A 24-year-old woman, with history of recent upper respiratory infection, was admitted to the ICU with a week of progressive ascending weakness. On initial examination she was awake, alert, oriented, and cooperative. She had quadriparesis and areflexia. She subsequently developed respiratory distress and was intubated. Head and cervical spine CT are read as normal. A lumber puncture is performed, which reveals the following CSF profile:
What is the next best step in management?
Correct Answer: B
The patient described has a classic presentation of Guillain-Barré syndrome. This disease often appears following a febrile illness or a gastrointestinal tract infection. Typical features include ascending flaccid paralysis and a CSF sample with no WBCs and high protein. Nerve conduction and electromyography studies can be helpful to confirm the diagnosis; however, they are usually not needed to initiate treatment (Option C is incorrect). High-dose steroid therapy is not effective for Guillain-Barré syndrome (Option A is incorrect). First line treatment includes intravenous immunoglobulin or plasma exchange (Option B is correct). The patient’s clinical presentation is not suggestive of encephalitis as there are no clear signs of CNS involvement (no cortical deficits such as aphasia, or upper motor neuron pattern of injury) therefore Option D is incorrect.
A 38-year-old man suffers multisystem trauma and hemorrhagic shock after a motor vehicle accident. Initial CT imaging revealed a left-sided depressed skull fracture with underlying subdural hematoma (SDH) with contrecoup intracerebral hemorrhage, multiple bilateral rib fractures, lung contusions, and a splenic laceration. After initial damage control surgery, he was admitted to the ICU, where an increased intracranial pressure (ICP) monitor was placed. The patient remained sedated and ventilated, with limited neurological examination. On ICU day 2, he developed acute kidney injury with oliguria, and by day 3 he became anuric unresponsive to diuretic therapy. On the same day he had a sustained elevation in ICP to 27 mm Hg. Basic metabolic panel on day 3:
What is the next most appropriate step to manage the ICP elevation?
Correct Answer: C
The Cerebral edema and ICP are complications of severe TBI, which occurred in this patient. Cerebral edema in the settings of TBI is mainly cytotoxic and not vasogenic; therefore, steroids are not indicated (Option B is incorrect). The fastest and most effective intervention to reduce increased ICP in patients with TBI is osmotherapy. Although surgery might be indicated, osmotherapy is the initial therapy of choice (Option D is incorrect). There is no difference in outcome between the use of mannitol and 23.4% hypertonic saline in patients with increased ICP. However because mannitol’s effect is through diuresis, it may lead to systemic volume overload in an anuric patient. The correct answer is C. Hypertonic saline is the appropriate therapy to reduce intracranial pressure in a patient with TBI and ATN.
A 45-year-old man with a history poorly controlled hypertension is admitted to the ICU after elective endovascular repair of a descending thoracic aortic aneurysm. His intraoperative course was uneventful but on arrival in the ICU, he complains of lower limb weakness. His vital signs are within normal limits with blood pressure 110/65. On physical examination, he is awake, alert, and oriented. His cranial nerves and upper limb strength are intact. There is a clear nearly symmetric motor deficit in the bilateral lower limbs, with 3/5 weakness in the proximal muscles, and 2/5 in the distal ones. Tone is flaccid, and there is no Babinski sign or sensory deficit.
In addition to increasing the blood pressure what is the MOST appropriate next step in management?
Correct Answer: A
A devastating complication of thoracic aortic aneurysm repair is spinal cord ischemia. In open thoracic aortic aneurysm repair, cross clamping the aorta causes decreased blood flow to the spinal cord resulting in ischemia. In endovascular repair, such as in this vignette, there is no aortic crossclamp, but the endostent deployed can occlude small collateral arteries that perfuse the anterior spinal cord, which can result in hypoperfusion and spinal ischemia. Because of the anatomic blood supply of the spinal cord, the anterior motor fibers are supplied primarily by a single anterior spinal artery commonly resulting in bilateral leg weakness. Although there are no large randomized control trials, the common treatment aims at improving spinal cord perfusion by increasing MAP and inserting a lumbar drain for CSF removal. The result of these interventions is an increase in spinal cord perfusion pressure (SCPP), which is the difference between MAP and intraspinal pressure (ISP), i.e., SCPP=MAPISP. Although this treatment may be more effective when performed prophylactically before surgery, it may also be used as a rescue treatment when postoperative cord ischemia occurs (Option A is correct).
