Commentary case

1. Commentary case By PROF DR :FawzyMegahed Asst lec :RaafatSaeid

2. A 50-year-old man was transferred to the hospital because of cardiogenic shock.

3. A diagnosis of dilated non ischemic cardiomyopathy had been made when the patient was in his late 40s. When he was 48 years of age, a dual-chamber pace- maker and implantable cardioverter–defibrillator (ICD) was placed.

4. Four months before admission, trans thoracic echocardiography revealed a left ventricular ejection fraction of 40% Several days before admission, malaise, fatigue, increasing dyspnea, and chest tightness developed

5. One day before admission, the patient was seen by his primary care provider. The systolic blood pressure was reportedly between 80 and 89 mm Hg, the pulse higher than 100 beats per minute, and the oxygen saturation 77% while he was breathing ambient air. Emergency medical services were called

6. on examination, the patient’s mentation was normal, and the cardiac rhythm was rapid atrial fibrillation. During transport by ambulance to another hospital, ventricular tachycardia developed, and the patient became unresponsive and apneic.

7. Cardiopulmonary resuscitation was begun, and the trachea was intubated. In the emergency department, blood levels of magnesium and alkaline phosphatase were normal; other test results are shown in Table 1

11. Ventricular tachycardia was followed by transient pulseless electrical activity, with hypotension, hypoxemia, hypercapnia, and acidosis. Oxygen, multiple external shocks, epinephrine, amiodarone, dopamine, and sodium bicarbonate were administered

12. .After approximately 25 minutes, atrial fibrillation was restored, and the blood pressure rose to 98/20 mm Hg. Norepinephrinebitartrate, insulin, midazolam, cisatracurium, furosemide, fentanyl, vasopressin, sodium bicarbonate, magnesium, calcium gluconate, potassium chloride, potassium phosphate, heparin, and normal saline were administered.

13. Transthoracic echocardiography revealed a left ventricular ejection fraction of 15%, severe diffuse hypokinesis, regional wall-motion abnormalities, mild left ventricular dilatation, normal wall thickness, and moderate mitral regurgitation, without pericardial effusion

14. Chest radiographs reportedly showed increasing consolidation of the right middle and upper lobes. The patient was paralyzed, and mechanical ventilation was continued. Furosemide and chlorothiazide were administered for oliguria, and broad-spectrum antibiotic agents for presumed aspiration pneumonia;

15. venoarterial extracorporeal membrane oxygenation (ECMO) was initiated for persistent hypoxemia. On the second day, the patient was transferred to this hospital and admitted to the ICU.

16. The patient had muscle weakness that had begun approximately 15 years earlier, when in creasing weakness of his hands developed. When the patient was 40 years of age, episodes of lightheadedness occurred; a diagnosis of par-oxysmalatrial fibrillation was made, and prophylactic anticoagulation with warfarin was be gun

17. When he was 41 years of age, he noted difficulty walking (with occasional stumbling), gripping and releasing tools, and climbing stairs and ladders. Electromyography, which was performed at another hospital, reportedly revealed brief-duration, low-amplitude, polyphasic motor- unit potentials.

18. During the next 3 years, his symptoms worsened, and his left forearm was fractured after a fall. On examination when the patient was 44 years of age, extra ocular movements were normal, and mild bilateral ptosis, moderate bilateral facial weakness, and slight thinning of the temporalis muscles were present

19. His speech had a nasal quality, without dysarthria or tongue weakness. The muscle weakness was rated as 4 on the Medical Research Council (MRC) scale (which ranges from 0 [paralysis] to 5 [normal strength]) and involved the neck flexors, finger extensors, and intrinsic muscles of the hands; strength was normal in the proximal muscles of the arms.

20. Muscle tone was normal throughout. After the patient firmly grasped the examiner’s hand, hehad difficulty releasing his grip quickly.Tapping of the finger extensor muscles on the dorsal forearm and on the abductor pollicis in the thenar eminence produced muscle contractions that relaxed slowly.

21. There was slight weakness of the hip flexor muscles, normal strength in the quadriceps and hamstrings, and weakness of the dorsiflexors (MRC score, 3+) and plantar flexors (MRC score, 4) of the feet.

22. The patient was able to point to a target with his fingertip, perform the heel–knee–shin test, and carry out rapid, rhythmic tapping movements with his hands and feet. Vibration sense was mildly decreased in his toes. He rose from a chair without the use of his arms and had a high-stepping gait

23. Deep-tendon reflexes were hypoactive in the arms and absent at the ankles. Romberg’s sign was absent. Over the course of the next few years, weakness progressed. When the patient was 47 years of age, he had difficulty lifting gallon buckets at work.

