www.lesswrong.com/posts/bshZiaLefDejvPKuS/dying-outside
2 corrections found
the world's longest surviving ALS patient at over 40 years since diagnosis
This overstates Hawking's record. Medical case reports published before this 2009 post documented ALS patients surviving 44 and even 49 years after diagnosis, so Hawking was not clearly the world's longest-surviving ALS patient.
Full reasoning
Published medical literature available before October 2009 already described ALS survivors whose documented disease duration exceeded 40 years.
- A 2001 Neuromuscular Disorders case report states: "We report on a patient ... with definite amyotrophic lateral sclerosis (ALS), and who is still alive 44 years after the initial diagnosis." That alone means Hawking was not uniquely established as the longest survivor merely by being "over 40 years since diagnosis."
- A 2005 Neurology case report goes further, reporting "a 73-year-old patient with a natural history of early-onset ALS for 49 years." That is longer than Hawking's roughly 46 years since his 1963 diagnosis as of 2009.
Because peer-reviewed case reports had already documented ALS survival of 44 and 49 years, calling Hawking "the world's longest surviving ALS patient" was inaccurate or at minimum unsupported by the published record available at the time.
2 sources
- A case of amyotrophic lateral sclerosis with a very slow progression over 44 years - PubMed
Abstract: We report on a patient whose neurological and neurophysiological findings fulfil the El Escorial criteria for definite amyotrophic lateral sclerosis (ALS), and who is still alive 44 years after the initial diagnosis.
- Early-onset ALS with long-term survival associated with spastin gene mutation - PubMed
Abstract: The authors report a 73-year-old patient with a natural history of early-onset ALS for 49 years presenting with limb and bulbar amyotrophy and a pyramidal syndrome.
ALS kills only motor neurons, which carry signals to the muscles.
The word "only" is inaccurate. Medical literature before this post already described ALS as affecting non-motor systems too, including cognitive and behavioral functions and overlap with frontotemporal degeneration.
Full reasoning
This sentence is too absolute. By the time this post was published in October 2009, peer-reviewed medical literature had already established that ALS is not purely limited to motor neurons in all cases.
- A 2007 Lancet Neurology review states that although ALS degeneration "predominantly affects the motor system," "cognitive and behavioural symptoms" had long been described, and that there is evidence ALS and frontotemporal dementia overlap clinically, radiologically, pathologically, and genetically.
- A 2009 consensus paper states that ALS is "increasingly recognized to be a multisystem disorder" that includes clinical and neuropathological features of frontotemporal lobar degeneration (FTLD).
So while motor-neuron degeneration is the core feature of ALS, saying it kills "only motor neurons" is incorrect. A more accurate formulation would be that ALS primarily affects motor neurons, but in some patients it also involves non-motor brain systems, including cognition and behavior.
2 sources
- Cognitive impairment in amyotrophic lateral sclerosis - PubMed
Abstract: Although the degeneration predominantly affects the motor system, cognitive and behavioural symptoms have been described for over a century, and there is evidence that ALS and frontotemporal dementia overlap clinically, radiologically, pathologically, and genetically.
- Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis - PubMed
Abstract: Amyotrophic lateral sclerosis (ALS) is increasingly recognized to be a multisystem disorder which includes both clinical and neuropathological features of a frontotemporal lobar degeneration (FTLD).