Obtaining an MRI may be important to rule out other causes of spinal cord ischemia, such as spinal cord hematoma but would not be the immediate next step (Answer B is incorrect). Steroids have no demonstrated role in spinal cord ischemia (Option C is incorrect). The mechanism of spinal cord ischemia in this case is due to the endo-stent occlusion of perfusing arteries resulting in decreased anterior spinal cord perfusion; therefore, IR-guided thrombectomy will not treat the underlying problem (Option D is incorrect).
A 78-year-old woman with past medical history of hypertension, COPD, and coronary artery disease is admitted to the ICU with respiratory failure due to community-acquired pneumonia. She is intubated and mechanically ventilated. Empiric antibiotic therapy for pneumonia was initiated following obtaining cultures. On the morning of her third ICU day, she his noted to be less arousable when sedation is decreased. On examination she does not follow commands, has a gaze preference to the left, and only moves her left side spontaneously. A stat head CT does not show any acute findings. The last documented normal neurological examination was at the shift change the day prior. The patient’s home meds include a baby aspirin (81 mg) and a statin.
What is the MOST appropriate intervention at this time?
Correct Answer: D
The patient described in the vignette demonstrates evidence of an ischemic stroke, an infrequent but important complication in critically ill patients. Timely detection is challenging because physical examination can be limited by sedation, delirium, or other medications, which can make acute neurologic changes difficult to detect. The patient probably has a LMCA syndrome based on the description. Because her last known normal examination was over 4.5 hours before the diagnosis, she is out of the window for IV thrombolytic therapy (Answer A is incorrect). However, in the era of endovascular thrombectomy, there are still urgent interventions that could change the course and outcome. Although a seizure is in the differential when an acute neurological change occurs, the gaze deviation (to the left in this case) suggests that this is a stroke and not a seizure (Option C is incorrect).
According to the 2018 American Stroke Association, there is enough level I data to recommend pursuing a CT angiogram and CT perfusion study to assess the stroke burden even up to 24 hours from the time of last known normal examination. Several randomized controlled trials have shown the benefit of patient-selective approach to find the correct candidate for mechanical thrombectomy. Therefore, it is not necessarily too late for this patient to receive timely and potentially beneficial intervention (Option D is correct).
A 32-year-old woman is admitted to the hospital with a 2-week course of progressively worsening confusion. She has no past medical history except for a mild upper respiratory infection two weeks before admission. On examination, she is awake, not oriented to time, place, or person and is only able to follow simple commands. There are no obvious focal neurological deficits. During the assessment, she develops a generalized tonic-clonic seizure. The seizure does not break despite three 2 mg doses of IV lorazepam and is emergently intubated and a propofol infusion is initiated, which terminates the convulsions. She is treated empirically with antibiotics for bacterial meningitis and acyclovir for viral encephalitis. EEG monitoring is notable for left temporal focal nonconvulsive status epilepticus, and fosphenytoin is administered with resolution of the seizures is administered. A head CT is obtained but does not show any acute abnormality. An MRI of the brain shows bitemporal T2 hyperintensities and no sign of a space occupying lesion, hemorrhage, or stroke. A lumbar puncture is performed, and CSF content shows:
PCR for HSV 1&2, CMV, EBV, and VZV are negative, and acyclovir is discontinued. Gram stain and cultures are negative.
What is the MOST appropriate therapeutic intervention at this time?
The patient in this scenario presents with a clinical picture of autoimmune encephalitis. The subacute progressive process, new onset seizures, and the temporal findings on imaging, all point toward the diagnosis. As part of the workup, it is critical to rule out other causes including infections (viral and bacterial), neoplastic etiology, toxic metabolic syndromes, posterior reversible encephalopathy syndrome (PRES), and a central manifestation of a systemic autoimmune disease (SLE, Sjögren, Behçet, etc). Once other potential diagnoses are excluded, early treatment with high-dose steroids and plasma exchange or IVIg is recommended (Option A is correct).
Although it is possible to have an early false negative PCR for HSV, this patient has been symptomatic for two weeks, and therefore the chances of false negative PCR are low (Option B is incorrect).
The patient is treated with a second and third line agents to control her seizures (phenytoin and propofol), with a good response. There is no advantage at this point in adding another antiepileptic agent, particularly valproic acid because of the significant interactions it has with phenytoin (Option C is incorrect). As for seizure control, the aim is to control all seizures, focal or generalized, with medications, and one cannot “tolerate” focal status epilepticus to achieve other clinical goals such as an examination (Option D is incorrect).
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