An Introduction To Autism
Autism was first described in 1943 by the American child
psychologist, Leo Kanner. Autism is a type of neurodevelopmental disorder, and
usually appears within the first three years of a child's life. The hallmark of
autism is the lack of communication, and affected children have problems with
language, behavior, and social skills.
Autism is a lifelong condition, and its cause is unknown. Environmental and
genetic factors do contribute to the development of autism, but most children
with autism have normal health, with no medical reason for the autistic
symptoms. Autism is not caused by the parents.
HOW PREVALENT IS AUTISM?
It affects about 1 in 500 children.
It is much more common in males than females (4:1 ratio).
WHAT ARE THE EARLY SYMPTOMS OF AUTISM?
A baby who doesn't babble or gesture by the age of 12 months.
A baby who lacks eye contact with its mother by the age of 12 months.
A baby who resists being held or cuddled by its mother.
A baby who doesn't respond when its mother says its name.
A baby who appears to be deaf.
An infant who doesn't say single words by 16 months of age.
A toddler who doesn't say 2-word phrases by 24 months of age.
WHAT ARE THE CAUSES OF AUTISM?
There are many biologic causes, but none of them are unique to autism.
Genetic: There is a familial tendency for autism. There is a 3-8 percent
recurrence risk if a family already has one autistic child.
Syndromes: Fragile-X.
Medical Conditions: Tuberous sclerosis.
Prenatal Factors: Intrauterine rubella, and cytomegalic inclusion disease.
Postnatal Factors: Untreated phenylketonuria, infantile spasms, and herpes
simplex encephalitis.
HOW IS AUTISM DIAGNOSED?
Use the guidelines from the American Academy of Neurology.
Early diagnosis focuses on behavior. Early intervention is needed for
optimal care.
Two levels of evaluation are required: First, a general screening for
developmental problems or risk factors. Second, another evaluation is needed
to establish the diagnosis of autism.
Metabolic and/or genetic testing to rule out other conditions with
manifestations similar to autism.
Serologic studies, to see if a child has been infected with herpes
simplex, intrauterine rubella, or cytomegalic inclusion disease.
Hearing tests, to determine if the language delay is due to a hearing
problem. The two hearing tests used are: the behavioral audiometry test, and
the brainstem auditory evoked responses (BAER) test.
Neuroimaging, such as MRI, is performed if a structural brain lesions is
suspected.
WHAT IS THE TREATMENT FOR AUTISM?
There is no treatment or drug regimen which can cure autism. Autism is a
lifelong condition.
Early detection and early intensive remedial education and behavioral
therapy are the most important measures which need to be taken.
DENTAL CARE PROTOCOL FOR THE AUTISTIC CHILD:
Prevention of oral disease is very important for autistic children, and
repeated oral hygiene instructions are essential.
Autistic children rarely verbalize complaints about dental problems, and
periodic dental evaluations are therefore essential.
Autistic children are hypersensitive to loud noises, sudden movement, and
things that are felt. The dental treatment area should therefore be as free
from auditory and visual stimuli as possible.
Children with autism need sameness and continuity in their environment. A
gradual and slow exposure to the dental office and staff is therefore
recommended.
Dental appointments should be short.
A slow and step-wise approach to performing dental treatment is
recommended.
Instructions should be presented in clear, short, simple sentences.
The Tell-Show-Do method of behavior management is useful, along with
frequent positive reinforcement for acceptable behavior.
Inappropriate behavior should be ignored.
A recent article in Neurology presented guidelines for the
screening of autism in children, beginning as early as infancy. The article
listed a number of absolute developmental milestones which a child needs to
reach by a particular age:
Filipek PA, et al.: Practice parameter: Screening and diagnosis of
autism: Report of the Quality Standards Subcommittee and the Child Neurology
Society. Neurology. August 22, 2000 55(4):468-479.
Seizure Disorders In Children
Seizures occur when nerve cells in the body misfire.
