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.
Subject(s)
Kidney Neoplasms/diagnosis , Lambert-Eaton Myasthenic Syndrome/diagnosis , Wilms Tumor/diagnosis , Child, Preschool , Humans , Kidney Neoplasms/therapy , Lambert-Eaton Myasthenic Syndrome/therapy , Male , Wilms Tumor/therapyABSTRACT
Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. Our patients' cases highlight the underrecognized relationship between neurologic complications and linear scleroderma en coup de sabre and illustrate the importance of a thorough skin examination in patients with unexplained neurologic disease.
Subject(s)
Bell Palsy/complications , Epilepsy, Complex Partial/complications , Scalp Dermatoses/complications , Scleroderma, Localized/complications , Child , Child, Preschool , Disease Progression , Female , Humans , Male , Scalp Dermatoses/drug therapy , Scalp Dermatoses/pathology , Scleroderma, Localized/drug therapy , Scleroderma, Localized/pathologyABSTRACT
A 14-year-old girl presented with Complex Regional Pain Syndrome, Type I (CRPS-1) of the left ankle after a remote history of sprain. Allodynia, pain, temperature and color changes, and swelling were successfully treated with physical therapy, transcutaneous electrical nerve stimulation (TENS), gabapentin, amitriptyline, and tramadol. Five weeks later, she presented with a continuous, involuntary, intermittent coarse tremor of the left foot causing increased pain. The electromyogram showed rhythmic discharges of 3 Hz frequency lasting 20-80 milliseconds in the left tibialis, peroneus and gastrocnemius, suggestive of either basal ganglia or spinal origin. Tremor and pain were controlled with epidural bupivacaine, but the tremor reappeared after discontinuing epidural blockade. Carbidopa/levodopa 25/100 (Sinemet) was started and the tremor disappeared after two days. With continued physical therapy, pain and swelling resolved within two months and carbidopa/levodopa was discontinued after five weeks with no recurrence of the tremor. Our success in the treatment of CRPS-associated tremor in this young girl with carbidopa/levodopa suggests that this patient may have had underlying movement disorder which was unmasked by the peripheral injury.
