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36 Cards in this Set

  • Front
  • Back
rates of MR are higher in (males, females)
males
What other things are correlated with the level of intellectual functioning?
life expectancy, sensory and neurologic impairments.
4 domains of the Denver development screening test:
1) Gross motor
2) Language
3) Fine motor-Adaptive
4) Personal-Social
Defn of developmental delay
Child cannot yet do something that 90% of children same age can do
Mild MR: IQ range
50-70
Adult mental age of 50-70 IQ
9-12 years
Adult functioning levels of Mild MR
Often marry and have children, but have difficult time coping.

Usually can achieve minimal levels of self-support.

Can live independently or in supervised housing.
T/F Mild MR is indistinguishable from normal children in early elementary school
T
Trisomy __ and __ are incompatible with life beyond infancy
13, 18
most common individual cause of MR
Trisomy 21
Majority of cases of trisomy 21 due to what?
maternal nondisjunction, advanced maternal age
Most common form of inherited MR
Fragile X
Sx of Prader-Willi
H30 Syndrome:

Hypogonadism
Hyperphagia
Hypomentia
Obesity
Characterized by cafe au lait spots and neurofibromas, MR coocccurs if there's a seizure disorder
Neurofibromatosis
Neurocutaneous syndromes: defn and 3 examples
Mendelian inheritance with single gene disorder.

Neurofibromatosis

Tuberous sclerosis

Sturge-weber
should be highly suspected in episodes of acidosis and changes in consciouness, neurological and developmental deterioration, and family histry
inborn errors of metabolism
Cause of homocystinuria
decrease cystathione beta synthetase activity, resulting in buildup of homocystine and methionine.
due to defective oxidative decarboxylation of branch chain AA
maple syrup urine disease
What is Tuberous Sclerosis?
rare multi-system genetic disease that causes non-malignant tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. A combination of symptoms may include seizures, developmental delay, behavioral problems, skin abnormalities, lung and kidney disease.
What is Adenoma Sebaceum?
misnamed cutaneous disorder consisting of angiofibromas that begin in childhood and appear clinically as red papules on the face.
Mentally retarded people are (more, less) likely to suffer from psychopathology than the general population
more
What are the most prominent psychiatric disorders in MR ppl?
1) Mood disorders

2) Schizophrenia

3) Stereotyped, self-destructive, and autistic behaviors with worsening MR.
How should MR patients with psych d/os be treated?
The full spectrum of interventions used in general population.
What is Sturge-Weber syndrome?
Aka encephalotrigeminal angiomatosis.

rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by proliferation of arteries of the brain, resulting in multiple angiomas that occur on the same side as the physical signs described above. As a consequence, arteriovenous malformations often form. Normally, only one side of the head is affected.
coprolalia: defn
involuntary swearing or the involuntary utterance of obscene words or socially inappropriate and derogatory remarks.
When does Tourette's syndrome present?
between ages of 2-16, mostly 7-8
How does Tourette's progress?
Starts as simple tics then moves to upper body and becomes more complex.

May present first with ADHD symptoms and Tics develop later.
How many patients demonstrate corprolalia? How does it develop?
About 1/3 of Tourette's patients show it.

Starts as small vocal tics like simple clicks or throat clearing, then progresses to (usually in adolescence) curse words.
How are OCD symptoms usually related to Tourette's?
Liek "Tics of the mind"

intrusive thoughts
compulsive arranging of objects
counting, washing, checking
What are transient tics?
Very common (5-10% of all people) typically present as blinking, sniffing, facial tics. Occur at times of anxiety, stress or boredom (watching TV).

They don't progress.
What are chronic tics?
Transient tics that last longer than a year.

May improve during adolescence.
T/F Family history is extremely important in Tourette's
T. 65-90% of cases have a positive history of either TS or chronic tics.
What types of drugs/activity exacerbate symptoms of TS?
DA agonists (methylphenidate), noradrenergic activity (stress, anxiety)
Tx of TS
Neuroleptics - cause DA blockade.

eg, Haldol in small dosease.

Now Clonidine and Guanfacine (noradrenergic blockade) are used because of complications of neuroleptics.
What are other commonly associated difficulties/psych problems with TS?
irritability and emotionally lability
learning problems
impulsivity, ADHD (up to 50% of TS patients)
Drug of choice for TS
Clonidine (α2 agonist)