Re: CMT and Dysphasia

[Guest guest]

Swallowing can be a problem in CMT. See below for article from CMTI

in the 1992. Also see this for current info

http://www.nidcd.nih.gov/health/voice/dysph.asp

Problems in swallowing by Bonnie Pfeiffer, MA, CCC-Sp.

" Why is it when I went to have my throat looked at they couldn't find

anything wrong with my swallowing? I know there is a problem but how

do I prove it to my doctor? Is there a test? "

The answers to these questions are complex at best. As most of us are

aware, research in the area of degenerative neurologic disease

processes has been limited – particularly concerning CMT. There is

even less literature available about associated swallowing disorders.

Many studies have looked at swallowing in populations of individuals

having a variety of neurologic diseases. Others have focused on

groups of homogenous patients without regard to the degree of

neurologic involvement in individuals. This makes for difficulty in

identifying typical swallowing dysfunctions or in predicting

progression.

There is, however, a great deal known about the types of swallowing

problems symptomatic of degenerative diseases similar to CMT, tests

available to diagnose specific impairments, and techniques for

remediation or compensation.

Disordered Swallowing

Normal swallowing consists of three phases: oral, pharyngeal, and

esophageal. During the oral phase of swallowing, food must be chewed,

moved on to the tongue, gathered together, pushed to the back of the

mouth, and squeezed into the throat by the tongue muscles. In the

pharyngeal phase, the sensation of food entering the throat sets in

motion a wave of muscle contractions that propels the food to the

opening of the esophagus (food pipe) where a circular muscle, or

sphincter, opens to allow passage through the esophagus into the

stomach. Food travels through the esophagus in much the same way as

it does through the throat – by a series of muscle contractions

activated by sensory input.

Degenerative neurologic disease can create problems in the swallowing

system similar to those affecting other sensory/motor functions

elsewhere in the body: decreased sensory input, muscle weakness or

visible atrophy, and incoordination of movement. Symptoms may occur

such as difficulty in chewing or moving food around with the tongue,

inability to swallow (initiate the " swallowing reflex " ), the

sensation of food " getting stuck " in the throat or esophagus, choking

on liquids, or regurgitation of food into the mouth or nose. Poor

vocal cord closure, decreased laryngeal (voice box) elevation, and

insufficient or incoordinated breathing patterns can also interfere

with swallowing.

Evaluation

A series of tests, involving a number of different medical

specialists, can be used to assess swallowing. Because swallowing is

a dynamic process, a particularly useful method of evaluation is a

videofluoroscopic study – also called a " cookie swallow " or modified

barium swallow. A videotaped recording, under radiation, is made

while the patient swallows different food consistencies (liquids,

pastes, or solids) of radio-opaque material – usually barium. In most

facilities this test is performed as a cooperative procedure between

a radiologist and a speech pathologist or occupational therapist.

A number of additional medical tests may be recommended based on the

results of an initial videofluoroscopic swallowing study.Referral to

an ENT (ear, nose and throat doctor) for an indirect laryngoscopy is

warranted when there is concern about the ability of the larynx too

protect the airway during swallowing. The physician will look in the

throat with a special instrument to assess the ability to close the

vocal cords, elevate the voice box, and clear the airway of any

foreign substance by producing a strong cough.

Fiberoptic bronchoscopy is of similar diagnostic value in looking at

the larynx with the increased capabilities provided by the use of

sophisticated photographic techniques. The upper airway is also

visualized for the presence of infection, bleeding, and tumors or

other obstructions that may be causing breathing difficulties or

otherwise interfering with swallowing.

Further assessment of the esophagus can be accomplished by endoscopy

(insertion, through the mouth, of an instrument capable of allowing

the physician to view the food pipe from the inside) or manometry.

Esophageal manometry is useful in diagnosing weak or poorly

coordinated muscle contraction and dysfunction of the muscle that

permits food to pass from the esophagus into the stomach.

CMT individuals can and do have swallowing difficulties; but not all

of us show the same degree or combination of symptoms. It is,

therefore, important to obtain a thorough evaluation of swallowing

and its related systems by medical professionals. And the good news

is that many of these problems can be easily compensated for or

remediated by therapeutic techniques.

Getting help

Most major medical facilities evaluate swallowing function by

videofluoroscopy and selective additional testing. Contacting the

Departments of Speech Pathology (Communication Disorders) or

Occupational Therapy in area hospitals or rehabilitation centers may

be a good place to start for advice. Universities offering training

programs for rehabilitation professionals can also be of help.

In the United States, several regional swallowing centers are

available for direction and consultation services. The Consumer

Division of the American Speech Language and Hearing Association will

be able to provide information concerning services available in your

area.