The preserved speech variant: a subgroup of the Rett complex: a clinical report of 30 cases.
Some girls with autism and Rett-like regression keep their speech; this paper says they form a distinct, milder subgroup.
01Research in Context
What this study did
The team looked at 30 girls who had autism plus Rett-like traits.
Each child had lost some hand skills, showed hand-wringing, but could still speak.
Doctors wrote up each case to map out a milder form they called the preserved-speech variant.
What they found
All 30 girls fit autism criteria and also showed classic Rett signs.
Yet they kept usable words, so the group sits between classic Rett and autism.
The authors argue this mix is its own subtype inside a wider Rett complex.
How this fits with other research
Bauman (1991) first painted the picture of classic Rett; Wilkinson et al. (1998) now refines that picture by carving out a milder, talkative subgroup.
Wulffaert et al. (2009) later counted autistic traits across all Rett types and found they are common, backing the overlap M et al. saw.
Raslear et al. (1992) showed most Rett children lose speech and use only eye gaze; M et al. spotlights the minority who escape that fate.
Why it matters
If a girl has autism plus hand stereotypies and motor decline, but still speaks, think preserved-speech Rett.
Refer for genetic testing and tailor goals to her retained language; she may progress farther than classic Rett profiles suggest.
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02At a glance
03Original abstract
Thirty girls and young women, 6 from Sweden and 24 from Italy, are described in this paper. They ranged in age from 5 through 28 years. All but one of the cases met full symptom criteria for DSM-IV autistic disorder. However, they also showed many features of classic Rett syndrome (RS) as outlined by the Rett syndrome Diagnostic Criteria Work Group. All met the required 3 out of 6 main criteria and 47% also met both these and the required 5 out of 11 supportive criteria for RS variants as outlined by Hagberg. The course of the disorder was more benign than in classic RS, but all the girls were severely functionally impaired. There was familial clustering in a subgroup. It is concluded that these 30 cases represent a syndrome, similar and probably related, to classic RS. It is suggested that there is a spectrum of syndromes ranging from severe cases with classical presentation to considerably milder variants. We propose that, at the present state of knowledge, these conditions might be best categorized as subgroups of the "Rett Complex," in which classical RS and the preserved speech variant may be the most frequent.
Journal of autism and developmental disorders, 1998 · doi:10.1023/a:1026052128305