Speech problems

Recently while evaluating a child with speech problems, the parents mentioned whether the child had apraxia or not. What ensued was a discussion in which neither the parents nor myself knew what we were talking about. To add to the confusion, my hippocampus had thrown out several associations to apraxia of speech, like dysarthria, phonological disorder and aphasias. Doing an internet search compounded the difficulty just as asking various people did. Everyone answered something that they were not sure about and soon my head was swimming about their differences. Luckily I found an article in Neurocase (2005) 11, 427-432 by Ogar et al.

APRAXIA OF SPEECH: Impaired ability to coordinate the sequential, articulatory movements neccessary to produce speech sounds is called apraxia. Articulatroy erros and prosodic abnormalities are hallmarks. Signs include effortful trial and error grouping with attempts at self correction, persistent dysprosody, articulatory inconistency on repeated productions of the same utterance and/or obvious difficulty initiating utterances. Vacular lesions, trauma, tumors can cause this. Apraxia of speech is also the first symptom in neurodegenerative diseases such as corticobasal degeneration or non-fluent progressive aphasia.

CONDUCTION APHASIA: Result of damage to communication between Wernicke and Broca. This communication is through extreme capsule and/or arcuate fasiculus. Speech is fluent, comprehension is good but oral reading is poor and major impairment in repitiion. Many paraphasias occur and transpositions of sounds within a word also occurs (television -> velitision).

BROCA'S APHASIA: In this the speech is understood, and the all the levels of speech planning are intact except for motor execution which is not intact.

(Apraxic speakers are believed to select the correct phonemes, only to have trouble with their motor execution; People with conduction aphasia typically speak with near normal prosody, whereas halting effortful speech with abnormal prosody. They may also lack awareness of their speech errors and therefore may not always make attempts at self correction while the opposite is true in cases of apraxia of speech)

DYSARTHRIA: Dysarthria is caused by impairment of muscle strength, tone, range of motion and/or coordination. It can be caused by UMN or LMN lesions of the cranial nerves.

(Errors heard in dysarthric speech are typically consistent and predictable, while the speech errors heard in apraxia of speech tend to be highly irregular. Sound distortions, prolonged segment durations (e.g prolonged vowels or consonants) and prolonged inter segment durations (e.g. abnormal pauses within sounds, syllables or words) are characteristic for apraxia of speech.

PATHOPHYSIOLOGY OF APRAXIA: Van der Merwe's model of speecch planning and programming says that initially, basic linguistic units or phonemes are selected. During a second motor planning phase these phonemes are organized into temperospatial codes for speech production. In the third, motor programming phase, muscle specific motor programs are selected and sequenced before moving forward to fourth phase when these sequences are carried out by the speech musculature. Apraxia of speech is caused by problem with second phase.

TREATMENTS: For mildly apraxic patients, poor prosody may be the primary speech deficit and therefore, goals designed to improve intonation and stress. For the moderately or severely apraxic patient, therapy might focus on relearning oral postures for individual speech sounds.
-PROMPT uses rate and rhythm control strategies
-Wambaugh and colleagues use remediation of misarticulated consonants through modeling repetition of minimally contrastive words, graphic cues and phonetic placement cueing.

Dyslexia

Dyslexia: A specific reading disability due to a defect in the brain's processing of graphic symbols.

Dyslexias are characterized by their characteristic hemishperic deficit.
Pirozzolo (1979) and Pirozzolo and Hess (1976) suggest that there are two fundamental types of dyslexia: auditory-linguistic dyslexia and visual-spatial dyslexia. Persons with the former exhibit difficulties in the verbal and language area, have naming problems, and are slow in carrying out any types of verbal tasks. The persons with the former struggle with visual perceptual difficulties. Bakker (1973; 1979; 1982; 1983) thinks about different approaches to reading. The linguistic-auditory group uses their left type hemishphere hence called L-type dyslexics. The visual dyslexics have a difficulty with perceptual requirements in word representation and use the disorder stems from right hemisphere, hence the term P-called dyslexics. The same kind of differentiation is meant by the the words dysphonetic(auditory problems) and dyeidetic dyslexics (visual problems). Hemishpheric EEGs also show preferential brain activation with the different types of dyslexics.

Psychic trauma in children: review of an article

AM J Psychiatry 138:1 January 1981 by Lenore C. Terr, M.D.

Background:
This article discusses the response to psychic trauma in children who were exposed to the kidnapping in Chowchilla.
The story of the kidnapping goes something like this: July 1976, 26 children (5-14 year) disappeared for 27 hours and they eventually escaped their captors. The school bus had been stopped by a van blocking the road and masked men had taken over the bus at gun point. The children were transferred to boarded over vans in which they were driven for 11 hours and then transferred into a "hole" (actually a buried truck trailer). The kidnappers covered the truck-trailer with earth. The children were buried for 16 hours until two of the oldest and the strongest boys 14 and 10 dug them out. By then the kidnappers had left the vicinity.

Methods:
The children were interviewed by the author and one or both of the parents had also been interviewed. The school bus driver and the kidnappers were not interviewed. This interview took place between 5-13 months.

Notes:
Initial signs of traumatic disruption (the breach of the ego):
-Omens: Children tend to associate things that happened before the incident and related that as the causal factor of why they were kidnapped.
-Fear of further trauma: Traumatophobia. (am I going to be killed?)
-Disturbances in cognition: During trauma disturbance in cognitive function such as perception, time sense and thought.

