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This case report discusses a 67-year-old male patient with a history of GI bleeding who developed generalized weakness, fatigue, fever, and cognitive decline. The patient was diagnosed with central pontine myelinolysis based on MRI findings and clinical presentation.
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Patient InitialsClincopathologic Conference (CPC)Date: 3/4/16 Neurology Resident: Brian Hanrahan
HPI: Admission 2/8/16 • Patient is a 67Y M with a PMH of MI (2010, 11/2015), GI bleeding, recurrent bladder cancer, DVT and PE’s on warfarin who presented with generalized weakness, fatigue and fever with recent GI bleeding. • On admission had SBP 103, appeared pale and diaphoretic. Also had melanotic stools with gross hematura. • Lab studies showed Hb 5.9, INR of 7.2. The patient was transfused and received vitamin K and fresh frozen plasma.UGI endoscopy shows gastric ulcer which was treated with thermal therapy. • Stabilized and transferred to the ICU for BP stabilization, monitoring after GI bleed and FUO/concern for sepsis
Change in mental status • On 2/10/16 patient started to have “intermittent confusion” For this psych was consulted. MRI and EEG was also performed. Per psych review on 2/10: “He has had periods when he does not know where he is, the date, or why he is here. When I spoke with him he knew he was in SMH but did not know why, how long. He gave date as March 2015. He appears to be distracted, often gazing off in the distance. He denies prior psychiatric problems or treatment, but I see he has record of being tx with Celexa.” “Pt's wife said pt has no existing cognitive deficits. She notes that he has had worsening medical problems and with three hospitalizations since October. After his hospitalization in November, "he was not the same person". He had episodes of visual hallucinations (e.g., seeing a truck in the yard when there was none, presented wife with a "big strawberry" when his hands were empty). He always recognizes family but sometimes misenterprets what is happening around him. He has had several episodes that she felt were seizure-like when he would seems to lose his balance, fall backward, "eyes roll back" and hands would shake. He would have ~ 5 minutes of confusion after these episodes.” “His appetite is variable and he sometimes refuses to eat, even specially prepared foods. He is not able to read, is anergic and generally uninterested in things he used to enjoy. He is normally an easygoing, accomodating person but now has more frequent episodes of irritability” “Wife endorses hx of heavy drinking for many years. He stopped drinking a year ago because beer no longer tasted good to him.” “He is frequently disinhibited, saying things that are somewhat rude and that he would never say before. He does not appear to connect with other people like before. He has poor planning, poor organization, poor judgment. His energy and interest are extremely poor. Psych DDx: The patient likely has significant delirium given his very poor attention, visual hallucinations, and disorientation.
Examination 2/12/16 • Mental Status: Alert, inattentive, oriented to person, St. Margret's hospital and 2016 but not month. • CN: EOMI, Face symmetrical, no aphasia • MOTOR: Move UE well, withdraws LE • SENS: ? • REFLEX: ? • COORD: ? • GAIT: ?
Hospitalization cont. • 2/11-2/18: Patient continued to have recurrent fevers and periods of hypotension. Continued concern for septic shock despite negative cultures and broad spectrum abx. • Patients mental status continued to fluctuate. • On 2/18: Exam worsened. Stroke code called. Found to be in SVT with resultant hypotension requiring adenosine. Repeat MRI ordered. • NIHSS b/w 2/11-2/18 over hospitalization:
Hospitalization Cont. • 2/19-patient more awake today but remains confused slurred speech on 4 L nasal cannula no fevers • 2/20-Remained unchanged from prior, however patient was placed up in chair and found to be unconscious, hypoxemic and in respiratory arrest. CPR was initiated per the ACLS protocol. He was found to be in VT/VF and shocked multiple times without ROSC
Differential Diagnosis • MRI findings 2/2 pontine stroke vs. central pontinemyelinolysis • AMS 2/2 Delirium, underlying dementia or systemic illness • Unifying pathologic process? Autoimmune vs. Paraneoplastic
Central Pontine Myelinolysis • Oligodendroglia are most susceptible to CPM related osmotic stress • Microscopic findings • Sharply demarcated areas of pallor representing myelin loss. • In initial stages may have preservation of axons • Eventual increase of macrophages and astroglia • In severe cases can become necrotic Luxol fast blue stain used to observe myelin Macrophages with intracellular myelin fragments
Bilateral Medial Medullary Infarction • Quadriparesis observed in only 9% of cases (asymmetry of the lesions). • If dorsal pons spared then gaze is preserved. • Nystagmus is common • Most often causes of atherothrombotic disease obliterating the perforating branches.
Branch occlusion of the pontine perforators 2/2 atherosclerosis of the vertebro-basilar junction https://books.google.com/books?id=vRwnCgAAQBAJ&pg=PA429&lpg=PA429&dq=pons+%22heart+shaped%22+stroke&source=bl&ots=LlnXNg6yH9&sig=HuzlvwuakdkoJuiZGVUiCxPi8zo&hl=en&sa=X&ved=0ahUKEwiu5MLf9KDLAhWBWh4KHYo0ArsQ6AEIIjAB#v=onepage&q&f=false
Stroke pathology @ 1-2 weeks • Subacute changes • Resolving edema from initial insult • Neovascularization occurs • Clear demarcation of lesion, soft friable tissue • Increasing presence of macrophages (peaks at 3-4 weeks) • Starting process of liquifactive necrosis • Would also like to look at Vertebro-basilar junction for any thrombus or atherosclerosis
Virtual Microscopy • Pons Slide G • H&E • CD20 • LFB/PAS • Neurofilament • MBP • GFAP • Medulla Slide J • H&E • CD20