulminant form of MS, typically characterized as monophasic with widespread involvement at onset with rapid progression to death usually within weeks to months. Balo concentric sclerosis is a demyelinating disease that affects the white matter of the brain involving alternating concentric rings of myelin loss and preservation. Initially the prognosis was considered similar to Marburg variant, but now there are reports of patients surviving and even having spontaneous remissions. 

Neuromyelitis optica or Devic disease is a heterogenous inflammatory and demyelinating condition of the optic nerve and spinal cord. It has a relapsing and remitting course however with the discovery of a specific autoantibody NMO-IgG which targets astrocyte aquaporin-4, and it is now considered distinct from MS. Schilder disease or diffuse myelinoclastic sclerosis presents as pseudotumoral demyelinating lesions usually affecting children 5 to 14 years old. These intracranial lesions are often mistaken for tumors or abscess.

References:

  1. Karussis D. The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 2014;48- 49:134-142.
  2. Karaarslan E, Altintas A, Senol U, et al. Balo’s concentric sclerosis: clinical and radiologic features of five cases. AJNR Am J Neuroradiol. 2001;22:1362-1367.
  3. Nunes JC, Radbruch H, Walz R, et al. The most fulminant course of the Marburg variant of multiple sclerosis-autopsy findings. Mult Scler. 2015;21:485-487.
  4. Morrow MJW. Dean neuromyelitis optica. J Neuroophthalmol. 2012;32:154- 166.
  5. Garell PC, Menezes AH, Baumbach G, et al. Presentation, management and follow-up of Schilder’s disease. Pediatr Neurosurg. 1998;29:86-91.