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Diagnosis Deconstructed

Diagnosis Deconstructed: A Surgical Strike

Morchi, Ravi MD

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doi: 10.1097/01.EEM.0000432267.33911.45
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    I know her. She looks like she did the other day. I move to shake her hand. That must be the tremor they mentioned. I guess it's back. A high-frequency oscillation upon intention only.

    She appears alert and attentive but anxious. Her mother insists that she still has memory problems, stopping mid-sentence at times and unable to continue. The patient endorses feeling nervous, and is aware of her own confusion.

    Tachycardia at 130; nothing else on cardiopulmonary exam. Other than a fine intention tremor, mild exophthalmos, and a lean appearance, her neural and endocrine exams are normal. A gravid uterus is palpable just a few centimeters above the pubic symphysis. Otherwise, she has a soft abdomen and no organomegaly. Extremities are warm and not edematous. No skin lesions.

    Yesterday. She was seen for the same. The patient is a 24-year-old African-American G2P0 with one elective abortion presenting for two days of memory issues and “not behaving herself.” She recently lost 20 pounds, has a history of depression, and was previously on SSRIs. Ultrasound noted a normal IUP in the late first trimester.

    “I have a patient I want you to take a look at and see what you think,” her attending says to me. He goes on to describe mild confusion and a tremor. He is looking for a second opinion.

    She looks and feels better on my exam, having already received a liter of saline and a small dose of lorazepam. HR is 100, no hyperkinesia. Exophthalmos but no goiter. I presume the appearance is normal for her. She can rattle off answers to basic questions, and does not seem confused.

    “It looks to me like she was just anxious and a little dehydrated,” I say reassuringly. I don't think a TSH is going to give us an answer, but we can send it. Toxicology testing is negative, but maybe she took a stimulant that remains undetected on our screen. Maybe the pregnancy has her nerves on edge. The last one she did not want, and this one she also plans to abort.

    But she can't interlock pentagons. She could not recreate an image of overlapping shapes. She could not recall three items at five minutes. It was the MMSE that concerned my colleague.

    How much weight should I put on this? Effort-related? Administered by our resident before treatment, was the patient so frantic at the time that she could not concentrate on details? I certainly do not perform an MMSE on all anxious patients so I cannot say. Had they uncovered something meaningful? Or background noise? Maybe they just need a little encouragement to discharge this one.

    Today, second visit. My resident and I spend a few more minutes speaking to her with mom out of the room. And then it surfaces. She pauses mid-sentence at times. I ask why. She cannot say. Not that she cannot say, but that she should not say. For fear of repercussions. Repercussions from someone watching and listening? Someone other than the two of us? Hmmm. Time for the tough questions.

    “Why do you not want this baby?” She is convinced she has to be rid of it. I persist. Why? Because God has told her so. Told her she must abort. Or else? Bad things will befall her.

    I sense that she feels herself to be cursed. She pauses, and her eyes dart from side to side. I suspect she is withholding information. “Oh, no,” she mutters, worried she has already said too much. Concerned she cannot divulge the truth, lest all three of us are drawn into the hex.

    “Tell me about the voices you are hearing.” I normally avoid leading questions, but on this occasion, I need to push my way in. “What are they saying to you?” Her delusion is fixed and consistent. Paranoia and irrational fear leave her volitionally mute. Responding to internal stimuli, her eyes stray from mine, landing in the corners of the room. Has the pregnancy accentuated and lured out what had been brewing in the background for some time now?

    Psychotic break. It takes more than a moment to dive deep enough into conversation to uncover psychosis. We often gloss over it in our superficial ED interaction. The stories become more elaborate, more fantastically orchestrated and enthralling if we stay engaged for longer periods.

    I review her labs in the doctor's room to ensure she is medically cleared for a psychiatric evaluation. A head CT done on her first visit is officially read as normal. Her chemistry profile was normal. HIV screen negative. Thyroid panel from yesterday returned normal. But then things become not normal.

