OBJECTIVE Adapting classifiers for the purpose of brain signal decoding is a major challenge in brain-computer-interface (BCI) research. In a previous study we showed in principle that hidden Markov models (HMM) are a suitable alternative to the well-studied static classifiers. However, since we investigated a rather straightforward task, advantages from modeling of the signal could not be asse...

Three children with frontonasal dysplasia associated with tetralogy of Fallot are reported. All cases had true hypertelorism and a median nasal groove with absence of the nasal tip. There was no mental deficiency. The facial anomaly is a sporadic, non-genetic interference of the normal development of the face. This is the first report of frontonasal dysplasia associated with a cardiac defect. M...

We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of ...

291 Dear Editor-in-Chief, We have read the paper of Song et al. [1] on frontonasal dysplasia deformity. It was an interesting discussion of an image that depicts a boy with a nose deformity, and it was reported that no basal encephalocele was observed. We would like to point out that we had a similar case of a girl with the same nose deformity. As in the report by Song et al., our patient also ...

ing the duplication generates increased levels of EFNB1 transcript, compared to the normal chromosome. We also show that imbalance of ephrin-B1 between X chromosomes in a mouse model containing a hypomorphic Efnb1 conditional allele results in aberrant cell mixing of the cranial primordia during development and hypertelorism. Taken together these data suggest that cellular mosaicism for differe...

ing the duplication generates increased levels of EFNB1 transcript, compared to the normal chromosome. We also show that imbalance of ephrin-B1 between X chromosomes in a mouse model containing a hypomorphic Efnb1 conditional allele results in aberrant cell mixing of the cranial primordia during development and hypertelorism. Taken together these data suggest that cellular mosaicism for differe...

LEOPARD syndrome (LS) is a congenital developmental disorder and is an acronym for multiple lentigines, electrocardiographic conduction abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retardation of growth, and sensorineural deafness (1). These clinical features overlap those of Noonan syndrome (NS), and heterozygous germline PTPN11 mutations have been ident...

V.J. Rodrigues S. Elsayed B.L. Loeys H.C. Dietz D.M. Yousem BACKGROUND AND PURPOSE: Loeys-Dietz syndrome (LDS) is a recently described entity that has the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate. Its neuroradiologic manifestations have not been well delineated. We sought to describe the neuroradiologic features of LDS and to assess the manifest...

Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder with a predisposition to cancer. Features like basal cell carcinoma, odontogenic keratocysts, calcification of falx cerebri, bifid ribs, pits on palms and soles and hypertelorism are evident. A case of this rare disease seen on a 13 year old female patient is presented here, where multiple odontogeni...

We report a case of partial deletion of 9p with partial trisomy of 1q42 syndrome, which is a rare clinical and cytogenetic report. The dysmorphic features of the patient include microcephaly, plagiocephaly, trigonocephaly with metopic ridge, arched eyebrows, hypertelorism, down-slanting palpebral fissure, ptosis, blepharophimosis, unilateral left epicanthic fold, long eyelashes, low-set and pos...