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Neuromuscular Electrical Stimulation Therapy for Dysphagia Caused by Wilson's Disease.

Lee SY, Yang HE, Yang HS, Lee SH, Jeung HW, Park YO - Ann Rehabil Med (2012)

Bottom Line: A 33-year-old man visited our hospital with a complaint of difficulty in swallowing, since about last 7 years and which had worsened since the last 2-3 months.On the initial VFSS, reduced hyoid bone movement, impaired epiglottic movement and moderate amount of residue in the valleculae during the pharyngeal phase were noted.After 10 sessions of neuromuscular electrical stimulation for 1 hour per day, decreased amount of residue was observed in the valleculae during the pharyngeal phase on the follow-up VFSS.

View Article: PubMed Central - PubMed

Affiliation: Department of Physical Medicine & Rehabilitation, Seoul Veterans Hospital, Seoul 134-791, Korea.

ABSTRACT
Wilson's disease is an autosomal recessive disorder of abnormal copper metabolism. Although dysphagia is a common complaint of patients with Wilson's disease and pneumonia is an important cause of death in these patients, management of swallowing function has rarely been reported in the context of Wilson's disease. Hence, we report a case of Wilson's disease presenting with dysphagia. A 33-year-old man visited our hospital with a complaint of difficulty in swallowing, since about last 7 years and which had worsened since the last 2-3 months. He was diagnosed with Wilson's disease about 13 years ago. On the initial VFSS, reduced hyoid bone movement, impaired epiglottic movement and moderate amount of residue in the valleculae during the pharyngeal phase were noted. After 10 sessions of neuromuscular electrical stimulation for 1 hour per day, decreased amount of residue was observed in the valleculae during the pharyngeal phase on the follow-up VFSS.

No MeSH data available.


Related in: MedlinePlus

Brain magnetic resonance imaging on fluid attenuated inversion recovery imaging shows high signal intensity (arrow) in the pons (A) and the midbrain (B).
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Figure 1: Brain magnetic resonance imaging on fluid attenuated inversion recovery imaging shows high signal intensity (arrow) in the pons (A) and the midbrain (B).

Mentions: A 33-year-old man had difficulty in swallowing solids and liquids since the last 7 years. This difficulty in swallowing was aggravated since the last 2-3 months prior to hospitalization. His medical history revealed that he was diagnosed with Wilson's disease in 1997 and he had no other diseases. His family history revealed that his elder sister died of Wilson's disease 13 years ago. The physical examination showed that the cognitive function was normal while dysarthria was evident. The evaluation of cerebellar function tests showed mild ataxia and dysdiadochokinesia. The limb muscle strength testing using the MRC (Medical Research Council) demonstrated normal results within a range of 5 degrees and no muscle atrophy. Generalized distal sensory disturbance was observed in both the upper and lower limbs, and normal deep tendon reflexes and a normal gag reflex were also observed. The laboratory tests showed that the amount of serum copper and ceruloplasmin level was reduced; 21.15 ug/dl and 8.3 mg/dl respectively. The abdominal ultrasound showed liver cirrhosis with significant splenomegaly. As far as the radiologic finding is concerned, the brain magnetic resonance imaging (MRI) on FLAIR (Fluid attenuated inversion recovery) imaging showed high signal intensity in both the pons and the midbrain (Fig. 1).


Neuromuscular Electrical Stimulation Therapy for Dysphagia Caused by Wilson's Disease.

Lee SY, Yang HE, Yang HS, Lee SH, Jeung HW, Park YO - Ann Rehabil Med (2012)

Brain magnetic resonance imaging on fluid attenuated inversion recovery imaging shows high signal intensity (arrow) in the pons (A) and the midbrain (B).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3400883&req=5

Figure 1: Brain magnetic resonance imaging on fluid attenuated inversion recovery imaging shows high signal intensity (arrow) in the pons (A) and the midbrain (B).
Mentions: A 33-year-old man had difficulty in swallowing solids and liquids since the last 7 years. This difficulty in swallowing was aggravated since the last 2-3 months prior to hospitalization. His medical history revealed that he was diagnosed with Wilson's disease in 1997 and he had no other diseases. His family history revealed that his elder sister died of Wilson's disease 13 years ago. The physical examination showed that the cognitive function was normal while dysarthria was evident. The evaluation of cerebellar function tests showed mild ataxia and dysdiadochokinesia. The limb muscle strength testing using the MRC (Medical Research Council) demonstrated normal results within a range of 5 degrees and no muscle atrophy. Generalized distal sensory disturbance was observed in both the upper and lower limbs, and normal deep tendon reflexes and a normal gag reflex were also observed. The laboratory tests showed that the amount of serum copper and ceruloplasmin level was reduced; 21.15 ug/dl and 8.3 mg/dl respectively. The abdominal ultrasound showed liver cirrhosis with significant splenomegaly. As far as the radiologic finding is concerned, the brain magnetic resonance imaging (MRI) on FLAIR (Fluid attenuated inversion recovery) imaging showed high signal intensity in both the pons and the midbrain (Fig. 1).

Bottom Line: A 33-year-old man visited our hospital with a complaint of difficulty in swallowing, since about last 7 years and which had worsened since the last 2-3 months.On the initial VFSS, reduced hyoid bone movement, impaired epiglottic movement and moderate amount of residue in the valleculae during the pharyngeal phase were noted.After 10 sessions of neuromuscular electrical stimulation for 1 hour per day, decreased amount of residue was observed in the valleculae during the pharyngeal phase on the follow-up VFSS.

View Article: PubMed Central - PubMed

Affiliation: Department of Physical Medicine & Rehabilitation, Seoul Veterans Hospital, Seoul 134-791, Korea.

ABSTRACT
Wilson's disease is an autosomal recessive disorder of abnormal copper metabolism. Although dysphagia is a common complaint of patients with Wilson's disease and pneumonia is an important cause of death in these patients, management of swallowing function has rarely been reported in the context of Wilson's disease. Hence, we report a case of Wilson's disease presenting with dysphagia. A 33-year-old man visited our hospital with a complaint of difficulty in swallowing, since about last 7 years and which had worsened since the last 2-3 months. He was diagnosed with Wilson's disease about 13 years ago. On the initial VFSS, reduced hyoid bone movement, impaired epiglottic movement and moderate amount of residue in the valleculae during the pharyngeal phase were noted. After 10 sessions of neuromuscular electrical stimulation for 1 hour per day, decreased amount of residue was observed in the valleculae during the pharyngeal phase on the follow-up VFSS.

No MeSH data available.


Related in: MedlinePlus