Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy.
Sporadic late onset nemaline myopathy (SLONM) is a progressive myopathy of indeterminate etiology and poor outcome. If associated with a monoclonal gammopathy, SLONM carries a more unfavorable prognosis. Immunotherapy was unsuccessful. We report two HIV-negative SLONM/monoclonal gammopathy patients who improved following intravenous immunoglobulin (IVIg) treatment alone or in combination with immunosuppressant agents. This favorable response to treatment suggests that a dysimmune mechanism is operative in some SLONM individuals. We suggest that IVIg deserves initial consideration for SLONM therapy.
Milone
Margherita
M
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Katz
Amiram
A
Amato
Anthony A
AA
Soderland
Carl A
CA
Segarceanu
Miruna
M
Young
Nathan P
NP
Jones
H Royden
HR
D004359:Drug Therapy, Combination; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D017696:Myopathies, Nemaline / Q000175:diagnosis / Q000188:drug therapy*; D011379:Prognosis; D016896:Treatment Outcome
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents