TY - JOUR
TI - Acute Encephalitic Syndrome Induced by Scleromyxedema
AU - Magira, E.E.
AU - Malouchou, A.
AU - Karathanasi, V.
AU - Mavropoulou, N.
AU - Siempos, I.I.
AU - Vourlakou, C.
AU - Sykaras, A.
AU - Anastasiadis, G.
JO - The American Journal of the Medical Sciences
PY - 2020
VL - 360
TODO - 2
SP - 192-195
PB - Elsevier B.V.
SN - 0002-9629, 1538-2990
TODO - 10.1016/j.amjms.2020.05.018
TODO - aciclovir;  ampicillin;  ceftriaxone;  immunoglobulin;  immunoglobulin lambda chain;  thalidomide;  vancomycin;  immunoglobulin;  immunoglobulin G;  immunologic factor;  liothyronine;  thyrotropin;  thyroxine, adult;  artificial ventilation;  case report;  clinical article;  coma;  demato neuro syndrome;  drug dose increase;  drug megadose;  endotracheal intubation;  female;  fever;  headache;  human;  human tissue;  intensive care;  middle aged;  monoclonal immunoglobulinemia;  myalgia;  nausea;  neurological complication;  papular rash;  protein electrophoresis;  Review;  scleromyxedema;  skin induration;  unconsciousness;  acute febrile encephalopathy;  biopsy;  blood;  brain;  coma;  complication;  diagnostic error;  diagnostic imaging;  differential diagnosis;  infectious encephalitis;  intensive care unit;  pathology;  scleromyxedema;  seizure;  skin;  x-ray computed tomography, Acute Febrile Encephalopathy;  Biopsy;  Blood Protein Electrophoresis;  Brain;  Coma;  Diagnosis, Differential;  Diagnostic Errors;  Female;  Humans;  Immunoglobulin G;  Immunoglobulins, Intravenous;  Immunologic Factors;  Infectious Encephalitis;  Intensive Care Units;  Middle Aged;  Monoclonal Gammopathy of Undetermined Significance;  Scleromyxedema;  Seizures;  Skin;  Thyrotropin;  Thyroxine;  Tomography, X-Ray Computed;  Triiodothyronine
TODO - Dermato-neuro syndrome is a potentially fatal neurological complication of scleromyxedema consisting of fever, seizures, and coma. This is an overlooked scleromyxedema case of a 62-year-old female patient from 2-years ago. She was admitted to our ICU because of high fever, colloid speech, muscle ache, and nausea. Molecular methods in the cerebrospinal fluid for neurotropic viruses ruled out acute infectious encephalitis. Her thyroid hormones were within normal values while the serum protein electrophoresis confirmed the monoclonal gammopathy of immunoglobulin G lambda (IgG(λ)), known for the last 2 years. The subsequent bone-marrow biopsy excluded the development of multiple myeloma. The patient fulfilled fundamental diagnostic criteria of scleromyxedema (monoclonal gammopathy, normal thyroid function and the appearance of marked sclerosis and induration of the skin papules on the face, neck, extremities, and skin creases) presenting as dermato-neuro syndrome, which was histologically confirmed. She demonstrated a remarkable improvement after intravenous immunoglobulin treatment during the first 24 hours. Mimics of non-infectious acute encephalitis should include the clinical diagnosis of scleromyxedema, especially when patients present in the emergency department with acute fever, coma, and skin lesions of diffuse sclerodermoid and papular type. © 2020 Southern Society for Clinical Investigation
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