Posted by Daniel Rubio, Drexel University College of Medicine Class of 2014
“A man” obsessing over “bats”…
“A man” eating “bats”…
The patients above are displaying symptoms resulting from a disease known as Lytico-Bodig Syndrome (LBS), a neurologic disease resembling amyotrophic lateral sclerosis (ALS/Lou Gehrig’s Disease), Parkinson’s disease, and Alzheimer’s disease. The country with the greatest number affected is the US territory of Guam. In fact, between the 1940-1960 it was the leading cause of death among the Chamorro people, a tribe found on the island of Guam. The afflicted were usually between the ages of 25-40 years of age.
What’s up with the bats?
Although yet to be proven, it is believed that the high incidence of LBS is due to the consumption of fruit bat, a cultural delicacy on the island. These fruit bats feed on specific fruits containing high concentrations of an altered amino acid called beta-N-methylamino-L-alanine (BMAA). BMAA is a known neurotoxin and is believed to be the cause of LBS. BMAA is a protein building block that is incorporated into neuronal proteins to produce an abnormal form that creates clumps with neurons resulting in their dysfunction and death.
I don’t eat bat though?
I addition to the high concentrations found in the fruit bats on the island of Guam, multiple sources have been proposed leading to BMAA exposure within the United States. Certain bacteria in fresh and salt waters produce BMAA; and, fish and crustaceans will concentrate BMAA within their tissues when they consume the bacteria as part of their normal diet. It is believed that human consumption of fish and crustaceans in at-risk areas might increase the incidence of neurodegenerative diseases, like Alzheimer’s, Parkinson’s, and Lou Gehrig’s. This association has been seen in many areas within the United States, especially around the gulf regions and around large bodies of water. Click here for more details about this.
Am I more lytico or bodig?
Presenting symptoms exist along the continuum of lytico-bodig. Patients on the lytico spectrum present more like ALS/Lou Gehrig’s disease. These patients have muscle wasting/atrophy and accompanying weakness, paralysis of mouth and tongue, and an inability to swallow resulting in choking to death. Over time, paralysis involves the breathing muscles requiring mechanical ventilation to help the patient breath and to prevent choking on secretions. Lytico patients remain aware of their deterioration. The form of LBS is fatal in all cases.
On the other end of the spectrum, patients with bodig presentations look more like Parkinson’s disease and Alzheimer’s disease patients. Bodig patients present with “freezing” with progressive immobility with loss of starting purposeful movement and loss of spontaneous movement. Progressive dementia with loss of speech and irrational behavior, including violence and rapid fluctuations in mood, are common. Over time, patients are left in stiff and immobile postures with inability to speak and swallow.
How would I know if I had LBS?
Currently Lytico-Bodig syndrome is diagnosed based upon appropriate history and physical exam by a neurologist. Definite LBS is declared in post-mortem autopsy. However, there is research being done to develop rapid tests using cerebral spinal fluid analysis.
What can I do if I have LBS?
Treatment is mainly supportive as is based upon symptoms present, whether it’s Parkinson’s, Alzheimer’s, ALS, or a combination of the three spectrums of diseases. The more the symptoms resemble Lytico, the greater the mortality: in patients with predominantly lytico-type symptoms the disease is practically 100% fatal.