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Ted is a 67-year-old male with no significant past medical history, except for occasional migraine headaches. He is married, has two grown children, and is retired from the Air Force. He does not smoke and does not use alcohol. He has no regular exercise program, but does try to eat healthy. On June 15, Ted awoke with pain in his chin and jaw that radiated to his left ear. The next day, he visited his physician, as the pain had become more intense. He described the pain as severe, sharp, and constant. His physician could find no cause for the facial pain, and convinced it was likely a dental problem, he advised Ted to see his dentist. 

Since the pain remained constant with unremitting intensity, Ted saw his dentist the next day. He was prescribed amoxycillin for a likely developing dental abscess and was to return in 1 week for definitive treatment. When he returned to his dentist 1 week later, the pain had somewhat subsided, but no evidence of a dental abscess could be found either by x-rays or clinical symptoms.

Over the next two days Ted was not improved. He continued to have fever, and he described shooting pains in his face, accompanied by pain in his back and legs. He was brought to the emergency room by family members for the fever pain, and dizziness. He was treated for viral syndrome and discharged with amitriptyline, an antiviral medication, oxycodone for pain, and meclizine for nausea. Throughout the following evening, Ted’s nausea worsenede, the fever increased, and his mental status declined. His family noticed mild confusion, particularly with timing of events and short-term memory. He also developed a rash on his lower legs, both axillae and over his shoulders. Ted’s family brought him back to the emergency department because of persistent fever, worsening mental status, and the inability to keep down his food. 

Upon this third visit to an emergency department, Ted was found to be in obvious discomfort, mildly confused, but oriented to person and place. He had no history of trauma or seizures and demonstrated no photophobia. He did experience left-sided neck pain. Pupils were equal and reactive to light. Tympanic membranes were clear bilaterally, and his oropharynx and nasopharynx were dry without swelling or drainage. He had slight crackles in the right lung indicating the presence of fluid, but the left lung was clear. Neurological exam revealed cranial nerves II-XII were normal. Sensation was equal bilaterally in upper and lower extremities. Muscle strength was normal in all extremities. Vital signs were as follows: temperature 102.8F, pulse 98 bpm, normal heart rhythm, blood pressure 136/79 mm Hg, respiratory rate 24/min, and weight 96 kg.

Question 1 – There are several signs and symptoms here, but what part of Ted’s body do you think should be the focus of further testing?

During the 2 hours in the emergency department, while awaiting laboratory and X-ray results, Ted became more confused, failed to recognize family members, and ceased to follow commands. A computed tomography (CT) scan of the brain was done, which was normal. A lumbar puncture was performed with the following results: protein 101, glucose 89, nucleated cells 960, segmented neutrophils 78. The cerebrospinal fluid (CSF) was slightly cloudy, but colorless.

Question 2 – Ted is getting worse. Carefully looking at the laboratory tests, what do they suggest?

The attending physician ordered Ted be admitted to intensive care. With the hospital being at high census, Ted was kept in the emergency department for the night awaiting a monitored bed in a respiratory isolation room. He was started on intravenous (IV) normal saline at 150 ml/hr, clear liquids as tolerated, vancomycin 2 grams IV every 12 hours, and metoclopramide 10 mg every 3 hours as needed for nausea.  Ted was received in the medical intensive care unit (ICU) the following morning. Upon admission to the ICU, Ted was slowly moving his extremities. He did not open his eyes to voice or pain, and did not follow commands or recognize family members. Vital signs were as follows: temperature 102.6 [degrees] F, pulse 116 bpm, respiratory rate 36/min, and blood pressure 118/67 mm Hg. His breathing was rapid, shallow, and labored. An arterial blood gas sample showed his oxygen intake was below normal. He experienced increasing respiratory distress with crackles and wheezing in both lung fields. A repeat chest X ray revealed bilateral pulmonary edema. A pulmonary consult was ordered, and Ted was intubated and placed on mechanical ventilation due to clinical respiratory failure and inability to control his airway. Ted’s pulmonary status continued to worsen over the next 3 days, with increased airway pressures, decreased pulmonary compliance, and increased oxygen requirements. His chest X ray, as well as clinical evidence, indicated adult respiratory distress syndrome (ARDS).

Question 3 – What organ system is being affected the most?

Over the next 7 days, Ted remained neurologically unresponsive, mechanically ventilated, and received sedation with lorazepam and morphine to achieve comfort and maintain ventilator synchrony. His mental status did not improve even during short periods of time off sedation. Supportive care consisted of enteral feedings via gastric tube, physical therapy to maintain muscle tone, and fever management. During one of several conversations with the attending nurse, Ted’s wife recalled he had a large mosquito bite on his left ear before leaving home 2 weeks earlier.  By day 10, Ted had made some improvement and was responsive to voices and able to follow simple commands. On day 11, Ted was sleepy but arousable, and slowly followed commands. Due to improved pulmonary function, the physician ordered Ted be removed from the ventilator. He tolerated it well and maintained appropriate oxygen saturation levels. He denied breathing difficulties. 

Question 4 – Ted is getting better. Any new information that gives you a clue as to Ted’s diagnosis? 

Laboratory Findings typically seen with Ted’s disease:

* Total leukocyte (white blood cell) counts in peripheral blood were mostly normal or elevated with low lymphocyte counts and anemia also present. 

* Hyponatremia (low blood sodium levels) was sometimes present.  

* CSF showed presence of white blood cells ally with a predominance of lymphocytes. Protein was universally elevated. Glucose was normal.

* CT of brain usually did not show evidence of acute disease, but in about one-third of patients, magnetic resonance imaging (MRI) showed swelling of the brain.

Treatment is supportive, involving hospitalization, IV fluids, respiratory support, and prevention of secondary infections for those with severe disease such as Ted. Potential complications of the disease include decreased level of consciousness, irritability, muscle weakness, respiratory complications, immobility, and nutritional risks. Ted demonstrated all these complications, with the gravest being markedly decreased level of consciousness and profound respiratory distress.

Ted’s lab results were, for the most part, consistent with these findings. His WBC was normal, but did show slight lymphocytopenia. However, he was not anemic. The CSF analysis revealed elevated protein, and normal glucose levels. The CT of his brain was normal.  Signs and symptoms of mild disease generally last a few days. However, signs and symptoms of severe disease may last several weeks, and some neurological effects may be permanent. Children recover faster than adults. Muscle weakness and pain can persist for an extended period of time. 

Ted suffered severe disease and at the time of this writing, 6 weeks after symptom onset, he still experiences muscle weakness, restlessness, and slow response times. His family states that when he is fully awake, his mental status is becoming more normal.

Question 5 – Here it is; what disease did Ted suffer from?

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