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A 67-year-old man was evaluated in the sleep clinic at this hospital
because of odd behaviors during sleep, daytime sleepiness, and apnea detected on
a home sleep study.
Three years before the current evaluation, the patient’s wife noticed that the
patient had vocalizations, including talking and yelling, while sleeping. He also
had thrashing movements while sleeping; he fell out of bed more than once and
inadvertently hit his wife during these movements.
Two years before the current evaluation, the patient’s wife noticed that the
patient had prolonged episodes of daytime sleepiness. During the episodes, he
appeared to fall asleep while sitting up and was unresponsive to shaking and loud
voices; several hours later, he returned to normal interaction without any intervention. The daytime sleepiness was intermittent and fluctuated in severity.
Four months before the current evaluation, the patient fell asleep while washing
dishes. This resulted in a fall and unstable C1 spinal fracture, which required
urgent surgical repair. After the surgical repair, melatonin and trazodone were
prescribed for insomnia, and abnormal movements during sleep abated. However,
vocalizations during sleep — including singing, talking, and expression of fear
and anger in the context of upsetting dreams — continued and were most prominent in the early morning hours.
The patient also had a motor neurologic syndrome that had started before the
onset of sleep symptoms, approximately 3.5 years before the current evaluation.
Symptoms included progressive gait imbalance with falls, hand clumsiness, hoarse
voice, and dysphagia. Serial neurologic examinations, performed over a 3-year
period by multiple neuromuscular specialists, revealed slow progression of generalized fasciculations and the development of bulbar hyperreflexia, postural instability, and hypophonia, although strength in the arms and legs remained intact.
Serial electromyography revealed progressive and widespread involvement of
muscles showing chronic reinnervation changes, including eventual involvement
of craniobulbar muscles (tongue and masseter muscles).
Case study Reference: Videnovic, A., Babu, S., Zhao, B., Reda H. M., & Linnoila, J. J.. (2022). Case 1-2022: A 67-Year-Old Man with Motor Neuron Disease and Odd Behaviors during Sleep. The new england journal of medicine. https://doi.org/10.1056/NEJMcpc2115844