[W3] - CH4

Question 1

Intellectual Disabilities

Answer 1

A permanent condition originating sometime between birth and age 18.

Characterized by general intellectual functioning that is significantly below average (roughly an IQ of 70 or below), with concurrent deficits in adaptive behaviour.

Accounted for 0.19% of the total student enrolment in 2016.

A neuropsychological assessment is rarely needed; except when an intellectually disabled child had an unusual scatter of performance (with splinter skills [abilities that are disconnected from their usual context and/or purpose / ability to do a specific task that does not generalize to other tasks] well above the typical significantly below average range) – as an evaluation could help to identify relative skill/cognitive strengths relevant to intervention development.

Question 2

ADHD and Neuropsychological Deficits

Answer 2

The neuropsychological deficits associated with this disorder are inattention, poor response inhibition and/or impulse control, and executive dysfunctions.

9.4% had an ADHD diagnosis in 2016

Question 3

Autism Spectrum Disorders

Answer 3

Its diagnostic criteria include persistent deficits in social communication and social interactions; restricted, repetitive patterns of behavior, interests, or activities; symptoms present in the early developmental period; symptoms cause clinically significant impairment in social, occupational, or other important areas of current functioning; cannot be better explained by intellectual disability or global developmental delay.

Diagnosing autism typically requires a multidisciplinary team; of which a school neuropsychologist may or may not be a member.

The known neuropsychological processes often impaired in children with ASD include executive functions, attention, working memory, sensory-motor, and language.

Question 4

Children with Head Injuries who are Having Academic or Behavioral Difficulties

Answer 4

A Traumatic Brain Injury (or acquired/head injury) occurs when a sudden trauma causes damage to the brain. This injury can either be closed (the skull is not penetrated but the impact of the blow causes damage) or open (the object penetrates the skull and enters brain tissue).

TBI’s can be classified as mild (no or minimal consciousness loss, headaches, confusion, dizziness, blurred vision, ringing in the ears, bad taste in the mouth, lethargy, behavioural or mood changes, trouble with memory, attention, or thinking), moderate, or severe (same symptom pattern as the above accompanied with a headache that gets worse or does not go away, repeated vomiting or nausea, convulsions or seizures, an inability to awaken from sleep, dilation of one or both pupils of the eyes, slurred speech, weakness or numbness in the extremities, loss of coordination, and increased confusion, restlessness, or agitation).

Long-term neurocognitive deficits have not been associated with mild head injuries. But they have been associated with moderate to severe TBI and include problems with alertness and orientation; attention and concentration; intellectual functioning; language skills; academic achievement; adaptive behaviour/behavioural adjustment; problem solving; learning and memory.

The first few years after a TBI hold the most potential for functional change and remediation! Children with or without visible deficits should be monitored and may need to be re-evaluated more frequently than every three years (the standard for most special education children). Note that damage to a given brain region may not present in the same way across children due to secondary deficits related to axonal shearing, swelling of the brain, infections etc.

Question 5

Anoxia vs. Hypoxia

Answer 5

Anoxia is a complete absence of oxygen supply to organ tissues (including the brain).

Hypoxia is a decreased supply of oxygen to organ tissues.

They can be caused by various factors including drowning, strangulation, smoke inhalation etc. They can cause loss of consciousness, coma, seizures, or death. Their prognosis depends on the speed with which the child’s respiratory and cardiovascular systems can be supported and the extent of the associated injuries.

Even relatively minor hypoxia at birth can result in significant cognitive impairments in selective and sustained attention, receptive vocabulary in preschoolers, emergent math skills, overall cognitive and academic functioning, and social skills.

Question 6

Cerebral Vascular Accidents

Answer 6

One of the 10 major causes of death for children.

There are three major arteries that supply oxygen and nutrients to the cerebral cortex; the anterior cerebral artery (ACA), the middle (MCA), and the posterior (PCA).

