Facial Involuntary Movements and Respiratory Failure in CANOMAD, Responsive to IVIG Therapy

Abstract

CANOMAD is a rare chronic neuropathy, characterized by chronic sensory ataxia and intermittent brain stem symptoms due to antidisialosyl antibodies. The disorder results in significant morbidity but is poorly understood and often misdiagnosed. We describe a unique case of CANOMAD, associated with involuntary movements of the face; patient reported exacerbations with citrus and chocolate and respiratory muscle weakness. Our patient was initially misdiagnosed with Miller Fisher Syndrome, highlighting the need for vigilance should neurological symptoms recur in patients initially diagnosed with a Guillain Barre variant. Moreover, the optimal treatment is unknown. This patient responded remarkably to intravenous immunoglobulin and has been maintained on this treatment, without further exacerbations.

Légzési elégtelenséggel járó CANOMAD szindróma

“…We report a case of CANO -MAD syndrome with severe respiratory failure followed by neuropathological evaluation. In another case described in the literature, acute respiratory failure was resolved with IVIg treatment 2 . Despite the stabilization of symptoms after IVIg administration in our patient, persistent severe respiratory failure required additional therapeutic steps (rituximab).…”

“…Earlier described CANOMAD cases reported patients with optic nerve involvement, extramembranous glomerulonephritis, and a syndrome of inappropriate antidiuretic hormone secretion (SIADH) 2,[11][12][13][14] . Cases without ophthalmoplegia, fur thermore, with temporary respiratory failure associated with facial involuntary movements were also mentioned 2,[11][12][13][14] . We report a case of CANO -MAD syndrome with severe respiratory failure followed by neuropathological evaluation.…”

“…C ANOMAD (chronic ataxic neuropathy ophthalmoplegia M-protein agglutination disialosyl antibodies) syndrome is a rare, chronic, immune-mediated demyelinating polyneuropathy 1 . About thirty cases can be found in the literature 2 . The syndrome is caused by the presence of a specific IgM paraprotein, which reacts with ganglioside-containing disialylated NeuAc(α2-8)NeuAC(α2-3)Gal neural epitopes 3 .…”