Wilms Tumor Presenting With Lambert-Eaton Myasthenic Syndrome

Abstract

Paraneoplastic syndromes may affect the central and peripheral nervous system of adults and children with cancer. Neurological symptoms may resolve with treatment of the underlying neoplasm. We report the case of a child with Wilms tumor who presented with generalized weakness, fatigue, ptosis, hypokinesis, dysarthria, urinary retention, facial diplegia, ophthalmoplegia, and autonomic dysfunction. Routine electrodiagnostic testing, including repetitive nerve stimulation, was normal. Clinical features and stimulation single-fiber electromyogram were consistent with a neuromuscular junction transmission disorder, likely Lambert-Eaton myasthenic syndrome. The child's neurological status returned to normal with successful treatment of the tumor.

More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma

“…These effects have been reported almost in all organ systems, most commonly endocrine, dermatologic, musculoskeletal, and neurological. 4 Paraneoplastic neurological syndromes rarely associate with Hodgkin lymphoma. It is projected that, after triggering by ectopically expressed antigens by the tumor, misdirected immune responses occur against antigens that are normally present in the nervous system.…”

“…1 Though rare; achalasia is reported in HL. 4,6 A recent case report from India points at a child with Hodgkin lymphoma who presented with achalasia cardia, and Holmes-Adie pupil occurring synchronously with the cancer. 3 He had pneumatic dilatation treatment twice, had better esophageal function afterwards but no recovery in bilaterally dilated pupils and his visual acuity remained the same despite chemotherapy.…”

Use of therapeutic plasma exchange for pediatric neurological diseases

“…115 There are an additional three pediatric patients with LEMS that were described in other case reports. [116][117][118][119] The reported cases of LEMs in patients ≤19 years old support the rarity of this disease in children. This makes it difficult to ascertain true prevalence and incidence in this population.…”

“…A 2014 observational study with associated literature review of LEMS included a total of 12 patients, whose ages ranged from 3 to 19 years, nine who were part of the literature review in addition to the three patients described in the study 115 . There are an additional three pediatric patients with LEMS that were described in other case reports 116‐119 . The reported cases of LEMs in patients ≤19 years old support the rarity of this disease in children.…”

Amifampridines are the Most Effective Drugs for Treating Lambert-Eaton Myasthenic Syndrome With a Focus on Pediatric Lambert-Eaton Myasthenic Syndrome

“…Excluding four patients aged 4–16 years with autosomal dominant LEMS due to synaptotagmin 2 mutation because this disorder is hereditary, 30 we identified 23 cases of acquired pediatric LEMS in the literature ( Table 1 ). 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 Four of these 23 cases were excluded because they did not meet the second criterion of a CMAP increase of ≥60% either after brief exercise or HRS in the RNS test. 13 The RNS test was not performed in three cases and was normal in one case.…”