Specific Visual Perceptual Problems in daily life in children

Specific visual perceptual problems

Any dysfunction in different components of visual perception may lead to problems in activities of daily living.

·         Attention

If child’s state of alertness or arousal is impaired, he/she may demonstrate behaviors of over attentiveness, under attentiveness or poor sustained attention.

Children who are over attentive are easily distracted by visual stimuli around them rather then attend to task at hand. Children who are under attentive may have difficulty orienting to the visual stimuli, may habituate quickly to a visual stimuli and may fatigue easily. A child with difficulty in area of selective attention demonstrates a reduced ability to focus on a visual target. The child is easily confused and therefore does not obtain specific information needed for the task.

·         Memory

Child with visual memory deficits has poor or reduced ability to recognize visual information and to store visual information in short or long term memory. Child may have good memory for life experiences but not for factual material and may fail to relate information to prior knowledge. Child may demonstrate poor ability to use mnemonic strategies for storage.

·         Visual Discrimination

If this area is affected child may demonstrate an inadequate ability to recognize, match and categorize. Child may have difficulties matching the same shape presented in a different spatial orientation or may confuse similar shapes.

·         Object/form vision

Children with form constancy problems have difficulty recognizing forms and objects presented in different sizes, different orientations in space, or when there are differences in detail.

This may result in difficulty recognizing letters or words in different styles of print or in making transition from printed letters to cursive ones.

Child with visual closure deficit may be unable to identify a form or object if an incomplete presentation is made. e.g. child may not be able to differentiate between pen and pencil on his desk if they are partially covered by paper.

Child with figure-ground problems may not be able to pick out a specific toy from a shelf, and may have difficulty sorting and organizing personal belongings. These children may have difficulty attending to a word on a printed page because they cannot block out other words around it.

·         Spatial vision

A child with position in space difficulties has trouble discriminating among objects because of their placement in space. They may show letter reversals past age of 8 yrs and may show confusion regarding sequence of letters or numbers in a word (e.g. was/saw)  or math problem. Spacing letters and words while writing on a paper may also be a problem. Child may show difficulty in understanding directional language such as in, out, on, under, next to, up, down, in front of.

Problem in depth perception can affect child’s ability to walk through space and catch a ball. Child may be unable to visually determine when the surface plane has changed and may have difficulty with steps and curves. Transference of visual spatial notations across to visual planes can make copying from blackboard difficult.

A child with diminished topographical orientation may be easily lost and unable to find his/her way from one location to next.

Visual perceptual problems in performance areas

Effects of visual perceptual problems may be subtle in nature, with no obvious disabilities. However, when the child is asked to perform a visual perceptual task, he or she may be slow, or unable to perform the task.

The child with visual perceptual deficits may show problems with cutting, coloring, constructing with blocks or other construction toys, doing puzzles, using fasteners, and tying shoes. In grooming, there could be difficulty in using a mirror to comb and style hair, applying toothpaste to the brush, donning doffing clothes, prostheses and orthoses; tying shoes, matching colors etc.

 ·         Problems in reading

According to Gibson (1971) different characteristics of printed (written) information necessary for reading are:

       Word’s graphic configuration

       Orthography (order of letters)

       Phonology (sound represented)

       Semantics (meaning)

Segmenting of written words in early reading needs a variety of skills. Child must be able to recognize individual letter symbols. It requires visual attention, memory and discrimination.

In the dysfunction there is interference with acquisition of sight vocabulary. Child has good language abilities but trouble processing written words.

Children with visual memory problems may be unable to remember the visual shape of letters and words.

Children with weakness of visual-verbal associative memory have difficulty establishing easily retrievable sound symbol associations. Children with difficulty with active working memory cannot hold one aspect of reading process in suspension while pursuing another component. The child cannot recall the beginning of sentence while reading end of it.

Children with visual discrimination deficit may not be able to recognize symbols and therefore may be slow to master alphabets and numbers.

Confusions over letters “p, q and g” and “a and o” as well as letter reversal may ensue, such as notorious differentiation between “b and d”.

·         Problems in spellings

Children who have strong sound-symbol association sense may make dyseidetic errors i.e.  Spellings words phonetically but incorrectly. Visual sequential memory is necessary for remembering the sequence of letter in a word.

·         Problem in handwriting

Children may have problem in correct letter formation, spelling, mechanics of grammar, punctuation and capitalization, and formulation of sequential flow of ideas necessary for written communication.

Child does not recognize error in his own handwriting, and may be unable to recognize letter in different prints and thus have difficulty in copying from different types of print to handwriting.

