Oral self-mutilation in a patient with rhombencephalosynapsys.
Severe mouth-focused self-biting in adults with ID may point to a rare cerebellar malformation—get the scan before you treat.
01Research in Context
What this study did
Doctors wrote up one adult who chewed his own lips and tongue until tissue was lost.
The man also had mild intellectual disability and a rare brain malformation called rhombencephalosynapsis.
No treatment or follow-up data were given; the paper only describes what they saw.
What they found
Severe oral self-injury can be the first clue that a hidden brain birth defect exists.
The team urges clinicians to order brain scans when adults with ID show mouth-focused self-harm.
How this fits with other research
Goodwin et al. (2012) remind us to define self-injury narrowly in ID; their review would label this case repetitive SIB, not general self-mutilation.
Boudreau et al. (2015) show opioid antagonists help about half of adults with severe SIB and ID, but the target paper gives no drug trial data, so the two pieces slot together: scan first, then consider meds.
Martin et al. (1997) tracked deaths in adults with severe SIB; their warning about respiratory risks adds urgency to the scan-and-treat path the 2000 case implies.
Why it matters
If you see an adult client who keeps biting mouth tissue, pause the behavior plan long enough to request neuroimaging. A quick MRI can reveal a fixable brain lesion and save months of trial-and-error treatment. Pair the scan with the SIB definition tools from Goodwin et al. (2012) and, if indicated, discuss opioid antagonists per Boudreau et al. (2015).
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02At a glance
03Original abstract
Rhombencephalosynapsis (RS) is a rare cerebellar malformation. Its essential features are the absence of the incisura cerebelli posterior, fusion of the cerebellar hemispheres, the absence of the velum medullare anterius and nuclei fastigii, and fusion of the dentate nuclei, which are shifted towards the mid-line. Clinically, affected patients present with signs of cerebellar and motor disturbances. The present report describes a new patient affected by RS. The subject first presented at the age of 22 years because of a psychiatric symptomatology which was characterized by obsessive oral self-mutilation associated with an intellectual disability. Objective evaluation documented dysmorphic features, while neurological examination showed only a slight truncal ataxia. The subject's IQ was 74 on the Wechsler Scale (verbal IQ = 79, performance IQ = 74). Psychiatric evaluation with DSM-IV criteria documented an obsessive-compulsive personality disorder associated with emotional instability and oral self-mutilation. The typical picture of rhombencephalosynapsis was evident on magnetic resonance imaging. Both chromosomal analysis and routine biochemical investigations were normal. The relationship between oral self-injurious behaviour and cerebellar malformations is discussed with particular regard to the behavioural aspects of cerebellar congenital pathology in affective disorders and in autism.
Journal of intellectual disability research : JIDR, 2000 · doi:10.1046/j.1365-2788.2000.00242.x