Additional authors (in order):Samuel Dang, Brittany Harvey BA, CCRC, Jerrica Farias ARNP, Adam Hart, Natalie Tucker CNCT, R.EEG.T, Nichole Jones LPN, Raul Alsina BS, and Clifton Gooch MD
Institution name, city, state, country of additional authors: University of South Florida, Tampa, FL, USA
Background: ALS is a rare disease, with an incidence of ~ 2 per 100,000. Conjugal ALS is even rarer, with an estimated incidence of about 1 in 510,000 couples. We report here a married couple who concurrently developed ALS. Hypothesis: Environmental factors are thought to contribute to the pathogenesis of ALS. Examination of conjugal ALS cases may contribute to our understanding of the disease. Methods: We reviewed the medical records of a married couple who are being treated in our ALS clinic who concurrently developed ALS. Results: The wife is a 68 y.o. woman who developed left leg weakness in November of 2014. She subsequently became wheelchair bound. Neurological examination showed UMN signs in bulbar, cervical, and lumbar regions, and LMN signs only in the lumbar region. However, EMG showed diffuse LMN involvement. Thus, she met the el Escorial criteria for probable ALS, laboratory supported. Her husband was a 73 y.o. man who began to have proximal arm weakness in 2014. He then developed bulbar symptoms, eventually requiring a PEG tube placement. He had UMN and LMN signs in 3 regions, meeting the criteria for definite ALS. They were married for 40 years. CSF analysis in both patients was unremarkable. Infectious screen for lyme, West Nile virus, HIV, and syphilis was negative. They had no known toxic exposure and no significant head trauma, and they did not travel to Guam or serve in the military. There is no known family history of ALS otherwise. Discussion/Conclusions: There are 20 occurrences of conjugal ALS reported in the literature outside of Guam. To date, no consistent environmental factors have been found. Genetic analysis, when performed, showed no mutations. Conjugal ALS appears to be coincidental in nature.
Title: A case of conjugal amyotrophic lateral sclerosis