24. examination reportedly revealed weakness of the neck flexors (MRC score, 4−), wrist and finger extensors (MRC scores, 4), and finger flexors and distal leg muscles (MRC scores, 3+) and normal strength in the deltoid, biceps, triceps, hip flex- ors, quadriceps, and hamstrings.

25. Deep-tendon reflexes were absent. The patient also had obstructive sleep apnea, sudden sensorineural hearing loss in the left ear (which had begun when he was 45 years of age and which was unresponsive to glucocorticoidtherapy), hypertension, hyperlipidemia, morbid obesity, cholelithiasis (which had required cholecystectomy

26. problems with ejaculation, non–insulin-dependent type 2 diabetes mellitus, gastroesophageal reflux disease, depression, vitamin B12 and vitamin D deficiencies, and episodes of pneumonia that were thought to be related to aspiration

27. . Medica- tions that he was taking before this acute illness included warfarin, pravastatin, pioglitazone, metformin, ketoconazole, atenolol, citalopram, furo- semide, pantoprazole, and bupropion (all daily), as well as a vitamin B12 supplement (monthly) and a vitamin D supplement (weekly).

28. He reportedly had allergies to morphine, codeine, and tramadol. He was married and lived with his wife and children. He worked in a retail store.

29. He drank alcohol occasionally and did not smoke. His father and one sister had an undefined neuromuscular problem. His father had had lung cancer, and his mother uterine cancer; both died when they were in their 70s

30. One of his children had scoliosis and a neuromuscular disorder similar to that of the patient.

31. On examination, the patient was sedated and paralyzed. Coarse breath sounds in the lungs and active bowel sounds were present. There was trace edema of both legs, and the abdomen was soft and nondistended.

32. The pupils were sluggishly reactive; oculocephalic and corneal reflexes were absent. The arms and legs had minimal withdrawal and possible posturing.

33. The blood levels of magnesium and amylase were normal; other test results are shown in Table 1. Sodium bicar- bonate, insulin, glucose solution, and furose- mide were administered, and urine output in- creased (450 mm3 of urine over a 2-hour period).

34. A cannula was placed for distal perfusion, and ECMO was continued.

36. A chest radiograph showed bilateral diffuse consolidation that was most confluent in the left lower lung, an enlarged cardiac silhouette

37. bilateral layering pleural effusions, and the presence of medical devices, including a dual-chamber pacemaker and ICD and an ECMO catheter.

38. Electroencephalography re-vealed alternation of low-amplitude bursts with near-complete suppression of cerebral activity. Continuous venovenoushemofiltration was begun, without improvement in neurologic function.

39. Overnight, multiple units of red cells and fresh-frozen plasma were transfused, and albumin was administered. Heparin, insulin, vaso- pressin, and norepinephrine were administered. Results of a cosyntropin stimulation test were normal.

40. On the second day, computed tomography of the head revealed no evidence of hemorrhage or mass lesions. Repeat transthoracicechocardiog-raphy revealed severe diffuse left ventricular hypokinesis, with regional variation and a left ventricular ejection fraction of 13%

41. Fentanyl and midazolam were discontinued, and hydrocortisone was administered, without improvement in the patient’s level of consciousness.

42. Portable electroencephalography revealed generalized slowing with neartotal suppression of cerebral activity and without evidence of epileptiform abnormalities.

43. On the fifth hospital day, in consultation with the patient’s family, mechanical respiratory support was withdrawn and comfort measures were instituted; shortly thereafter, the patient died. An autopsy was performed.

44. Differential Diagnosis

45. Differential Diagnosis In summary, this 50-year- old man had a 15-year history of progressive distal weakness, delayed relaxation of muscle, and cardiac arrhythmias

46. causes of progressive distal weakness in adults? 1- Motor-neuron disorders include amyotrophic lateral sclerosis and spinal muscular atrophy. 2- Many types of peripheral neuropathies 3- Primary muscle disorders; several muscular dystrophies or inherited or acquired myopathies can be manifested by progressive distal weakness.

47. The progression of weakness that had occurred for several years before the electromyographic examination and the absence of compelling evidence of severe denervation suggest that a myopathy is a more likely diagnosis in this patient than is motor neuron disease or peripheral neuropathy.

48. a detailed neurologic examination revealed severe distal weakness that involved the limbs and some muscles of the face, head, and neck. Results of a sensory examination were mostly normal, thus confirming a motor process.

49. The patient had difficulty releasing his grip quickly, and tapping of the muscles of the forearm or thenar eminence produced muscle contractions that faded slowly; these findings suggest abnormal muscle activity and are important clues to the diagnosis

50. At this time, the patient reported that he had type 2 diabetes mellitus and that his father, sister, and one of his children had a similar neuromuscular disorder; this family history is suggestive of an autosomal dominant inheritance pattern