Kanven Sherry - CMT (Lebanon) Posted on October 23, 2014
Name: Kanven Sherry
Sex: Female
Country: Lebanon
Age: 52 years
Diagnoses: 1. Charcot- Marie - Tooth disease 2. Hyperlipemia
Date: September 21, 2014
Days Admitted to Hospital: 19 days
Before treatment:
The patient suffered from involuntary beating of fingers of right hand 15 years ago. She also suffered from weakness of four limbs' far-end. The EMG and other examination showed she had Charcot-Marie-Tooth disease. She took VB for treatment, but had no effect. The disease aggravated gradually. She suffered from different muscle atrophy of four limbs' far-end. The patient's condition was aggravated gradually 6 years ago, she felt heaviness and weakness of both legs when she walks. The right limb was more severe. Her right hand had difficulty with grasp. There was obvious muscle atrophy in four limbs' far-end. Before the treatment, patient had walking difficulty, she only could walk for 10 minutes, and the balance control ability was not very good. She was unable to walk with high-heeled shoes. She had poor balance ability when she walked.
From the onset of disease, the patient's spirit was good. The diet was good. The sleep quality was normal. There was no cough when she drinks. The urine and defecation were normal. Her weight had increased to 15Kg in recent years.
Admission PE:
Bp: 130/70mmHg; Hr: 83/min. Temperature: 36.6 degrees. Nutrition status is okay, slight fat, the skin and mucosa were normal with no hemorrhaging spots or yellow stains. Breathing sounds of both lungs are clear, no rales, heart rate is regular, no murmur, abdomen is slight bulge and soft, no pressing pain or rebound tenderness. Liver and spleen were not enlarged. There are obvious muscle atrophy of lateral side of bilateral forearms in the distal side, the thenar muscles of both sides, bilateral interphalangeal muscles, calf muscles of both lower limbs, muscles in the 1/3 lower thigh area and bilateral bottom of feet area. The muscle atrophy is much severe in the right side of the body, patient has wrist joint movement limitation, no edema of lower limbs.
Nervous System Examination:
Patient is alert, speech clearly, memory ability, calculation ability and orientation ability are all normal. The bilateral pupils are equal and round, diameter is 3.0mm, react well to light. The movement ability of both eyeballs are basically normal, while the right side converge ability is less than normal. The forehead wrinkle pattern is symmetrical. The eyes could close strongly. The nasolabial sulcus is equal in depth. The tongue was in the center of the oral cavity and the teeth are shown without deflection. The bilateral soft palate can be raised normally. The pharyngeal reflex is normal. Grip force of left hand is 4 degree, of right side is 3 degree; muscle power of left fingers is 4- degree, of right side is 3- degree; the muscle power of proximal upper limbs is 4 degree, of distal side is 3 degree; muscle power of proximal lower limbs is 4 degree, of distal side is4- degree. She was unable to stand with single leg. Muscle tone of 4 limbs are normal, tendon reflex and abdomen reflex could not be induced by examination; bilateral palm-jerk reflex are negative; bilateral Hoffmman sign are negative; Rossilimo sign of both sides are negative; Babinski sign of both sides are negative. Deep sensation and superficial sensitivity are normal, patient complains radiating pain of the bilateral shoulders area and right side suffers a lot. Finger-to-nose test, finger opposite test and fast alternating test are basically normal, heal-knee-tibia test is normal. No meningeal irritation sign.
Treatment:
We gave patient complete body examination after her admission and confirmed the diagnosis of 1. Charcot-Marie-Tooth disease. The patient received treatment for nerve regeneration and also for activation of stem cells in vivo. Doctors also gave patient treatment to nourish the neurons, improve the blood circulation, accelerate the neural system repair, provide enough nutrition to the muscles, help patient to adjust the blood lipid level, etc, and this was combined with rehabilitation training. Patient had cough and sputum in the second week, doctors considered she had upper tract infection, we provided patient with active treatment for infection, and the therapy to alleviate the cough and reduce the sputum.
Post-treatment:
Patient's blood lipid level reduced very much after the treatment, there is obvious new muscle tissue growth in the bilateral thenar muscles, and the muscle power has increased. The hold power of left hand is at level 4. The hold power of right hand is at level 3+. The muscle strength of fingers of left hand has reached level 4. The muscle strength of fingers of right hand has reached level 3. The muscle strength of upper limbs' near-end is at level 4. The muscle strength of upper limbs' far-end is at level 3+. The muscle strength of both lower limbs' near-end is at level 4, the muscle strength of lower limbs' far-end is at level 4. Her exercise tolerance has improved, and does not feel tired easily. Patient balance control ability has improved, she can stand with single leg for several seconds.
