A 46-year-old, right-handed, African American man presented to the emergency department after 1 week of persistent hiccups and 3 days of nausea and vomiting. His family member also noted that he had been experiencing right-hand tremor and gait imbalance for the last 3 months. Over the 6 months prior to presentation, the patient's coworkers had noticed that he was completing his work more slowly, that he was forgetting how to perform simple tasks, and that several times he had come to work at the wrong hours. The patient had a history of HIV diagnosed 10 years earlier and reported good adherence to an antiretroviral regimen consisting of tenofovir, emtricitabine, and atazanavir boosted by ritonavir, without any changes for several years. His viral load was undetectable when tested 8 months prior to presentation.The patient had experienced a 9-kg (20-lb) weight loss over the last 6 months and watery, nonbloody diarrhea for 3 days. He denied illicit or IV drug use, new sexual partners, recent travel, ill contacts, or toxic exposures.Neurologic examination revealed frequent hiccups; decreased short-term recall (1 of 5 words) after 5 minutes with normal registration; a 4-6 Hz, lowamplitude postural and action tremor of the right hand; appendicular ataxia of the upper limbs; and gait ataxia, especially prominent with tandem gait. The remainder of the physical examination was normal.Questions for consideration:1. Based upon the patient's history and neurologic examination, where do each of the patient's symptoms and signs localize? 2. Which are the most common neurologic causes of persistent or intractable hiccups? 3. What medications can be used for symptomatic treatments of persistent hiccups?
GO TO SECTION 2From the The patient's history and examination revealed executive dysfunction localizing to the frontal lobe and decreased recall localizing to the temporal lobe, both signs of cognitive dysfunction. The tremor of the right hand could localize to the left basal ganglia, the spinocerebellar tracts, or the rubrocerebellothalamic projections (Holmes rubral tremor). With the addition of cerebellar findings such as appendicular ataxia localizing to the lateral cerebellum, and gait ataxia localizing to the midline cerebellum, a cerebellar tremor is favored. However, rubral and cerebellar tremors can be difficult to distinguish because they have similar frequency and are present with action and at rest. The varying localizations should indicate to the clinician that this disease process is either multifocal or diffuse, rather than the result of a single, focal lesion. Hiccups, also called singultus, are paroxysmal myoclonic activations of the diaphragm involving a reflex arc with afferent vagal input, central integration in the medulla area postrema, and efferent output via the phrenic nerves.1 Ischemia, encephalitis, neuromyelitis optica, 2 or neoplastic lesions affecting the medullary integrating center including the area postrema are the most common primary neurologic causes, although many other causes have been de...