A 28‐year‐old white man was transferred to our facility from an outside hospital for 1 week of fatigue and cough. He smoked half a pack a day and had a history of asthma for which he took an albuterol inhaler twice daily. He denied any fever or sick contacts. In the emergency department his respiratory status rapidly declined. Chest X‐ray showed a large pleural effusion on the right side (figure). He was intubated and placed on mechanical ventilation. His white count was 23,000 K/μL with 8% bands, and albumin was 2.9 g/dL Broad‐spectrum antibiotics were started. A CT scan of the chest showed right‐sided loculated pleural effusion with collapse of the right middle and lower lobes. A liter of serosanguineous, exudative fluid was drained via thoracentesis, and a chest tube was placed. The patient was successfully weaned off the ventilator but had to be placed back on it within a few hours of extubation. Bronchoscopy did not reveal any significant secretions or airway stenosis. A repeat CT of the chest showed persistent pleural fluid, and the patient underwent decortication and parietal pleurectomy. After this procedure several attempts at extubation failed. Pleural tissue peel grew Staphylococcus non‐aureus, Streptococcus viridans, and Prevotella melaninogenica. This was thought to be secondary to recurrent aspiration of oropharyngeal flora. A transthoracic echo showed mild pulmonary hypertension. All autoimmune workup and HIV testing results were negative. Electromyogram (EMG) and nerve conduction studies (NCS) were done, and findings were those of a myopathy with myotonic features. The patient was diagnosed with myotonic dystrophy type 1. He eventually got a tracheostomy and a percutaneous gastrostomy and was discharged to a chronic nursing facility for further therapy.
Myotonic dystrophy is an autosomal‐dominant multisystem disorder with a prevalence of 1:8000. There are 2 types. DM 1 (Steinert's disease) commonly involves facial, neck, forearms, hand intrinsic, and foot dorsiflexor muscles. Respiratory and swallowing complications are more common. Limb girdle (upper and lower) involvement occurs in DM 2 (proximal myotonic myopathy), and muscle pain is often a presenting symptom. Heart blocks, testicular failure, and cognitive impairment can occur in both types. Diagnosis is through EMG and NCS. Treatment is via a multidisciplinary approach with yearly electrocardiogram, orthotics, speech therapy and genetic counseling. Average life expectancy is 50 years, and the most common cause of mortality is pneumonia.
Myotonic dystrophy, although a rare disorder, should be suspected in young patients with recurrent aspiration pneumonia and respiratory failure. This patient carried an outpatient diagnosis of asthma, which was probably muscle weakness from the myotonic dystrophy. Early recognition can prevent significant comorbidities and decrease mortality.
F. Haider, none; 0. Rahman, none.
To cite this abstract:Haider F, Rahman O. Young Man with Respiratory Failure. Abstract published at Hospital Medicine 2009, May 14-17, Chicago, Ill. Abstract 157. Journal of Hospital Medicine. 2009; 4 (suppl 1). https://www.shmabstracts.com/abstract/young-man-with-respiratory-failure/. Accessed January 21, 2020.