Seizures are a common childhood neurologic disorder, because the young nervous
system is more susceptible to seizures than the adult nervous system. About 4
to 10 percent of children have experienced an unprovoked seizure, with no
recurrence. The vast majority of seizures cease spontaneously.
Recurrent seizures which are not correctable are considered epilepsy. Children
with epilepsy often have an underlying central nervous system disorder which
causes behavioral problems, as well as cognitive impairment.
SIGNS AND SYMPTOMS OF CHILDHOOD SEIZURES:
Seizures may present in differing ways - ranging from a brief staring
spell to a life-threatening major motor seizure.
An aura before a generalized seizure. A generalized seizure is one that
simultaneously arises in several parts of the brain.
Loss of consciousness.
Bodily muscle spasms and violent convulsive movements.
Temporary lack of breathing.
Bowel or bladder incontinence.
Increased salivation and perspiration.
Confusion after the seizure, followed by deep sleep.
CAUSES OF CHILDHOOD SEIZURES:
Genetic factors.
Bacterial meningitis or encephalitis.
Trauma to the head.
Childhood fever.
Brain tumor or stroke.
Disturbances of the body's metabolism or electrolytes.
EPILEPTIC SYNDROMES OF INFANCY AND CHILDHOOD:
Neonatal Seizures: They are epilepsies of undetermined origin, and have a
35% to 50% mortality.
Benign Focal Epilepsy: Genetically inherited. Onset of seizures between 3
and 13 years of age.
West's Syndrome, also called infantile spasms: The majority of children
have an underlying CNS disorder. The problem may be misdiagnosed as colic.
Over 90% of affected children are developmentally delayed.
Complex Partial Epilepsy: Occurs at any age.
Lennox-Gastaut Syndrome: Presents as generalized seizures.
Juvenile Myoclonic Epilepsy: Genetically inherited. Onset between 12 and
18 years of age. The hallmark is an early morning, myoclonic (brief jerk)
seizure.
Febrile Seizures: Strong genetic predisposition. Occurs in 3 percent of
children between the ages of six months and five years. One third of febrile
seizures are "complex," since they are either multiple, focal, or
prolonged.
Video Game Related Epilepsy: Uncommon.
Status Epilepticus: The grand mal is the most harmful type of status
epilepticus seizure. It is defined as more than 30 minutes of continuous
seizure activity, or two or more sequential seizures.
CLASSIFICATION OF CHILDHOOD SEIZURES:
Partial Seizures: Arise in a specific area of the brain (cerebral cortex).
Simple Partial Seizures (consciousness is maintained).
Complex Partial Seizures (consciousness is impaired).
Generalized Seizures:
Convulsive (consciousness impaired): includes generalized tonic-clonic and
grand mal seizures.
Non-convulsive (altered mental status): includes absence, myoclonic, tonic
and atonic seizures.
Unclassified Epileptic Seizures
DIAGNOSTIC TOOLS FOR CHILDHOOD SEIZURES:
EEG (electroencephalogram) is recommended for all children who experience
a first seizure (except for febrile seizure).
Blood Tests for all children who experience vomiting, diarrhea, or
dehydration.
Lumbar Puncture for children younger than 18 months who are suspected of
having meningitis or encephalitis.
MRI (magnetic resonance imaging) for children with:
Refractory seizures.
Significant cognitive or motor impairment.
Possible temporal lobe abnormalities.
DRUG THERAPY FOR CHILDHOOD SEIZURES:
Lorazepam is currently the drug of choice for treating status epilepticus
seizures.
All seizure medications have side effects.
MANAGEMENT OF STATUS EPILEPTICUS IN THE DENTAL OFFICE:
Treatment of this medical emergency begins with evaluating and managing
the "ABC's" - airway, breathing, and circulation.
Position the child to best manage ventilation, control the airway, and
prevent injury. Provide passive restraint to protect the child from injury.
