
evolution of a characteristic speech disorder in
juvenile cerebral lipidosis
juvenile cerebral lipidosis is being diagnosed with increasing frequency in
this country, especially in schools for the visually handicapped.  the
manifestations of rapidly developing loss of vision, seizures, muscular
rigidity, ataxia, retinitis pigmentosa and eeg abnormalities are well known in
this disorder.  a speech disorder described as a "lolling, stammering speech"
was mentioned by sjogren (1931) in his comprehensive description
of juvenile amaurotic idiocy, but this has not been emphasized in
recent descriptions.  this report, based upon an analysis of 5 cases,
describes the evolution of a characteristic form of speech pathology that
is invariably present in juvenile cerebral lipidosis.
the initial speech abnormality seen early in the disorder is a hesitancy of
speech and occasional repetition of words.  in time, dysarthria becomes
evident with slurring, nasality and impairment of phonation similar to that
seen in pseudobulbar palsy.  in addition to the dysarthria, a highly
characteristic perseverative speech appears which often has a close resemblance
to true stuttering.  this stuttering-like speech has been observed in 4 of the
5 cases at some time during the course of their illnesses.  as the speech
patterns deteriorate, the patient becomes less communicative and finally lapses
into mutism.  the speech disorder is often the most disabling aspect of this
syndrome.
