Severe developmental delay and epilepsy in a Japanese patient with severe congenital neutropenia due to HAX1 deficiency.

Haematologica. 2007 Dec;92(12):e123-5

Authors: Matsubara K, Imai K, Okada S, Miki M, Ishikawa N, Tsumura M, Kato T, Ohara O, Nonoyama S, Kobayashi M

HAX1 deficiency has recently been identified as a cause of severe congenital neutropenia (SCN), but little is known about the phenotype. We described an SCN patient with a homozygous 256C-to-T transition causing an R86X mutation in the HAX1 gene. Notably, the patient has been complicated by epilepsy and severe delay of motor, cognitive, and intellectual development; each developmental quotient was 21-26 at 7 years old. Growth failure and dental development delay were also noted. Neurodevelopmental delay in this patient expands the clinical phenotype of HAX1 deficiency and suggests an important role of HAX1 on neural development as well as myelopoiesis.

PMID: 18055975 [PubMed - in process]

Sensory impairments and health concerns related to the degree of intellectual disability in people with Down syndrome.

Downs Syndr Res Pract. 2006 Sep;11(2):78-83

Authors: Määttä T, Kaski M, Taanila A, Keinänen-Kiukaanniemi S, Iivanainen M

The relationship between poor health and cognitive impairment is not fully understood yet. People with Down syndrome are prone to a number of health problems, including congenital heart defect, visual impairment, hearing loss, autoimmune diseases, epilepsy, early-onset Alzheimer's disease and intellectual disability. Our aim was to assess the impact of impaired health on cognitive performance in people with Down syndrome. A series of people with Down syndrome (n=129) were studied for their intellectual disability, sensory impairments and health concerns. The medical and psychological records of all persons with Down syndrome in the Intellectual Disability Service Register of Kainuu from 1970 to 2004 were analysed. The detected health issues were related to the individuals' cognitive levels. Visual impairment, poor dental health and acute neurological disease were associated with the severity of intellectual disability and ageing.

PMID: 17048801 [PubMed - indexed for MEDLINE]

A 50-year-old man with clubbing has seizures after a dental procedure.

Chest. 2005 Nov;128(5):3634-8

Authors: Maimon N, Chan RP, Cusimano MD, Faughnan ME

PMID: 16304324 [PubMed - indexed for MEDLINE]

The dental health of children with refractory epilepsy in a residential school.

Arch Dis Child. 2005 Dec;90(12):1318

Authors: Huyton M, Nutt J, Scheepers S, Hindley D

PMID: 16301567 [PubMed - indexed for MEDLINE]

Access to dental care in Alabama for children with special needs: parents' perspectives.

J Am Dent Assoc. 2004 Apr;135(4):490-5

Authors: Al Agili DE, Roseman J, Pass MA, Thornton JB, Chavers LS

BACKGROUND: Access to dental care and delivery of quality dental health services are important for children with special needs. The authors surveyed parents of children with special needs in Alabama to determine their perceptions of access and barriers to dental care for their children. METHODS: The authors sent a questionnaire to 2,057 parents of children aged 3 to 13 years with special needs--cleft lip and/or palate; cerebral palsy, or CP; spina bifida; or epilepsy/seizure disorders--who were listed in a database provided by Children Rehabilitation Services of Alabama. The authors conducted univariate and multivariate analyses to calculate odds ratios and 95 percent confidence intervals. RESULTS: The overall response rate was 38 percent (N = 714). Eighty-five percent of respondents reported that their children had received some form of routine dental care. However, 35 percent of respondents reported they had had problems finding dentists willing to treat their children. Among those with problems, significant barriers to dental care included their children's having Medicaid insurance, poor oral health or CP, as well as a shortage of dentists with training in the care of children with special needs. CONCLUSIONS AND PRACTICE IMPLICATIONS: While the majority of respondents said their children had access to dental care, one-third said their children had problems receiving this care. Many of these problems can be ameliorated. Increasing providers' participation in the Medicaid program and improving their knowledge about, empathy for and training in the care of children with special needs is essential in improving access to dental care for this population.

PMID: 15127874 [PubMed - indexed for MEDLINE]

Phenytoin-induced gingival overgrowth in un-cooperated epilepsy patients.

Yonsei Med J. 2004 Apr 30;45(2):337-40

Authors: Marakoglu I, Gursoy UK, Cakmak H, Marakoglu K

Phenytoin-induced gingival overgrowth is a well-known and frequently reported gingival lesion, which was first detected in 1939. However, there are conflicts in the literature about the agents which affect the severity of the lesion. Un-cooperative dental patients are one of the most unsuccessfully treated periodontal patient groups because of the difficulty in maintaining their oral hygiene. This case report consists of two cases with the same characteristics: phenytoin usage, comprehension and speech defects and poor oral hygiene, but each case differs in the duration of the phenytoin therapy. Both of the cases received scaling, root planning and a gingivectomy.

PMID: 15119009 [PubMed - indexed for MEDLINE]

[Intranasal and buccal midazolam in the treatment of acute seizures]

