Paraneoplastic motor neuronopathy and malignant acanthosis nigricans

Neuronopatia motora paraneoplásica e acantose nigricans maligna

A 40-year-old woman presented with rapidly progressive quadriparesis and severe weight loss. Examination disclosed global amyotrophy, absent deep tendon reflexes and malignant acanthosis nigricans (Figure). Neurophysiologic studies disclosed pure motor neuronopathy. Paraneoplastic screening with full-body CT/MRI scan revealed an intravesical mural lesion (Figure) and urethrocystoscopy showed low-grade papillary urothelial carcinoma of the urinary bladder. High-titer serum anti-Hu antibodies were identified. There was marked motor and dermatological improvement after lesion removal, combined with intravenous immunoglobulin and methylprednisolone.

Figure
Malignant acanthosis nigricans and paraneoplastic screening. (A,B) Symmetric hyperpigmented cutaneous lesions without mucosal involvement. Paraneoplastic imaging screening disclosing an intramural intravesical lesion (white arrow) in ultrasonography study (C), abdominal and pelvic CT (D) and T2-weighted (E) and post-gadolinium STIR (F) sequences in MR imaging of the abdomen and pelvis.

Dermatological signs are key diagnostic clues in the suspicion of underlying malignancies¹, including rarely urological malignancies², in the context of paraneoplastic motor neuronopathy³,⁴.

References

• ¹
  Silva JA, Mesquita KC, Igreja AC, Lucas IC, Freitas AF, Oliveira SM, et al. Paraneoplastic cutaneous manifestations: concepts and updates. An Bras Dermatol. 2013 Jan-Feb;88(1):9-22. https://doi.org/10.1590/S0365-05962013000100001
  » https://doi.org/10.1590/S0365-05962013000100001
• ²
  Sacco E, Pinto F, Sasso F, Racioppi M, Gulino G, Volpe A, et al. Paraneoplastic syndromes in patients with urological malignancies. Urol Int. 2009;83(1):1-11. https://doi.org/10.1159/000224860
  » https://doi.org/10.1159/000224860
• ³
  Sharp L, Vernino S. Paraneoplastic neuromuscular disorders. Muscle Nerve. 2012 Dec;46(6):841-50. https://doi.org/10.1002/mus.23502
  » https://doi.org/10.1002/mus.23502
• ⁴
  Mélé N, Berzero G, Maisonobe T, Salachas F, Nicolas G, Weiss N, et al. Motor neuron disease of paraneoplastic origin: a rare but treatable condition. J Neurol. 2018 Jul;265(7):1590-9. https://doi.org/10.1007/s00415-018-8881-0
  » https://doi.org/10.1007/s00415-018-8881-0

• Ethical statement: Full consent was obtained from the patient for the case report. This study was approved by our Ethics Institution.

Publication Dates

• Publication in this collection
  29 July 2019
• Date of issue
  July 2019

History

• Received
  22 Dec 2018
• Accepted
  11 Mar 2019