Table 1 The spectrum of neurodevelopmental disabilities in adolescents and adults with HPE

Subjects	HPE type	Developmental disability category	VINELAND Adaptive Behavior Scales	Full Scale IQ or estimate range	ASHA swallow outcome scale	ASHA diet restrictions scale
Subject 1	MIH	Intermediate	–	Moderate ID	–	4
Subject 2	Semilobar	Severe	20–21	–	6	4
Subject 3^a	Semilobar	Severe	20–21	–	6	4
Subject 4	Semilobar	Severe	24	Profound ID	1	1
Subject 5	Semilobar	Severe	24	Profound ID	6	2
Subject 6	Lobar	Severe	–	–	–	2
Subject 7	MIH	Mild	53	Moderate ID	6	6
Subject 8	Semilobar	Severe	23	Profound ID	–	2
Subject 9	MIH^b	Intermediate	36	–	–	4
Subject 10	MIH	Mild	55	Mild ID	6	6
Subject 11	Septopreoptic^c	Mild	72	Average	6	6
Subject 12	Lobar	Intermediate	–	–	–	3
Subject 13	semilobar	Severe	–	–	–	1
Subject 14	Lobar	Intermediate	28	Profound ID	–	4
Subject 15	Alobar^d	Severe	–	–	–	2
Subject 16	Semilobar	Severe	–	–	–	3
Subject 17	Semilobar	Severe	–	–	–	2
Subject 18	MIH	Mild	–	–	–	6
Subject 19	Semilobar	Severe	–	–	–	2
Subject 20	Semilobar	Severe	–	–	–	2

ASHA, American Speech Language Hearing Association; HPE, holoprosencephaly; ID, intellectual disability; IQ, intelligence quotient; MIH, middle interhemispheric.
The WASI-II was administered to subject 10; the Mullen was administered to subjects 4, 5, 8, and 14; the WAIS-IV was administered to subjects 7 and 10. The WISC-IV was administered to subject 1, who was not able to complete the test. His score is based on the general ability index. Full Scale IQ or estimate ranges: average=average range (85–115); borderline=71–84; mild ID=55–70; moderate ID=40–55; severe ID=26–39; profound ID=<25.
ASHA diet restriction scale: 1=individual unable to swallow anything safely by mouth; 2=individual not able to swallow safely by mouth for nutrition and hydration; 3=individual taking less than 50% of nutrition and hydration by mouth; 4=swallowing is safe but moderate cues are required to use compensatory strategies, or the individual has moderate diet restrictions, or requires some gastrostomy-tube feeding; 5=swallowing is safe with minimal diet restriction and cueing, all nutritional needs are met per mouth; 6=swallowing is safe and the individual eats and drinks independently and requires cueing rarely. They may need to avoid specific foods or require additional time; 7=Swallowing is safe and efficient.
^aSibling of subject 2;
^bwith dysplastic cortex;
^cpatient was previously described by Hahn et al.;¹⁷
^dbased on a computed tomography scan.

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