• Ischemia (a blockage of the flow of blood) is the most common type of stroke in children, with cardiac disorders/heart disease being the most common cause.
• Haemorrhagic strokes (the rupturing or breaking of a blood vessel) are typically caused by trauma (e.g., TBI, shaken baby syndrome).
• Perinatal strokes are those which occur in utero, at birth, or within the first few months of life (sometimes as a consequence of prematurity or very low birth weight).

The long-term symptoms vary depending on the type of accident, the location etc.

Question 7

Meningitis

Answer 7

An inflammation of the lining around the brain and spinal cord that is relatively common in children and can be life-threatening.

Symptoms include severe headache, stiff neck, dislike of bright lights, fever/vomiting, drowsiness and less responsive/vacant, rash anywhere on the body, and possible seizures.

Moderate evidence suggests that surviving bacterial meningitis had a negative impact on cognitive abilities (approximately 5-point reduction in IQ, and 5 times more likely to be intellectually impaired) and development (developmental delay in the form of a 0.5 SD deficit).

Question 8

Encephalitis

Answer 8

An inflammation of the brain usually caused by viruses that occur perinatally or postnatally.

Acute symptoms include fever, altered consciousness, seizures, disorientation, and memory loss.

Under researched, but some suggestions that severely impacted children have a higher likelihood of exhibiting intellectual disability, irritability and lability, seizures, hypertonia, and cranial nerve involvement.

Question 9

Seizure Disorders

Answer 9

One of the most prevalent neurological diseases worldwide. Typically caused by complex brain disease.

There are approximately 30 different seizure types which vary in severity and effects.

A school neuropsychologist should monitor children with seizure disorders and evaluate them for neurocognitive strengths and weaknesses as needed.

Question 10

Cerebellar Astrocytoma

Answer 10

• Benign/Slow-Growing.
• Symptoms: Clumsiness of one hand, stumbling to one side, vomiting/headache.
• Treated with Surgical Removal.
• 20% of brain tumors (peak: 5-8)

Question 11

Medullo-Blastoma

Answer 11

• Cancerous/Malignant; can metastasize/spread along spinal cord
• Symptoms: Uncoordinated movements, lethargy, vomiting/headache.
• Treated with Surgical Removal AND radiation/chemotherapy.
• 10-20% of brain tumors - most common malignant paediatric tumor - (peak: 5 / in boys)

Question 12

Ependymoma

Answer 12

• Growth Rates Vary - located in ventricles and obstruct cerebrospinal fluid flow.
• Symptoms: Uncoordinated movements, vomiting/headache.
• Treated with Surgical Removal AND radiation/chemotherapy - cure rate varies.
• 8 - 10% of brain tumors.

Question 13

Brainstem Glioma

Answer 13

• Located in pons and medulla - essentially exclusive to children (large = symtpomatic)
• Symptoms: Double Vision, Facial Weakness, Difficulty Walking, vomiting.
• Shrunk with radiation/chemotherapy - 5-year survival rate low.
• 10 - 15% of brain tumors (peak: 6)

Question 14

Craniographynioma

Answer 14

• Located near pituitary stalk (close to vital structures)
• Symptoms: Vision Changes, Headache, Weight Gain, Endocrine Changes.
• Treated with Surgical Removal AND/OR radiation (radiation can affect cognition) - controversy over best approach - survival rates favorable.
• Rare = 4% of brain tumors (peak: 7-12)

Question 15

Neuromuscular Disease; Cerebral Palsy

Answer 15

A heterogeneous group of chronic movement disorders (not diseases), caused by faulty development in the brain structures that control movement/structure (the pyramidal or extrapyramidal tracts).

Possible causes include congenital brain malformations, genetic abnormalities, infections, high fevers in the mother during pregnancy, or injury to the foetus.

It is generally classified based on the type of movement disorder involved: spastic (stiff muscles), athetoid (writhing movements), or ataxic (poor balance and coordination).