Repetitive phenomena (Repetition compulsion):
-Traumatic dreams: 1)terror dreams 2)exact repetition of kidnapped events 3)modified repetitions of kidnapping events 4)deeply disguised dreams
-Post traumatic play
-Reenactment: Direct reenactments of attitudes, fears or actions that have occurred before or after the kidnapping.
-Absence of flashbacks: Adolescents exhibited "voluntary" visions in contrast to the involuntary intrusive thoughts that adults have. Children younger than 9 did not complain of having visions. The ability to day dream develops after 9 and that might be the reason why children below 9 did not have flash backs.

Fears: (Kidnap related fears)
-All children had kidnapping related fears.

Personal Commentary:
Note that the DSM required criteria of a distressing event, re experience, avoidance and increased arousal are necessary for PTSD. Children in this incident also displayed re experience (exeplified by the repetitive phenomena), avoidance (is an end point of multiple psychological factors including omens and fears) and hyperarousal (exemplified by fears) .

Diagnosis of Tourette's Syndrome

Tourette's disorder is named after Gilles de la Tourette, a french neurologist who was a student of Charcot. He worked as his resident in Salpetriere, Paris. Charcot named this disease for his resident when Tourette published a case series of nine patients with Tourette's syndrome. Previously thought to be rare, it is now thought to be a spectrum disorder with neuropsychological sequelae. Incidence ranging from 1-10 per 1000. Tourette's is a disorder which poses as many different things and is usually misdiagnosed with many things before it is correctly diagnosed. The DSM IV TR criteria for Tourette's are:

A. Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently. (A tic is a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalization.)

B. The tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than 1 year, and during this period there was never a tic-free period of more than 3 consecutive months.

C. The onset is before age 18 years.

D. The disturbance is not due to the direct physiological effects of a substance (e.g., stimulants) or a general medical condition (e.g., Huntington's disease or post viral encephalitis).

As per criteria it is useful to screen patient's for tics but sometimes it is also useful to keep a flexibility to go the opposite way, i.e, look for a particular type of a personality and then look for tics. The typical tourette's personality reflects the development of individuals in a certain way. Individuals with Tourette's have problems with inattention, hyperactivity, anxiety symptoms, obsessive thoughts, compulsive actions, stubbornness, mood swings and tics. The development of Tourette's children is probably parallel to the development of a child without Tourette's because of the fact that people with Tourette's can be found in all walks of life.

Some people call the associated features of Tourette's as co morbid diagnoses. Anxiety disorders, ADHD especially inattentive type and Cyclothymia vs Bipolar can be seen. So searching for tics once seeing a constellation of symptoms as above can be helpful.

VACTERL association

V- Vertebral anomalies
A- Anal Atresia
C- Cardiovascular anomalies
T- Tracheoesophageal fistula
E-Esophagial Atresia
R- Renal anomalies or Radial anomalies
L- Limb anomalies

This is an association not a syndrome because of a lack of one single pathogenic gene. PubMed as of 9/15/08 does not give any psychological sequelae to this association. That is odd, given the neuroectodermal involvement as evidenced by the vertebral anomalies.
One paper found that IQ did not tend to be different from any other children hence the need to address individual anomalies early on is essential. Like any other chronic disease the earlier any barriers to development are removed the more "normal" the development.
Here is a network for family and children with VACTERL .
I have seen one child with this syndrome with anger issues. I did not have the opportunity to engage in a psychiatric interview though so I cannot delineate the etiology of the anger.

More research needed. :)

Pakistan and ADHD

I was trying to research ADHD in Pakistan today and came across this article from J Pak Med Assoc. 2003 Sep;53(9):441-3

Qureshi A, Thaver D.Aga Khan University Hospital, Karachi.

Ok so ADHD exists in Pakistan. Now what is the treatment for it. Primarily would be the use of analeptics. If one class of analeptics is not effective the medication can be switched to the other class and ~90% children respond. One class being the Methylphenidate products like Ritalin and the long acting methylphenidate products and methylated compounds of methylphenidate. The other class is the amphetamine salts like dextroamphetamine and the mixture of the two enantiomers of the dextroamphetamine (Adderall, Vyvanse, Dexedrine). In Pakistan only the MPH compounds are available.

I found Ritalin at an online pharmacy. 10 mg tablets. The price is Rs 4.7 for 10mg. It is certainly not steep. A 40kg child would be taking roughly 1mg/kg/dose as the high end would end up spending ~Rs 18. If this dose is taken two to three times a day the cost increases up to 18x3=54. If this is not given on days off from school, the child will take it ~20 days. So Rs 1080 for an entire month.

Now the question is what is the other option besides Ritalin. Nothing. I have not been able to find Amphetamine salts. Second line medications like Alpha 2 agonists (Guanfacine and Clonidine) which are also used as blood pressure medications are also not available.

Bupropion is available. This also a second to third line agent. 75 mg tablet costs Rs 10. It is usually needed in doses of 150mg-300mg and everyday medication will make it a cost of anywhere from Rs 600-Rs 1200 per month.

Atomoxetine is not available. That would also be a second to third line agent. Especially useful in children who cannot withstand analeptics.

Then there is the tricyclics, which are available but that would be third line agents and not the best medications for a hyperactive child. Side effect profile unfavorable.