André Bordat-Amyotrophic Lateral Sclerosis (ALS)-(French)

Patient Name: André Bordat
Gender: Male
Age: 72 years old
Nationality: French
Diagnosis: Amyotrophic Lateral Sclerosis (ALS)

Condition on Admission:
The patient was admitted with a diagnosis of amyotrophic lateral sclerosis, presenting with "progressive limb weakness, muscle atrophy accompanied by fasciculations for 2 years, and dysarthria for half a year". The patient’s condition progressively worsened and gradually involved all four limbs, predominantly the upper limbs, accompanied by muscle atrophy and fasciculations. In the past six months, the patient developed dysarthria, reduced voice volume, occasional dysphagia, choking when drinking water, slight breathing difficulty, weak productive cough, accelerated respiratory rate, with a blood oxygen saturation of 94%. Non‑invasive ventilator therapy was administered at the local medical facility.

Physical Examination on Admission:
The patient’s heart rate was 95 beats per minute, respiratory rate 28 breaths per minute, blood pressure 136/74 mmHg, and blood oxygen saturation 94%. The patient had normal physical development and fair nutritional status. Perioral mild cyanosis was observed. Thoracic respiratory mobility was diminished; breath sounds were weak in the bilateral lower lungs, more remarkable in the right lower lung, with no obvious dry or moist rales auscultated. Cardiac sounds were strong and regular, and no significant murmurs were heard over each cardiac valve area. The abdomen was flat without tenderness or rebound tenderness; the liver and spleen were not palpable below the costal margin. Scoliosis with a 15‑degree mild right‑ward convexity was noted from the T12 to L4 vertebral segments. No obvious pitting edema was found in the bilateral lower extremities.

Neurological Examination:
The patient was conscious and in fair spirits. Articulation was unclear with weak phonation. Memory, calculation and orientation were all normal. Bilateral pupil diameter measured 3 mm with brisk light reflex. Bilateral nasolabial folds and forehead wrinkles were symmetrical. Elevation strength of the soft palate was reduced. The tongue protruded in the midline with poor power; the tongue tip could only reach the lower lip. Marked tongue muscle atrophy with fasciculations was present. Masticatory strength was decreased, accompanied by occasional choking on water. Muscle strength for neck rotation and shoulder shrugging was Grade 4. Left upper limb muscle strength was Grade 2 with a grip strength of Grade 2; right upper limb muscle strength was Grade 1+ with a grip strength of Grade 1+. Muscle strength of the bilateral lower limbs was Grade 4. Muscle atrophy and fasciculations were seen in all four limbs, most prominent in the supraspinatus, infraspinatus, teres minor, deltoid, thenar, hypothenar and interosseous muscles. Only the left index finger could complete the finger‑to‑finger test. The heel‑knee‑shin test was performed slowly. Limb muscle tone was roughly normal. Tendon reflexes of all four limbs were diminished. The left Hoffman sign and Babinski sign were positive. Meningeal irritation signs were negative.

Treatment Course:
A definitive admission diagnosis of amyotrophic lateral sclerosis was established. During hospitalization, the patient received neural stem cell therapy to repair motor nerve injury and mesenchymal stem cells for neurotrophic, endocrine and immunological support. Adjuvant CAST therapy was administered with medications including edaravone, neurotrophic factors, monosialoganglioside and reduced glutathione, together with comprehensive rehabilitation therapy.

Post‑treatment Outcome:
The patient’s motor function is improved with increased muscle strength in all limbs. When be seated, the patient can lift the left upper arm by 10 cm; all fingers of the left hand are able to perform the finger‑to‑finger test, and the left hand can hold a pen for simple tracing. Passively flexing the right elbow to place the forearm against the chest can be sustained for 2 seconds; the right forearm is capable of pronation and supination. Flexion‑extension movement of the right fingers becomes more flexible, the right index finger can complete the finger‑to‑finger test, and grip strength is markedly enhanced. Re‑examination: muscle strength for neck rotation and shoulder shrugging is recovered to Grade 5. Proximal muscle strength of the left upper limb is Grade 3 with grip strength Grade 3; proximal muscle strength of the right upper limb is Grade 2+ with grip strength Grade 3‑. Muscle strength of the bilateral lower limbs is reached Grade 5. Cardiopulmonary function is improved: heart rate is ranged from 70 to 80 beats per minute; the resting respiratory rate is approximately 20‑22 breaths per minute. Breath sounds in the right lung are louder than before, and blood oxygen saturation is maintained above 95%, peaking at 97‑98%. Energy, physical stamina and exercise tolerance are ameliorated.

    

    

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