Child may show reversal of letters such as “m, w, b, d, s, c, and z”. Over spacing and under spacing might be there and staying within the margins could be a trouble. Also there is difficulty in adapting letter size to space provided in paper.

 ·         Problem in mathematics

There is difficulty in correctly aligning columns for calculation and thus incorrect answers. Worksheets with rows and columns may be disorganizing to children. Children with poor visual memory may have difficulty using calculator. Multiple step problems are difficult.

Visual Perception - what it exactly is?

Visual perception is the ability to interpret and use what is seen. Interpretation is a mental process involving cognition, which gives meaning to the visual stimulus. Visual perception could also be defined as total process responsible for the reception and cognition of visual stimuli. The visual receptive component is the process of extracting and organizing information from the environment and the visual cognitive component is the ability to interpret and use what is seen. These 2 components allow us to understand what we see, and are both necessary for functional vision.

Visual perceptual skills include recognition and identification of shapes, objects, colors, and other qualities. With the help of visual perception a person makes accurate judgments of size, configuration and spatial relationships of objects.

The interactions of visual-receptive and visual-cognitive components help in the following functions:

·         Respond and adjust to retinal stimuli (anatomic and physiologic integrity)

·         Move both the head and eyes to collect raw data (occulomotor and vestibular control)

·         Effectively interpret visual information (visuo-perceptual ability)

·         Respond to visual cues through efficient limb movements (visuo-motor ability)

·         Accomplish integration of all these abilities.

The components of visual perception could easily be explained with the following flowchart:

Visual- receptive components include fixation, pursuit and saccadic eye movements, acuity, accommodation, binocular vision and streopsis and convergence and divergence.

• Visual fixation is fixing one’s gaze on anything. This and movement of eyes is done with the help of 6 extra-ocular muscles. Two types of eye movements are used to gather information from environment: pursuit eye movement (tracking) and saccadic eye movements (scanning). Visual pursuit involves the continued fixation on a moving object while Saccadic eye movements are rapid change of fixation from one point to another in visual field.

• Acuity – capacity to discriminate fine details of objects in the visual field.

• Accommodation – ability of each eye to compensate for a blurred image. It is the process used to obtain a clear vision i.e. focus on an object at varying distances. The internal ocular muscle (ciliary muscle) contracts and causes a change in crystalline lens to adjust for objects at different distances.

• Binocular fusion – ability to mentally combine images from 2 eyes into single percept.

• Streopsis – binocular depth perception or 3-D vision.

• Convergence or divergence – ability of both eyes to turn inwards towards the medial plane and outwards from the medial plane.

Visual cognitive components include visual attention, visual memory, discrimination and integration of visual stimulus with other sensory modalities.

·         Visual attention is focusing on one part of the visual field while ignoring others. The 4 components of visual attention are alertness, selective attention, vigilance and shared attention. Alertness reflects natural state of arousal. Alerting is transition from an awake to an attentive and ready state needed for active learning and adaptive behavior. Selective attention is ability to choose relevant visual information while ignoring less relevant information; it is conscious focused attention. Visual vigilance is conscious mental effort to concentrate and persist at a visual task. Divided or shared attention is ability to respond to 2 or more simultaneous tasks.

o   Deficit in the area of visual attention in a child is usually manifested as easy visual distractibility. He/she would not be able to focus on one object in environment and would get distracted while trying to focus on everything at one time. He will find it hard to maintain his gaze on the task at hand and would consistently look at other objects without staying at one for long.

·         Visual memory: it involves integration of visual information with previous experiences. Long-term memory is the permanent storehouse which has expansive capacity; while short term memory can hold a limited number of unrelated bits of information for approximately 30 seconds.

o   A child having deficit in this area will have difficulty in comprehending long sentences as he tends to forget the initial words of the sentence read by the time he reaches the end of sentence. Also copying from blackboard is troublesome for him as he ll forget what was read in the time it took him to read what’s written and shifting gaze from blackboard to copy.

·         Visual discrimination: ability to detect features of stimuli for recognition, matching and categorization. Recognition is ability to note key features of a stimulus and relate them to memory. Matching is ability to note similarities among visual stimuli. Categorization is ability to mentally determine a quality or category on which similarities or differences can be noted.

§  Object (form) perception

                                            i.  Form constancy – recognition of forms and objects as the same in various environments, positions and sizes. It helps a person develop stability and consistency in visual world. It enables a person to recognize objects even with differences in orientation or detail, and to make assumptions regarding the size of an object even though visual stimuli may vary under different circumstances (a school age child can identify the letters whether they are in type, written in many script, cursive, italics or written in upper or lower case letters).

