Amyotrophic lateral sclerosis (ALS)
Patient presentation
R.B. is a 67 year old male with a known history of hypertension, arthritis, and heartburn. Patient saw his neurologist one month prior to admission and it was noticed that he had wasting of his intrinsic hand muscles, muscle twitching, and weight loss. His neurologist was concerned that R.B. had ALS. Five days later R.B. presents to the emergency room with increasing progressive weakness, shortness of breath, and loss of appetite. Upon assessment patient is lethargic, and unable to answer questions.
Vital Signs
HR 75 with Normal Sinus Rhythm, BP 170/85, RR 14, SaO2 100%, Temp 98.8
Labs
BMP: Na+ 141, K+ 4.9, BUN 15, Creatinine 0.9
Troponin I: 0.013
Thryroid panel: T3 free 2.48, T4 free 1.27, TSH 0.320
CBC: WBC 4.1, Hgb 16.4, Hct 48.9, Platelet 186
ABG on 100% nonrebreather mask: pH 7.115, PaCO2 167.5, PaO2 115, HCO3 52.6, B/E 14
Based on initial ABG and clinical presentation, patient was intubated for respiratory distress. R.B. was sent to intensive care for further treatment and management.
Diagnosis
ALS was confirmed by EMG studies and clinical picture.
Acute Respiratory failure secondary to ALS.
Pneumonia — possibly aspiration related.
Treatment
R.B. was started on Riluzole 50 mg q 12 hours for his ALS. CT of the chest on admission revealed patchy infiltrates in the right upper lobe. Respiratory cultures revealed H. influenzae pneumonia and patient completed course of Zosyn® IV. Additionally, Mucomyst® nebulizer treatment every 4-6 hours PRN and percussion and vibration therapy every 2-3 hours were used.
One week after admission, repeat CT of the chest revealed collapse of the right lower lobe bronchus, and bilateral infiltrates. Patient underwent bronchoscopy and was found to have a collapsed right lung from a mucus plug occluding the right main bronchus. Bronchial washing was performed.
R.B. failed weaning trials and did not have a gag or cough. It was decided to perform tracheostomy and PEG tube placement almost three weeks post admission.
Outcome
Patient R.B. was transferred to a long-term acute ventilator facility for further therapy and rehabilitation. Upon transfer R.B. continued to be ventilator dependent with following settings: AC 18, PEEP 8, Tidal volume 500 ml, FiO2 45%. Patient is now awake and follows commands, smiles at visitors and staff members, and is able to weakly move his upper and lower extremities to command.
Case created by Ronelia Balmoris, 2011.