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Liya Ah Al Malik-CMT-(Saudi Arabia)-Posted on Apr.29th,2015

Author Zhangqi Views Posted at 2015/04/29

Name: Liya Ah Al Malik          
Sex: Female
Country: Saudi Arabia
Age: 54 years
Diagnosis: 1. Charcot Marie Tooth disease 2. Hyperlipemia 3. Hyperthyrea
Date: Mar. 29th, 2015
Days Admitted to Hospital: 23 days

Before treatment:
Liya developed fast-growing weakness of the distal of her four limbs, balance disturbance and dysarthria for about 34 years ago with no apparent cause. She went to many hospitals and she was suspected to have” Peroneal muscular atrophy”. She took drugs for the suspected diagnosis. She couldn’t walk 24 years ago so she was on wheelchair. She wants to have a better treatment, so she came to our hospital.

She was in good spirit. Her diet, sleeping, drinking, urination and excrement were normal.

Admission PE:
Bp: 110/78mmHg; Hr: 82/min. BT: 36.5 degree, RP: 17/min. She was well nourished and obese. The skin and mucosa with no yellow stains or petechia. Her thorax was symmetrical, she had ecphysiesis and the rate of respiration was weak.  The respiration of both lungs was clear, with no dry or moist rales. Her heart rhythm was normal and strong, without no obvious murmur in the valves. The abdomen was bulge and soft with no tenderness or rebound tenderness. The liver and spleen were normal. 1/3 part below both upper limbs’ elbows to wrist, forearm muscle, interosseous muscles of both hands, thenar and hypothenar eminences and 1/3 part below both lower limbs’ knees to distal muscles were atrophied. From forefingers to little fingers of both hands and both ankle bones were contracture and she couldn’t move normally. There was dropsy on both lower limbs. The left side was worse.

Nervous System Examination:
Liya Ah Al Malik  was alert and good spirit. Her articulation was not clear, spoke slowly. Her memory, calculation and orientation abilities were normal. The ability to close her eyes was a little weak. The diameter of both pupils was 3.0mms, and both pupils were equal in size and round. Both eyeballs could move freely and the pupils reacted normally to light stimulus. The forehead wrinkle pattern was symmetrical. Both nasolabials were equal in depth. The tongue was in the centre of the oral cavity, with no teeth deflection. Both soft palates could be lifted, pharyngeal reflex was normal. She could turn her head and shrug her shoulders strongly. The abductor, flexor, pronator, supinator, left wrist muscle power of both upper limbs were at level 4+. The extensor muscle power of left upper limb and grip were at level 4+. The extensor muscle power of left upper limb was at level 3, the muscle power of cock waist of left side was at level 4, right side was at level 3. The right hand grip was at level 3+. The muscle power to bend hip and extend hip of left side was at level 4, right side was at level 3. The muscle power to bend knee and extend knee of left side was at level 3, right side was at level 2. The muscle power below ankles was at level 0. The external rotatory position of right lower limb and back easily get tired. It was hard for her to keep sitting. The muscle power of both upper limbs was normal. The muscle tension of both lower limbs was a little lower. The tendon reflex of four limbs were abnormal. Both side abdominal reflexes were abnormal. The pathology sign was negative. The rough depth determination of both sides were normal, finger-nose test and rapid rotation test were clumsy. The finger-to-finger test; she could only do numb to numb. Both side heel-knee-tibia test was hard to do. The meningeal irritation sign was negative. The blood cholesterol was higher, T4 was higher. The nerve conductive velocity: the feeling and motor conduction velocity, the vibration amplitude of sensory nerve action potentials were decreased. The electromyogram: the right musculus extensor digitorum brevis lost its innervation. The motor unit of right tibial muscle decreased. She didn’t have spontaneous movement. The motor unit potential was normal. The incubation period was longer.

Treatment:
We diagnosed Liya Ah Al Malik  based on the history of disease and past examination as: 1. Charcot Marie Tooth disease 2. Hyperlipemia. She received treatment for nerve nutrition, improving blood supply and also to improve muscle nutrition. The thyroid level was monitored. Regulation of blood lipid was done. We also gave her daily physical rehabilitation.

Post-treatment:
After the treatment, her lower limbs were not that dropsically. She could speak more clearly. The balance of upper limbs was better, and the muscle power of four limbs were better. The abductor, flexor, pronator, supinator, left wrist muscle of both upper limbs, the extensor of left upper limb, the grip of left hand were all at level 5-. The extensor of right upper limb was at level 4, the muscle power of cock waist of left side was at level 4, right side was at level 3. The grip of right hand were all at level 3+. The muscle power to bend hip and extend hip of left side was at level 4, right side was at level 3+. The muscle power to bend knee and extend knee of left side was at level 3, right side was at level 2. The muscle power below ankles were at level 1. The external rotatory position of right lower limb was better than before, the muscle power of back were stronger, and her back was more straight than before. The blood lipid was normal, the thyroid level was lower than before. Her treatment was over and discharge was permitted.

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