                                          ii.  Visual closure – identification of forms and/ or objects from incomplete presentations. It enables person to quickly recognize objects, shapes, and forms by mentally completing the image or by matching it to information previously stored in memory (a child working at his/her desk is able to distinguish a pencil from a pen, even though both are partially hidden under some papers)

                                        iii.  Figure-ground – the differentiation between fore ground and background forms and objects. It is ability to visually attend to what is important; separating essential important data from distracting surrounding information.

·         Spatial perception

                                            i.  Position in space – determination of spatial relationship of figures and objects to oneself or other forms and objects. It is important to understand directional language concepts such as in, out, up, down, in front of, behind, between, left and right. It provides ability to differentiate letters, and sequences of letters in a word or in a sentence (child knows how to place letters equal spaces apart, touching the line; he/she is able to recognize letters that extend below the line such as “p,g,q,y”)

                                          ii.   Depth perception – determination of relative distance between objects, figures, or landmarks and the observer and changes in planes of surfaces.

                                        iii.  Topographical orientation – determination of location of objects and settings and route to the location.

·         Visual Imagery : also called visualization. It is ability to “picture” people, ideas and objects in the minds eye, even when objects are not physically present. Visual-verbal matching provides foundation for reading, comprehension and spelling.

ADHD - Attention Deficit Hyperactivity Disorder

ADHD, also Known as AD/HD is the acronym for Attention Deficit Hyperactivity Disorder. It is also known as hyper-kinetic disorder. It is a Psychiatric disorder or a neuro-behavioural disorder. It is characterized by problems of inattention, impulsiveness and/or hyperactivity. 

Based on these symptoms ADHD is classified in three subtypes: 

• predominantly inattentive 
• predominantly hyperactive-impulsive 
• combined type (having all three difficulties)

To make a diagnosis of ADHD the above-mentioned symptoms should emerge before the child reaches age of 7 yrs. 

The prevalence of ADHD in India has been reported to be about 5.2 - 9%.  ADHD is twice as common in boys as in girls. 

A school going child suffering from ADHD would show following symptoms:

• Easily distracted - will face difficulty in maintaining focus on task at hand and his attention would be drawn to different sights and sounds in the environment. 
• Doesn’t want to give or maintain eye contact. Although it’s not a criteria for ADHD, but affected child finds it difficult to maintain eye contact with others while interacting. He might look at variety of things in the room and wouldn't stare at one object for long. The eyes are constantly in motion, taking in everything in the surrounding. 
• Gets bored easily - would want to switch activities after concentrating on one for a short while. It’s difficult to capture his interest on any one activity for long unless it’s of interest to him. 
• Children often lack organization in their life and would lose personal things at school, wouldn't complete class work, face difficulty in copying work from blackboard, difficulty in completing assignments. 
• Fidget and squirm in their seats in classroom, as they are expected to be seated in one place for long. Often would get up from desk and ask teacher's permission to go out for having water or to go to toilet. 
• Have difficulty in concentrating on instructions and often their work gets messy as they didn't follow all the steps required in completion of the activity. 
• Talk a lot - about anything and everything. At times child would talk about something totally irrelevant to the topic being discussed. He would have something to say about in all subjects and it’s difficult for the teacher to restrain him from expressing his thoughts. 
• Wouldn’t want to wait for his/her turn in a line for any game or anything being distributed. Would fidget and be not able to stand quietly in the morning assembly. 
• Mostly act impulsively without thinking about the consequences. 
• Don't form relationships easily, as the child has difficulty in processing verbal and non-verbal language. These kids are more often rejected by their peers than non-ADHD kids. 

Causes of ADHD

• Most common is Heredity - 25% of children have been reported to have at least one relative with some form of ADHD.
• Abnormal brain functioning - Abnormal activities in the areas of brain, which regulates attention, have been observed. MRI findings in investigations are suggestive of some smaller brain parts in children with ADHD. These studies also documented reduced blood flow to the frontal brain that regulates the executive functions.
• pre-natal and peri- natal causes - exposure of pregnant female to harmful chemicals or smoking or use of alcohol or drugs has also been known to cause some or other problem to fetus. Also, delayed birth cry, exposure to harmful chemicals after birth may cause the child to suffer from the disorder. 

Treatment 

Studies say that combination of Therapy and Medication is most effective in managing a child with ADHD. 

Therapies may include:

• Psychotherapy - This allows older children and adults with ADHD to talk about issues that bother them, explore negative behavioral patterns and learn ways to deal with their symptoms.