    UA. 3+ protein yesterday. Today it is the same. Not hypertensive or edematous. This is unlikely to represent preeclampsia in the first trimester of a single IUP. No diabetic or hypertensive nephropathy at baseline. This degree of proteinuria is not normal.

    CBC. Anemic to 8. And unlike so many of our iron-deficient women with menometrorrhagia from fibroids or hyperplasia because of hormonal imbalance, this patient's anemia is not microcytic. It is normocytic. Normocytic is not normal.

    Persistence in symptomatology and consistency in objective abnormalities make us change our thought process for any patient with the same complaint. We cannot simply pursue anxiety, dehydration, or, in other circumstances, gastritis or viral syndrome indefinitely. The return of the patient signifies a shift away from the typical and toward the atypical. Typical entities after all are often accurately named on first pass. We have to question our prior logic once a patient has two or three or four passes at the ED. If it ever was typical, then the diagnosis and therapy from earlier on would have averted the repeat visits. The very fact that she exists today, for the second time in as many days, means the atypical is creeping to the forefront.

    “I think we need to do an LP.” I sigh, and look to my resident's response. He immediately says OK. No startled eyes or look of disappointment. That was easy; I thought it would take more convincing. If we are both on the same track, maybe we are both right.

    CSF. 61 WBCs, 82% lymphocytes. Protein 280. Glucose 43. We found something.

    But what are we looking for? Not standard bacterial meningitis. This is not the presentation of meningeal inflammation, not just because she lacks meningismus or photophobia or fever but because she is alert, entirely attentive, and interactive, and she has no pain. She has only cognitive features: a fixed, consistent delusion and auditory hallucination. Nothing more. This is subtle. If a medical disease is behind this specific type of neuropsychiatric behavior, it is not a war going on in the meningeal space. It is not indiscriminate inflammation of arachnoid, dura, and neighboring brain tissue, with grunt, infantry neutrophils degranulating and phagocytosing. It is not a crude attack or random act of violence against whomever happens to be in the area. It is not bacterial meningitis or meningoencephalitis.

    What I am chasing is much more sophisticated. An illness targeting one particular part of the brain. Engineered by intelligent, conniving, mature cellular machinery deployed for a particular purpose. Lymphocytes with their eyes on one tissue, one cell, one receptor. This is a targeted attack. Only a surgical strike successfully carried out can mimic psychosis and leave attentiveness and level of alertness entirely untouched. Standard bacterial inflammation generally cannot. Tuberculous, fungal, and spirochetal meningitis are also not this discerning in their assault. Viral encephalitides must be considered, but even HSV generally affects consciousness as well as cognition.

    The culprit in my mind? It can be antibody-mediated in response to a prior, now-inactive infection (post-infectious encephalitis) or in response to neoplastic cells that reside somewhere else in the body (paraneoplastic). Or it can be primarily autoimmune.

    My thoughts turn to the recently described paraneoplastic limbic inflammation of young parous women: anti-NMDA receptor encephalitis. Antibodies originating in response to an undiagnosed tumor, possibly teratoma, act on the CNS NMDA receptors to produce memory deficits and cognitive disturbance often unaccompanied by changes in level of consciousness. Has it been described in relation to a normal IUP? Maybe she has a teratoma as well?

    The other immune phenomenon crafty enough to pull off the surgical strike of stand-alone psychosis? Neuropsychiatric, CNS Lupus. The most sophisticated of any of the autoimmune entities we come across. It is not simply destruction aimed at an organ. It is an incompletely understood attack, calculated and focused at a cellular and molecular level.

    Her MRI/MRA was negative as an inpatient. A repeat pelvic ultrasound did not show a teratoma. Extensive workup for an infectious cause produced nothing. Her ANA profile turned out strongly positive. C3 and C4 levels were indicative of complement activation. Proteinuria and normocytic anemia both secondary to SLE, tachycardia the accompaniment of systemic inflammation and complement consumption. She was treated with glucocorticoids and plasma exchange. An interdisciplinary discussion among rheumatology, neurology, OB-Gyn, and the primary medical service sided with therapeutic abortion to curb worsening neuropsychosis and avert what they predicted would be an otherwise poor prognosis.

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