A PEDIGREE WITH PROGRESSIVE BULBAR PARALYSIS APPEARING IN SEX-LINKED RECESSIVE INHERITANCE

Abstract

The author examined one male patient born in 1895 who has been suffering from progressive bulbar paralysis since 10 years: he also found in the proband's male relatives 2 more cases with typical symptomes of this disease and 3 individuals with only finger tremor. The one patient found in the relatives of the proband was born in 1903 and the other in 1881. In these 2 cases the desease began with finger tremor in the age of about 40. Since the finger tremor is considered as an early sign of this disorder, the author thinks that the above-mentioned 3 individuals with only fingertremor may belong to either the patients in an early stage of the disorder or those of fruste form.
All the above-mentioned 3 typical and 3 related cases show homochronous heredity and homotypy and neither immediate occasions nor external causes are evident concerning their manifestation.
They manifest in sex-linked recessive inheritance as distinctly seen in their pedigree. The inheritance of this disorder is regarded as recessive by some authors (Lovell, Saller) and as dominant by others (Gates etc.).
On the other side it is generally believed that amyotropyic lateral sclerosis and spinal progressive muscle atrophy are related to progressive bulbar paralysis, and no pedigree has been known in these disorders which manifests in sex-linked recessive inheritance.
The pedigree here reported will be the first instance in which the sex-linked recessive inheritance is evidenced.