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Zorluk: Çok zorNeurocognitive Disorders

A 71-year-old retired architect is brought to the neurologist by his daughter due to a 9-month history of progressive cognitive decline. Initially, he began having difficulty organizing his finances and interpreting architectural blueprints. Six months ago, he started experiencing vivid episodes during sleep where he thrashes around and shouts, sometimes injuring his wife. Over the last 3 months, he has reported seeing silent, unfamiliar children playing in his living room, though he is generally not distressed by them. He has a history of major depressive disorder treated with sertraline. Two weeks ago, his primary care physician suspected an underlying bipolar diathesis but ultimately only increased his sertraline dose. A few days later, he was prescribed linezolid at an urgent care clinic for a suspected skin infection. A thyroid panel obtained last week showed a TSH of 0.1 μU/mL (normal: 0.4-4.0 μU/mL) and a free T4 of 0.6 ng/dL (normal: 0.9-1.7 ng/dL). On physical examination today, his temperature is 37.1°C (98.8°F), blood pressure is 128/82 mmHg, and heart rate is 76/min. He has a mask-like facies and increased resting muscle tone with a catch-and-release pattern in both arms. Deep tendon reflexes are 2+ bilaterally without clonus. His Mini-Mental State Examination score is 21/30. Which of the following is the most likely diagnosis?

  1. Major neurocognitive disorder with Lewy bodiesCevap
  2. B
    Substance/medication-induced bipolar and related disorder
  3. C
    Serotonin syndrome
  4. D
    Major neurocognitive disorder due to primary hypothyroidism
  5. E
    Schizophreniform disorder

Cevap

Major neurocognitive disorder with Lewy bodies
The correct diagnosis is major neurocognitive disorder with Lewy bodies (DLB). DLB is characterized by progressive cognitive decline (often presenting with early executive and visuospatial deficits, as seen with this architect's difficulties) along with at least two core clinical features. This patient exhibits three core features: REM sleep behavior disorder (dream enactment behavior), recurrent well-formed visual hallucinations (silent children in the room), and spontaneous parkinsonism (mask-like facies, cogwheel rigidity). The timeline, with cognitive decline and parkinsonism occurring within 1 year of each other, differentiates it from Parkinson disease dementia.

Adım Adım Çözüm

1
Analyze the timeline and core symptoms of the patient's cognitive decline.
The patient has a 9-month history of executive dysfunction, 6 months of REM sleep behavior disorder, and 3 months of recurrent visual hallucinations.
Establishing the sequence and nature of symptoms is crucial for differentiating primary neurocognitive disorder subtypes.
2
Evaluate the physical examination findings.
The exam reveals mask-like facies and cogwheel rigidity (catch-and-release pattern), which are distinct signs of parkinsonism.
The presence of spontaneous parkinsonism, combined with the core symptoms identified in Step 1, strongly suggests Dementia with Lewy bodies.
3
Rule out iatrogenic and comorbid conditions that could mimic the presentation.
Serotonin syndrome is ruled out due to the timeline (symptoms predating linezolid) and lack of hyperreflexia/clonus. Medication-induced mania is unlikely given the absence of classic manic symptoms. Thyroid dysfunction is present (central hypothyroidism) but does not fully explain the classic triad of visual hallucinations, REM sleep disorder, and parkinsonism.
To confirm the primary neurocognitive diagnosis, acute delirium or substance-induced states must be excluded as the primary etiology.

Anahtar Kavram

Diagnosis of Dementia with Lewy bodies relies on identifying progressive cognitive decline alongside core features such as fluctuating cognition, recurrent visual hallucinations, REM sleep behavior disorder, and spontaneous parkinsonism.
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