Makiya - Motor neuron disease (Kuwait) Posted on October 26, 2012
Name: Makiya Ali KAMAL 
Sex: Female
Country: Kuwait
Age: 56
Diagnoses: 1. Motor neuron disease (MND) 2. Hypertension level 2 (High risk) 3. Type 2 diabetes 4. Sinus tachycardia 5. Post percutaneous endoscopic gastrostomy
Admission Date: September 8, 2012
Days Admitted to Hospital: 34
Before treatment: The patient suffered from barylalia in 2009. And her disease worsened in July 2010. She had obvious dysarthria and the speech couldn��t be understood by others. She also had dysphagia. She went to King's College Hospital and received an MRI examination. The examination showed periventricular periphery and basal ganglia had small lacuna infarctions. The EMG showed lingualis had lost neurological manifestations. Combined with the above findings, the Motor Neuron disease was considered. The treatment was not clear. The disease progressed gradually. The patient appeared with weakness of four limbs, muscle atrophy and restricted activity. She had a gastrostomy operation. At present, the patient has difficulty with opening the mouth or showing teeth. She, also, has weakness in swallowing or chewing. There was much muscle atrophy. She, also, has movement disturbance of four limbs.
Admission PE:
Bp: 142/90mmHg; Hr:99/min. Temperature: 36.3 degree. Gastrostomy fistula was red and mucus secretion could be seen. Periumbilical ruga skin was red, ulcerated, and smelly. There was obvious muscle atrophy in the shoulder girdle, deltoid muscle and both hands. The thorax was symmetrical, with no deformity. Thoracic expansion was symmetrical, but this was obviously lower than normal. The respiration of apex pulmonis was clear, the basis pulmonis had no respiration. When she took a deep breath, there was faint respiration, with no rale. The cardiac rhythm was regular, with no obvious murmur in the valves. Abdominal distension, and gastrostomy fistula could be seen. The abdomen was soft, with no pressing pain or rebound tenderness. The liver and spleen was not touched under the rib. Both lower limbs were not symmetrical. The leg circumference of the right calf is 35cm below the knee 9cm. The leg circumference of the left calf is 34cm below the knee 9cm. Both feet had mild edema. Construcre of bilateral achilles tendons. Drop feet.
Nervous System Examination:
Makiya Ali KAMAL was alert, normal spirits, but logagnosia. She had no cooperation in the examination of orientation, memory and calculation abilities. Both pupils were equal in size and round, the diameter was about 3.0 mms. Both pupils were sensitive to light stimuli. The movement of both eyeballs was flexible. The forehead wrinkle pattern was symmetrical. The closing ability of both eyes was strong. The bilateral nasolabial sulcus was equal in depth. She had difficulty opening her mouth. She also had difficulty with stretching here tongue. There was obvious muscle atrophy and fibrillation in the tongue. She had difficulty showing teeth. She, also, couldn't drum cheeks. The muscle strength of the soft palates was weak. The pharyngeal reflex exists. The patient also had salivation, cough with drinking and swallowing difficulty. The muscle strength of chew and cough was weak. This accompanied obvious dysarthria. The muscle strength of four limbs was level 2. The muscle tone was almost normal. The tendon reflex of both upper limbs and the left lower limb was low. The tendon reflex of the right lower limb was active. The abdominal reflexes were not elicited. Bilateral palm jaw reflex was positive. Bilateral Hoffmanns sign, Rossilimo sign and Babinski sign were neutral. Bilateral deep sensation and shallow sensation were normal through loose measure. The patient was unable to finish the finger-to-nose test, rapid rotation test and the heel-knee-shin test. There were no signs of meningeal irritation.
Treatment:
The patient received all of the relevant examinations. The heart rate reached 100-110 /min. She was diagnosed with 1. Motor neuron disease (MND) 2. Hypertension level 2 (High risk) 3. Type 2 diabetes 4.Sinus tachycardia 5. Post percutaneous endoscopic gastrostomy. She received treatment to control the blood pressure, regulate blood sugar and drop the heart rate. She was also given treatment in order to expand the blood vessels, nourishment for the neurons and strengthen nutrition. This was combined with neurological rehabilitation training.
Post-Treatment:
The patient's condition has improved. She is in good spirits. She sleeps 5-6 hours each night The blood pressure is maintained between 130-120/70-80mmHg. The heart rate is about 80-90/min. Fasting blood-glucose is between 4-5mmol/l. 2 hours after meal, the postprandial blood glucose is between 7-8mmol/l. Periumbilical skin damage has been healed. Gastrostomy fistula is clean and neat. The edema of lower limbs has faded. Neurologic symptoms show obvious improvement. She can open up her mouth 1-2cm. The tongue can reach the teeth. The pronunciation is clearer than before. The muscle strength of respiratory muscle is increased more than before. The thoracic expansion is almost normal. Both basis pulmonis has respiration and the basis pulmonis is clear. The muscle strength of four limbs is increased. The muscle strength of the left upper limb and both lower limbs is level 3. The left upper limb can offset 5-10 cm in the bed surface. The movement of the right upper limb is more flexible than before. Both knee joints can lift off the bed surface. She wears a ventilator 6-8 hours for breathing each day during her hospitalization. She also received low flow oxygen uptake 2L/min. At present, her condition is stable. Patient complained of throat discomfort, with white sticky sputum, rechecking the blood routine tests indicated the PMN was higher than normal, concerned the patient had an infection of the upper respiratory tract, gave patient an antibiotic to control the infection, drumming on the back and ultrasonic atomization therapy to help the sputum discharge, after our active treatment, the patient's infection has been controlled and her condition is stable.