• Behavior Therapy - This type of therapy helps teachers and parents learn strategies for dealing with children's behavior. Strategies may include token economy and timeouts

• Occupational Therapy - concentrated on improving sitting tolerance (i.e. increasing the duration of time child can sit at one place without getting anxious), reducing hyperactivity and impulsivity through the use of meaningful and functional activities. Occupational Therapist helps the child to overcome the sensory issues (frequently present in an ADHD child), behavior issues and provide social skills training. Academic intervention and modifications in both home and classroom are done by the therapist. Therapist also helps in prevention of development of maladaptive behaviors and enhancing attention span and concentration. 

Having ADHD is not the end of the world. A look at the famous people suffering from the disorder confirms this. They have achieved what a Non-ADHD person might, and more... 

Famous actors and entertainers: Will Smith, Jim Carey, Tom Cruise, Sylvester Stallone, Robin Williams, Whoopi Goldberg, Henry Winkler, Patty Duke, and Bill Cosby.

Famous athletes: Michael Jordan, Bruce Jenner, Magic Johnson, Nolan Ryan, Terry Bradshaw, Babe Ruth, Greg Louganis, Vince Lombardi, and Pete Rose.

Famous artists: Pablo Picasso, Ansel Adams, Vincent Van Gogh, and Salvador Dali.

Famous authors: Ernest Hemingway, F. Scott Fitzgerald, Leo Tolstoy, Robert Frost, and Edgar Allen Poe.

Famous business tycoons: Bill Gates, Ted Turner, Malcolm Forbes, Andrew Carnegie, William Randolph Hearst, Henry Ford, FW Woolworth, and Walt Disney.

Famous Hollywood movie directors: Steven Spielberg and Alfred Hitchcock,

Famous inventors: Henry Ford, Benjamin Franklin, Thomas Edison, Orville Wright, Wilbur Wright, and Alexander Graham Bell.

Famous musicians: John Lennon, Elvis Presley, Cher, Buddy Rich, Beethoven, Mozart, and Handel.

Famous politicians: US: President John F. Kennedy, President Thomas Jefferson, President Abraham Lincoln, President Dwight Eisenhower, President George Bush, and President George W. Bush. International: Anwar Sadat and Winston Churchill.

Famous scientists: Albert Einstein, Stephen Hawking, Nicolai Tesla, Louis Pasteur, Galileo, and Sir Isaac Newton

Other famous people: Eleanor Roosevelt, General George Patton, Norman Schwartzkopf, Christopher Columbus, Socrates, Napoleon, Nostradamus, and Evil Knievel.

Fibrodysplasia Ossificans Progressiva

Fibrodysplasia ossificans progressiva (FOP), a rare and catastrophic genetic disorder of progressive heterotopic ossification, is the most disabling condition of extraskeletal ossification known in humans. FOP causes immobility through progressive metamorphosis of skeletal muscle and soft connective tissue into a second skeleton of heterotopic bone. ⁽¹⁾

According to the International Fibrodysplasia Ossificans Progressiva Association (IFOPA), FOP is one of the rarest, most disabling genetic conditions known to medicine; it causes bone formation in muscles, tendons, ligaments and other connective tissues. Bridges of extra bone develop across joints, progressively restricting movement and forming a second skeleton that imprisons the body in bone. There are no other known examples in medicine of one normal organ system turning into another. ⁽²⁾

During the first decade of life, children with FOP develop painful and highly inflammatory soft tissue swellings (or flare-ups) that transform soft connective tissues, including aponeuroses, fascia, ligaments, tendons and skeletal muscles, into an armament-like encasement of bone. ^{(3, 4)} Spontaneous flare-ups of the disease arise in defined temporal and spatial patterns, resulting in ribbons and sheets of bone that fuse the joints of the axial and appendicular skeleton, entombing a patient in a skeleton of heterotopic bone. A flare-up occurs when the body starts to generate new bone, although not every flare-up results in a completion of the process. No one knows what initiates this process, but once it begins, inflammation, tissue swelling, and discomfort follow. While flare-ups are usually painful, the degree of pain can vary. In addition, sometimes the individual will not feel well and may develop a low-grade fever. ⁽¹⁰⁾

Minor trauma such as intramuscular immunizations, mandibular blocks for dental work, muscle fatigue and blunt muscle trauma from bumps, bruises, falls or influenza-like illnesses can trigger painful new flare-ups of FOP leading to progressive heterotrophic ossification. ^{(5, 6, 7, 8)} Surgical attempts to remove heterotopic bone commonly lead to episodes of explosive and painful new bone growth. ⁽¹⁾