Deliver oxygen via a face mask. The contraction of the diaphragm during
the tonic phase of the seizure may cause apnea and hypoxemia.
An intraoral suction device will be needed. The tongue of the child, along
with the increased airway secretions during a seizure may obstruct the
airway.
A recent article in Neurology presents practice recommendations
for the evaluation of the first nonfebrile seizure in children. Routine EEG
as part of the diagnostic evaluation was recommended in all such cases. The
EEG appears to be most valuable in predicting the recurrence for seizure,
and for classifying the seizure type.
Hirtz D, Ashwal S, Berg A, Betis D, Camfield C, Camfield P, Crumrine
P, Elterman R, Schneider S, Shinnar S: Practice parameter: Evaluating a
first nonfebrile seizure in children: Report of the Quality Standards
Subcommittee of the American Academy of Neurology, the Child Neurology
Society, and the American Epilepsy Society. Neurology 2000 Sepember 12;
55(5):616-623.
Cytomegalovirus Disease In
Childhood
Cytomegalovirus is the most commonly acquired birth
infection of infancy. It is now the major intrauterine infection associated
with congenital mental retardation and deafness.
Cytomegalovirus (CMV) is a member of the herpes viruses. It is a
double-stranded DNA virus. During initial infection of an infant, CMV enters
the epithelial cells of the salivary gland, resulting in viral shedding. This
virus also replicates in the kidney. CMV infection is for life.
PREVALENCE OF CYTOMEGALOVIRUS DISEASE:
Few people escape cytomegalovirus infection during their lifetime, since
CMV is very common in humans.
About 1 percent of all newborns are infected with CMV in the uterus, but
90 percent of these newborns have no symptoms of the disease.
The majority (90 percent) of children who are infected in utero appear
healthy, but they may show signs of CMV infection later in life. The other
10 percent of the infected children become extremely ill, and may die.
CLINICAL SIGNS OF CMV:
Usually, no signs of cytomegalovirus disease are present at birth.
Jaundice (hyperbilirubinemia).
Low number of platelets (thrombocytopenia).
Slow growth in utero.
Small head size (microcephaly).
Infection of the retina (retinitis).
Deafness.
Mental retardation.
MODES OF TRANSMISSION FOR CMV:
Person to person contact with someone who is excreting the virus through
saliva, urine, or nasal droplets.
Young children attending day care.
Indirect transmission via contaminated toys.
Feces (of infants).
From the placenta to the fetus.
Vaginal secretions.
Breast milk from an infected mother.
Organ transplant.
Blood or blood products.
Sexual transmission.
HOW TO PREVENT THE SPREAD OF CMV INFECTION:
The immune system is very important in controlling cytomegalovirus
infection.
When a fetus comes in contact with CMV, the infection may be limited due
to the immune defense systems of either the mother or fetus.
Women who work in day care or health care facilities should:
Use universal precautions.
Wash their hands after handling diapers.
Wash off all toys on a daily basis.
Avoid nuzzling infants.
Note that prenatal screening of women for CMV infection is not recommended
because is is inaccurate and not helpful.
TREATMENT FOR CMV INFECTIONS:
All of the drugs used for the treatment of cytomegalovirus disease affect
viral DNA replication.
Unfortunately, no drug produces a cure for CMV infection.
The drug of choice for managing cytomegalovirus disease is is ganciclovir.
This drug is deposited in the teeth, bone, and cartilage of infants and
children.
Another drug being used is foscarnet. It has been used for the treatment
of herpesvirus infections which are resistant to ganciclovir.
A recent article in Pediatrics investigated bacterial and
fungal contamination of toys found in neonatal intensive care units. The
study demonstrated that toys may be reservoirs for infection of infants.
Cleaning and decontaminating the toys found in an infant's environment may
be useful in controlling the spread of infection.
Davies MW, Mehr S, Garland ST, Morley CJ: Bacterial colonization of
toys in neonatal intensive care cots. Pediatrics. August 2000 106(2).
