They were the only affected people with the disorder in their family.The fifth case report was a re-examination of the Swedish family reported in 1940 by Samuelson et al. The phenotypical variability rate among affected family members was found to be high. Additional details were revealed in this reexamination of the family; the proband of the original study (whom had been long dead) was found to not only suffer from the typical deafness-visual  impairment phenotype commonly associated with the syndrome, but they also had hallucinations and depression. 4 of their other family members had underwent therapy for anxiety and 1 of their family members had died as a result of suicide. Visual/ocular features included optic atrophy which resulted in progressively lower visual acuity among those with the condition and glaucoma, which was highly treatable. Relation with Wolfram syndrome
Although both conditions are caused by a mutation in the WFS1 gene, they have different phenotypical features and different inheritance patterns, for example; while Wolfram syndrome tends to follow an autosomal recessive inheritance pattern, Wolfram-like syndrome follows an autosomal dominant inheritance pattern. Symptoms are another example of the difference between Wolfram syndrome and Wolfram-like syndrome; intellectual disabilities, ataxia, anosmia, ageusia, and/or sleep apnea, alongside other cardiac and/or endocrine symptoms are features that, while present in some to most cases of Wolfram syndrome, are absent in those with Wolfram-like syndrome, onset of certain symptoms also differs between patients with Wolfram syndrome and patients with Wolfram-like syndrome. Hearing impairments arent present in every person with Wolfram-like syndrome either.
Prevalence
According to OMIM, around 15-20 cases from 4 affected families with Wolfram-like syndrome have been described in medical lirerature. All of the families studied were European, both by ancestry and by nacionality, the countries said families originated from were Denmark, the Netherlands, France, and Sweden, respectively. History
The disorder was seemingly first described in 1940 by Samuelson et al, their patients were 4 members from a Swedish family with an apparently never-described-before combination of autosomal dominant sensorineural deafness and optic atrophy. Said family was later re-examined in 2011 by Rendtorff et al
In 2006, Eiberg et al. described 4 affected members from a 3-generation Danish family, said individuals showed a phenotype similar to that shown by patients with Wolfram syndrome, the pair of symptoms associated with the familys unique disorder was inherited following an autosomal dominant manner. All individuals had progressive hearing loss starting in childhood and optic atrophy with onset in either childhood or middle age. 3 out of the 4 individuals were found to have irregular glucose regulation, 1 had reduced glucose tolerance, another one was found to have diabetes which was previously undiagnosed, and yet another one of the patients was found to have decreased function of the pancreas beta cells. 1 out of the 4 individuals had a diagnosed anxiety disorder as well as a sleep disorder.