FOP involvement is typically seen first in the dorsal, axial, cranial and proximal regions of the body and later in the ventral, appendicular, caudal and distal regions. Several skeletal muscles including the diaphragm, tongue and extra-ocular muscles are enigmatically spared from FOP. Cardiac muscle and smooth muscle are not involved in the FOP process.⁽¹⁾

FOP is extremely rare with a worldwide prevalence of approximately one in two million.  It is found that genetic transmission is autosomal dominant and can be inherited from either mothers or fathers. However, most cases arise as a result of a spontaneous new mutation. ⁽¹¹⁾ FOP gene is ACVR1, a gene that is located within chromosome 2. ACVR1 stands for Activin Receptor Type 1A. The ACVR1 receptor is present in skeletal muscle and connective tissues, although exactly what its normal function in these cells and tissues is not currently understood. ⁽¹⁰⁾

Symptoms:

The skeletal malformation consists of:

• abnormal big toes. These are mostly short, monophalangeal with valgus deviation;
• short thumbs, due to short metacarpals;
• short broad femoral necks;
• abnormal cervical vertebrae with small bodies, large pedicles and large spinous processes.

Two clinical features define classic FOP: malformation of the great toes; and progressive heterotopic ossification (HO) in specific spatial patterns. Individuals with FOP appear normal at birth except for the characteristic malformations of the great toes which are present in all classically affected individuals. ⁽¹⁾

FOP is commonly misdiagnosed as aggressive juvenile fibromatosis (extra-abdominal desmoid tumours), lymphoedema or soft tissue sarcomas. Children often undergo unnecessary and harmful diagnostic biopsies that exacerbate progression of the condition. This can be particularly dangerous at any anatomical site, but especially so in the neck or back where asymmetric HO can lead to rapidly progressive spinal deformity and exacerbation of  Thoracic Insufficiency Syndrome TIS. The correct diagnosis of FOP can be made clinically even before radiographic evidence of heterotopic ossification is seen, if soft tissues lesions are associated with symmetrical malformations of the great toes. ⁽¹²⁾

Bone formation in FOP is episodic, but disability is cumulative. Most patients with FOP are confined to a wheelchair by the third decade of life, and require lifelong assistance in performing activities of daily living. The median age of survival is approximately 45 years, and death often results from complications of thoracic insufficiency syndrome (TIS). ⁽⁹⁾

References

1.      Frederick S. Kaplan, Martine Le Merrer, David L. Glaser, Robert J. Pignolo, Robert Goldsby, Joseph A. Kitterman, Jay Groppe, Eileen M. Shore. Fibrodysplasia ossificans progressive. Best Pract Res Clin Rheumatol. 2008 March; 22(1): 191–205.

2.      FOP factsheet [Internet]. 2011 [cited 2011 July 01]. Available from: http://www.ifopa.org/en/what-is-fop/overview.html

3.      Cohen RB, Hahn GV, Tabas J, et al. The natural history of heterotopic ossification in patients who have fibrodysplasia ossificans progressiva. J Bone Joint Surg Am. 1993;75:215–219. 

4.      Rocke DM, Zasloff M, Peeper J, Cohen RB, Kaplan FS. Age and joint-specific risk of initial heterotopic ossification in patients who have fibrodysplasia ossificans progressiva. Clin Orthop Rel Res. 1994; 301: 243–248.

5.      Janoff HB, Zasloff MA, Kaplan FS. Submandibular swelling in patients with fibrodysplasia ossificans progressiva. Otolaryngol Head Neck Surg. 1996; 114: 599–604. 

6.      Luchetti W, Cohen RB, Hahn GV. Severe restriction in jaw movement after routine injection of local anesthetic in patients who have fibrodysplasia ossificans progressiva. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996;81:21–25. 

7.      Glaser DL, Rocke DM, Kaplan FS. Catastrophic falls in patients who have fibrodysplasia ossificans progressiva. Clin Orthop Rel Res. 1998;346:110–116.

8.      Scarlett RF, Rocke DM, Kantanie S, Patel JB, Shore EM, Kaplan FS. Influenza-like viral illnesses and flare-ups of fibrodysplasia ossificans progressiva (FOP) Clin Orthop Rel Res. 2004; 423:275–279.

9.      Kaplan FS, Glaser DL. Thoracic insufficiency syndrome in patients with fibrodysplasia ossificans progressiva. Clin Rev Bone Miner Metab. 2005; 3:213–216.

10.  FOP guidebook for families. International FOP Association. 3^rd ed. Winter Springs, Florida; 2009.