Neuropsychologically it is characterised by specific impairments in nonverbal reasoning, attention problems, and visuospatial/visuoperceptual functioning.

Notably, children with CP struggle with motor movements which can make it difficult to carry out certain elements of school neuropsychological assessment. Therefore, assessments with accommodations can be used. Assessing baseline levels of functioning, , particularly in the areas of sensory-motor, visual-spatial, and academic achievement, is of great importance.

Question 16

Muscular Dystrophy Disorder and Subtypes

Answer 16

Congenital muscular dystrophy (CMD) refers to a group of disorders in which infants evidence muscle weakness at birth or shortly thereafter (in all muscle groups). Typically classified into one of 6 subtypes:

MMD/Myotonic:
- Intellectual disability (in its infant form), learning disabilities (in its juvenile form) - like ADHD and anxiety disorders. Affects a wide variety of muscle groups.
- Slow progression; across 50 to 60 years.

DMD/Duchene:
- Below average mean IQ (85); deficits in verbal fluency, reading, phonological processing, receptive/expressive language, verbal learning, attention, and working memory. Affects proximal muscle groups.
- Age of onset is between 2 to 6 years; with survival beyond the 30s being rare.

BMD; LGMD/Becker, Lam-Girdle:
- Below average verbal and nonverbal IQs (BMD). Affects limb girdle and proximal muscle groups.
- Age of onset is adolescence/adulthood; survival to mid-to-late adulthood.

FSH; FSHD/Facioscapulohumeral:
- No known neuropsychological correlates. Affects proximal, then distal muscle groups later. - Age of onset in the 20s.

SMA/Spinal:
- No known neuropsychological correlates. Affects proximal muscle groups.
- Age of onset in childhood; slow symptom progression through adulthood.

Question 17

Children with Central Nervous System Infection or Compromise - Asthma

Answer 17

The most prevalent health condition in American children. Some medications used in its treatment (Albuterol) can have effects on levels of arousal/attention, memory, motor steadiness, and visual-spatial planning.

Chronic asthma itself has been associated with deficits in academic achievement, executive functioning, processing speed, attention, visuospatial functioning, language, and learning and memory.

Question 18

HIV/AIDS (Human immunodeficiency/acquired immune deficiency)

Answer 18

Paediatric HIV/AIDS is often associated with deficits in academic achievement, impaired cognitive functioning, attentional difficulties, and behavioural and emotional disorders.

Treatment for AIDS also carries risk for cognitive deficits.

Question 19

Spina Bifida and Hydrocephalus

Answer 19

Spina bifida occurs due to the failure of the neuronal tube to fuse early in the course of gestation (three to six weeks).

Hydrocephalus is a medical condition that is characterized by the ventricles of the brain overfilling with cerebrospinal fluid, resulting in increased intracranial pressure. Hydrocephalus is not a disease by itself, but rather a symptom of some other physiological/congenital/perinatal disorder. The increase in pressure can inflate the head’s size and cause lasting damage to brain tissue compressed against the skull. A shunt can be implanted to drain the excess cerebrospinal fluid into the abdominal cavity (although approx. 50% fail with 2 years).

Deficits associated with spina bifida include core deficits in motor, timing, attentional processes, executive dysfunctions, and academic deficits in mathematics; hence these areas would also be monitored if a child had early-onset hydrocephalus.

Question 20

Juvenile Diabetes

Answer 20

Insulin-dependent diabetes mellitus (IDDM) destroys the cells within the pancreas that are essential to produce insulin.

Children with diabetes might have associated neurocognitive deficits in the areas of visual-motor, memory, and attention.

More specifically, visual-spatial abilities appear to be more adversely affected by early-onset diabetes (below 19), and language/memory/attention seem to be more adversely affected by late-onset diabetes (>19 years).

Educational progress in such children requires monitoring.