Rev Neurol. 2004 Mar 1-15;38(5):458-68

Authors: Armijo JA, Herranz JL, Pena Pardo MA, Adín J

AIMS: There are several personal and social problems involved in the administration of rectal diazepam that make it unsuitable for use in public places and by non medical workers, in children and especially in teenagers and adults. Intranasal and oral midazolam could be an alternative to rectal diazepam. We review the efficacy and safety of these ways of administering midazolam, which is already used in some countries as a sedative and as an anticonvulsive drug, despite the fact that it has not yet received authorisation. DEVELOPMENT: Intranasal midazolam (INM) was first used as a sedative in dental extractions, echocardiography, endoscopies or surgery, especially in children. After proving its efficacy electroencephalographically in patients with seizures, it started to be used to interrupt acute seizures. In three randomised trials, the efficacy of intranasal and oral midazolam in hospitalised patients was similar to, and even higher than, that of intravenous or rectal diazepam, with a similar speed of action and safety; no studies have been conducted, however, in the extra hospital milieu and its risk of respiratory depression may be like that of other benzodiazepines. One of the problems of using the parenteral solution for intranasal administration is the irritation that is produced by its acidic pH and the relatively large volume that has to be administered. These problems could be reduced by using aerosols containing a solution of midazolam in cyclodextrin, which accomplishes a greater concentration with a pH that is less acidic. Oral administration can be used in patients with nasal secretions or intense movements of the head. CONCLUSIONS: Intranasal or oral midazolam can improve the treatment of acute seizures in the hospital milieu and, more especially, in the extra hospital milieu when patients are attended by non medical staff. There is a need, however, for trials that prove its efficacy and safety in this situation.

PMID: 15029526 [PubMed - indexed for MEDLINE]

[Dental management of epilepsy patients]

Med Oral. 2003 Nov-Dec;8(5):384

Authors: Cutando-Soriano A

PMID: 14595266 [PubMed - indexed for MEDLINE]

The effect of electrical dental equipment on a vagus nerve stimulator's function.

J Am Dent Assoc. 2002 Dec;133(12):1657-64

Authors: Roberts HW

BACKGROUND: Dental patients who have epilepsy with pharmacologically refractory seizures may be treated with an implanted pulse generator that electrically stimulates the left vagus nerve. The pulse generator functions like a cardiac pacemaker. Some electrical dental devices have been shown to cause electromagnetic interference with the function of cardiac pacemakers. The potential effect of similar dental equipment on vagus nerve stimulators is unknown. METHODS: Common electrical dental devices were operated at maximum power settings in close proximity to a representative vagus nerve stimulator. The author assessed any interference of the dental devices with the nerve stimulator function by observing oscilloscope tracings. RESULTS: Under the conditions of this evaluation, none of the dental devices tested altered the function of the vagus nerve stimulator. CONCLUSIONS: Some commonly used electrical dental devices may be used in close proximity to patients who have implanted vagus nerve stimulators without adverse effects on the nerve stimulator function. CLINICAL IMPLICATIONS: Dentists and dental hygienists may encounter patients with implanted vagus nerve stimulators, and they need to be cognizant of developments in the treatment of epilepsy. Under the conditions of this study, use of common dental electrical devices did not alter the function of a vagus nerve stimulator. The findings of this study, however, should not be generalized to all types of electrical dental or medical devices, as a recent report indicates that treatment with diathermy devices is contraindicated for patients with implanted nerve stimulators.

PMID: 12512666 [PubMed - indexed for MEDLINE]

Occult maxillofacial trauma in epilepsy.

J Contemp Dent Pract. 2001 Nov 15;2(4):26-32

Authors: Aragon CE, Burneo JG, Helman J

Epilepsy is a relatively common neurological disorder with incidence in both developed and developing countries. Head, facial, and oral injuries can result from seizures experienced by the epileptic patient. Patients with severe epilepsy often experience other dental disease due to their inability to properly maintain their oral hygiene. This paper presents a case of a chronic mandibular fracture following an episode of seizures in a patient with epilepsy in whom the fracture was discovered when he developed a fistula in the submandibular region.

PMID: 12167918 [PubMed - indexed for MEDLINE]

Managing patients who have seizure disorders: dental and medical issues.

J Am Dent Assoc. 1995 Dec;126(12):1641-7

Authors: Sanders BJ, Weddell JA, Dodge NN

Some dental professionals may have concerns about treating patients who have seizure disorders. The authors contend that increased knowledge about seizures and how to manage them may make dental professionals more comfortable. This article reviews the various seizure types, discusses commonly used anticonvulsants and their side effects and outlines some special concerns dentists may have in providing care to these patients.

PMID: 7499665 [PubMed - indexed for MEDLINE]

Electroencephalography and evoked potentials: a PC-based analysis program for laboratory courses in physiology.

Am J Physiol. 1992 Dec;263(6 Pt 3):S16-22

Authors: Illert M, Wiese H, Wolfram U

A computer program (EEG Analysis) was developed for the preclinical laboratory course in physiology held for medical and dental students. It offers an off-line analysis of a set of typical and frequently occurring physiological and pathological electroencephalogram (EEG) and evoked potential (EP) recordings, which are stored in an IBM-compatible personal computer (PC) system. The users are requested to measure and analyze the data sets and to work through a base of questions relevant in the frame of the particular topic. The program is structured in several exercises: calibration, pickup of non-EEG signals (eye movements, chewing), waveforms in EEG recordings from awake subjects (alpha-waves, beta-waves), desynchronization of cerebral activity (visual activation, acoustic activation, mental activation), habituation of cerebral activity upon acoustic stimuli, EEG recordings from asleep subjects (different sleep stages, sleep-specific EEG signals), epileptic seizures, and EPs (principle of averaging, visually evoked potentials in different cortical areas). The program runs under MS-DOS and is network capable. The software structure ensures maximal flexibility for rapid changes and adaptations of the program to specific needs of a particular EEG course. The program has been used for three years, and the response from > 800 students has been consistently positive.

PMID: 1476209 [PubMed - indexed for MEDLINE]

Diazepam: its scope in anaesthetic practice.

Proc R Soc Med. 1969 Aug;62(8):806-7

Authors: Hollis DA

PMID: 5810290 [PubMed - indexed for MEDLINE]

Unforseen complications encountered during dental anaesthesia. Fits and faints.

Proc R Soc Med. 1966 Aug;59(8):734-8

Authors: Galley AH

PMID: 5911433 [PubMed - indexed for MEDLINE]