When was congenital-analgesia (aka congenital insensitivity to pain), the inability to feel pain, clinically documented? How rare?

Question

When was congenital-analgesia (aka congenital insensitivity to pain), the inability to feel pain, clinically documented?

How rare is it really?

Is it hereditary? Or just a sporadic fluke mutation?

I know this condition sounds like it would be a lot of fun (since one doesn't feel pain), but this can be an extremely dangerous situation to be in since one can't feel when they have broken bones, have a life-threatening infection, feel the burns of a stove, and may engage in risky behavior, etc. (since they don't feel the consequences of pain). So what is the life expectancy of someone with congenital-analgesia?

Answer 1

When was congenital-analgesia (aka congenital insensitivity to pain), the inability to feel pain, clinically documented?

It was first reported in 1932 in this paper:

Dearborn, G. V. N. (1932). A case of congenital general pure analgesia. Journal of Nervous and Mental Disease, 75, 612-615. http://dx.doi.org/10.1097/00005053-193206000-00002

However, I tried reading it, the print is very faded and near-impossible to read.

How rare is it really?

1 in 125 million

From: Daneshjou, K. et al. (2012). Congenital insensitivity to pain and anhydrosis (CIPA) syndrome; a report of 4 cases. Iranian Journal of Pediatrics, 22, 412–416.

But in this paper on the epidemiology in Japan, it's estimated at 1 in 600,000–950,000

From: Haga, N., Kubota, M., & Miwa, Z. (2013). Epidemiology of hereditary sensory and autonomic neuropathy type IV and V in Japan. American Journal of Medical Genetics Part A, 161, 871-874.

Is it hereditary? Or just a sporadic fluke mutation?

It's hereditary:

Congenital insensitivity to pain with anhidrosis (CIPA), also known as hereditary sensory and autonomic neuropathy type IV (HSAN IV)

Kim, W., Guinot, A., Marleix, S., Chapuis, M., Fraisse, B., & Violas, P. (2013). Hereditary sensory and autonomic neuropathy type IV and orthopaedic complications. Orthopaedics & Traumatology: Surgery & Research, 99, 881-885.

So what is the life expectancy of someone with congenital-analgesia?

There's age distribution of the population in Japan:

It doesn't say life expectancy, but it seems that it won't be past 40 years.