Question 21

Leukaemia

Answer 21

Acute lymphoblastic Leukaemia (ALL) is the most common malignancy in children. Treatment has a success rate of over 80%; but chemo and radiation can be toxic to the central nervous system, particularly in young kids.

A paediatric neuropsychologist can help oncologists determine the extent of the neurobehavioral outcomes relating to this treatment. Common cognitive impairments occur in areas like attention, executive functions, memory, and motor function. A school neuropsychologist can help such children maintain a sense of self-efficacy/connection to the school environment.

Question 22

End Stage Renal Disease

Answer 22

Associated with the following neuropsychological problems: intellectual impairments (lower cognitive performance and IQ scores), developmental delays in infants (motor and mental), memory disorders (impaired short-term memory and verbal learning problems), attentional dysfunction (impaired immediate span, slower reaction times, errors of impulsivity and inattention on tests of vigilance), and visuospatial and visuoconstructional problems (impaired two-dimensional construction, and impaired two-dimensional copying).

School Neuropsychologists can monitor and provide emotional support.

Question 23

Prenatal and Birth Neurodevelopmental Risk Factors

Answer 23

Prenatal Alcohol Exposure: Fetal Alcohol Spectrum Disorder;
- Relative strengths in auditory attention, verbal retention, and basic language functions.
- Relative weaknesses in overall general intellectual ability, executive functions, visual attention, verbal and nonverbal learning, motor functions, externalizing behaviours, and adaptive behaviours.

Prenatal Nicotine Exposure;
- Higher likelihood for ADHD or ADHD symptoms (but no causality evidence)
- Stronger evidence for conduct/externalising problems.

Prenatal Cocaine Exposure;
- Frequent premature and low birth weight births; smaller head size and shorter length.
- Subtle but significant deficits include difficulties with self-regulation, speed of information processing, and sustained attention.
Language and memory difficulties can persist into adolescence (potential confounding factors of other substance use/maternal neglect).

Prenatal Cannabis Exposure;
- Later executive function deficits (perhaps through fetal carbon dioxide exposure like in smoking/tetrahydrocannabinol exposure)
- Lower levels of reading/spelling achievement.

Prenatal Environmental Toxin Exposure (specifically, exposure to teratogens);
- Varies depending on stage/amount/duration of exposure – and genetic vulnerability of mother/foetus.
- Toxins include lead, mercury etc.

Prenatal Opioid Misuse;
- Visual-spatial deficits, impaired attentional and memory skills, and generalized frontal lobe dysfunction.

Low Birth Weight/Prematurity LBW;
- Associated with developmental delays, attention problems, behavioural difficulties, academic failure, and cognitive impairment. - Cognitive/motor functioning delays can be observed as early as 18-24 months.
- Extreme LBW in childhood: inattention and hyperactivity, internalizing and externalizing disorders.
- Extreme LBW in adolescence: inattention, hyperactivity, and oppositional behaviours.

Question 24

Children with Suspected Processing Weaknesses

Answer 24

Typically, children with learning problems are administered a psychoeducational evaluation prior to a school neuropsychological evaluation.

Generally, a processing weakness is defined as an ipsative score of at least 1.5 standard deviations below the average of their own other test scores and at least 1 standard deviation below the mean for a normative standardized group.

The purpose of a school neuropsychological assessment in this case is to determine the existence of any processing deficits, discuss the potential impact that may have on the learning potential of the child, and link appropriate educational interventions to the assessment data.

Question 25

Acculturation

Answer 25

The change in cultural patterns that result from the direct and continuous firsthand contact of different cultural groups.

[The Cultural-Language Interpretative Matrix (C-LIM) which is part of the Cross-Battery Assessment Software System (X-BASS) Online, is designed to classify cognitive ability tests based on degree of cultural loading and degree of linguistic demand. It can be a valuable tool when seeking to determine whether test results obtained from standardized testing are actually valid for a given examinee.]