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Chapter 8. Inflammatory Diseases

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A 74-year-old man with no known history has had progressive dysphagia and cognitive decline over the past 8 weeks. He is brought to the emergency department (ED) with acute psychosis after he assaulted his family members and accused them of poisoning him. On examination, his blood pressure is 196/85, heart rate is 120 bpm, respiratory rate is 26 breaths/min, and pulse oximetry of 94%. Limited neurologic examination reveals symmetric/reactive pupils bilaterally, incomprehensible speech, prominent dysarthria, increased tone in all 4 extremities but relatively symmetric strength, brisk reflexes, and upgoing plantar reflex bilaterally.

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During the evaluation, the patient also exhibits frequent involuntarily jerking of his extremities, which is exaggerated when he is startled. A magnetic resonance imaging (MRI) scan is obtained, and diffusion-weighted imaging (DWI) sequence reveals the findings shown in the figure below. Despite extensive lab workup, toxicology, and several lumbar punctures including analysis for cerebrospinal fluid (CSF) 14-3-3 protein, no conclusive diagnosis is found. Unfortunately the patient’s symptoms continue to progress and he dies 6 weeks later. What is the most likely diagnosis in this patient?

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MRI brain, sequential axial diffusion weighted images.

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A. Intravascular central nervous system (CNS) lymphoma

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B. Anti-Hu encephalomyelitis

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C. Hashimoto encephalopathy

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D. Creutzfeldt-Jacob disease

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E. Acute ischemic stroke

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D. This patient presents with rapidly progressive neurobehavioral symptoms, a hallmark of Creutzfeldt-Jacob disease (CJD). Rapidly progressive mental deterioration and “startle myoclonus” as exhibited in this patient are 2 cardinal clinical features in CJD. In addition, the MRI reveals restricted cortical diffusion, which, although not specific, is classical for CJD in conjunction with the current clinical picture. CSF 14-3-3 is an adjunctive lab test but is neither specific nor sensitive, with numerous false-positive CSF 14-3-3 results reported in other neurologic disorders. A recent assay (RT-QuIC) has been proposed as a sensitive diagnostic test in CJD, with sensitivity and specificity of 85% to 87% and 99% to 100%, respectively. Unfortunately, there is no treatment for CJD, which is uniformly fatal but with a varied course of weeks to months.

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Choice B is incorrect. Paraneoplastic CNS disorder is an umbrella term for various autoimmune neurologic disorders. Anti-Hu is one of many of the known paraneoplastic orders, which is often multifocal affecting the temporal lobes, brainstem, cerebellum, dorsal roots and autonomic system. See Table 8-1. It is most often found in conjunction with small-cell lung carcinoma. However, even in its most aggressive form, survival is typically months to years. Choice C is incorrect since Hashimoto encephalopathy generally has a more favorable prognosis, and symptoms progress very slowly. Choice A is incorrect as intravascular lymphoma should not present with myoclonus; in addition, ...

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