Objective: To investigate the misdiagnosis of area postrema syndrome (APS) manifesting as intractable nausea, vomiting and hiccups in neuromyelitis optic spectrum disease (NMOSD) and reduce the risk of misdiagnosis. Methods: We retrospectively analyzed data from NMOSD patients attending the Department of Neurology at the First Medical Center of PLA General Hospital between January 2019 and July 2021. SPSS25.0 was then used to analyze the manifestations, misdiagnosis, and mistreatment of APS. Results: A total of 207 patients with NMOSD were included, including 21 males and 186 females. The mean age of onset was 39±15 years (range: 5-72 years). The proportion of patients who were positive for serum aquaporin 4 antibody was 82.6% (171/207). In total, 35.7% (74/207) of the NMOSD patients experienced APS during the disease course; of these patients, 70.3% (52/74) had APS as the first symptom and 29.7% (22/74) had APS as a secondary symptom. The misdiagnosis rates for these conditions were 90.4% (47/52) and 50.0% (11/22), respectively. As the first symptom, 19.2% (10/52) of patients during APS presented only with intractable nausea, vomiting and hiccups; 80.8% (42/52) of patients experienced other neurological symptoms. The Departments of Gastroenterology and General Medicine were the departments that most frequently made the first diagnosis of APS, accounting for 54.1% and 17.6% of patients, respectively. The most common misdiagnoses related to diseases of the digestive system and the median duration of misdiagnosis was 37 days. Conclusions: APS is a common symptom of NMOSD and is associated with a high rate of misdiagnosis. Other concomitant symptoms often occur with APS. Gaining an increased awareness of this disease/syndrome, obtaining a detailed patient history, and performing physical examinations are essential if we are to reduce and avoid misdiagnosis.
Male ; Female ; Humans ; Child, Preschool ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Neuromyelitis Optica/diagnosis* ; Area Postrema ; Retrospective Studies ; Hiccup/complications* ; Vomiting/etiology* ; Nausea/etiology* ; Inflammation ; Syndrome ; Autoantibodies ; Diagnostic Errors ; Aquaporin 4

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating autoimmune disease of central nervous system characterized by relapsing attacks that target the optic nerves and spinal cord, as well as aquaporin-4 (AQP4) enriched periventricular brain regions. The area postrema (AP), located in the dorsal medulla, is the chemosensitive vomiting center and has high AQP-4 expression. The AP syndrome with unexplained hiccups, nausea, and vomiting is one of the core clinical characteristics in the NMOSD and maybe the first presenting symptom. We experienced a 25-year-old woman presented with intractable vomiting, dizziness and oscillopsia. Upbeat nystagmus detected on the bedside examination led to comprehensive neurological workups including magnetic resonance imaging, and she was diagnosed as the AP syndrome. Ten months later, she experienced a recurrence as a longitudinally extensive transverse myelitis and the diagnosis was finally compatible with NMOSD without AQP4-IgG. NMOSD, especially the AP syndrome, should be considered in any dizzy patient with intractable vomiting, and detailed neuro-otologic and neuro-ophthalmologic examinations are warranted for the correct diagnosis.
Adult ; Area Postrema ; Autoimmune Diseases ; Brain ; Central Nervous System ; Diagnosis ; Dizziness ; Female ; Hiccup ; Humans ; Magnetic Resonance Imaging ; Myelitis, Transverse ; Nausea ; Neuromyelitis Optica ; Nystagmus, Pathologic ; Optic Nerve ; Recurrence ; Spinal Cord ; Vomiting

Hiccup is an intermittent, involuntary and erratic contraction of the diaphragm, immediately followed by a laryngeal closure. Persistent and intractable hiccups are rare but severe, keeping a person from doing daily activities; these can result in depression, fatigue, impaired sleep, dehydration, weight loss, malnutrition, and aspiration. Therefore, proper treatments are necessary. We present a case with intractable hiccup treated with an unusual treatment. A 61-year-old man presented with intractable hiccups, which started 6 years ago after subarachnoid and intraventricular hemorrhage. Conventional pharmacologic treatments including metoclopramide, gabapentin, and baclofen were unsuccessful. Cooperating with cardiothoracic surgeons, phrenic nerve clipping operation was done under intraoperative electrophysiologic monitoring. This method was successful that the symptoms were relieved. Reversible clipping done under intraoperative electrophysiologic monitoring can be a promising therapeutic method for persistent and intractable hiccups in patients with stroke.
Baclofen ; Dehydration ; Depression ; Diaphragm ; Electromyography ; Fatigue ; Hemorrhage ; Hiccup* ; Humans ; Malnutrition ; Methods ; Metoclopramide ; Middle Aged ; Phrenic Nerve* ; Stroke* ; Surgeons ; Weight Loss

Hiccups are an involuntary contraction of the diaphragm that may repeat several times per minute. In general, hiccups are very common, transient, and self-limited. However, if the condition persists longer than days or months, it impacts a patient's quality of life. Pharmacologic and non-pharmacologic methods are used for the treatment of persistent or intractable hiccups. Nerve block and stimulation have been shown to be effective through neural pathway interruption or stimulation of the hiccup reflex arc. Stellate ganglion block (SGB) is an injection of local anesthetic adjacent to a group of nerves in the neck known as the stellate ganglion. The authors report a case of SGB as an effective treatment for a patient with intractable hiccups resulting from right lateral medullary syndrome.
Diaphragm ; Hiccup* ; Humans ; Lateral Medullary Syndrome ; Neck ; Nerve Block ; Neural Pathways ; Quality of Life ; Reflex ; Stellate Ganglion* ; Sympathetic Nervous System

Klinefelter syndrome a genetic disorder with various clinical manifestations. Neurological symptoms, such as seizures, are rarely reported with Klinefelter syndrome, and it response well to anti-epileptic drugs. A 5-month-old boy visited the Inha university hospital due to jerking movements and hiccups. The patient had been diagnosed with Klinefelter syndrome at birth and had a medical history of admission to the neonatal intensive care unit due to opisthotonus and ocular deviation at 26 days of age. The patient's serum testosterone level was decreased and his anti-Müllerian hormone level was increased. The brain image examination was normal and the electoencephalography and other blood test results showed no specific findings. However, after admission, the patient recurred generalized tonic-clonic-seizures recurred intermittently even after the administration of antiepileptic drugs. This paper reports a case of non-febrile seizures in a child with Klinefelter syndrome who presented with a refractory course.
Anticonvulsants ; Brain ; Child ; Epilepsy ; Hematologic Tests ; Hiccup ; Humans ; Infant* ; Infant, Newborn ; Intensive Care, Neonatal ; Klinefelter Syndrome* ; Male ; Parturition ; Seizures* ; Testosterone

While most benign hiccups can be controlled with empirical therapy, intractable hiccups lasting longer than one month tend to have significant adverse effects with obscure etiology. Treatment strategies for intractable hiccups have not been established. Only a few sporadic cases of bilateral phrenic nerve blockage have been reported. Here, we report a case of intractable hiccups that lasted five weeks in a 56-year-old male patient with a lung cancer above the right diaphragm. We hypothesized that his intractable hiccups were caused by irritation and mass effect caused by the lung cancer. We performed an ultrasound-guided right unilateral phrenic nerve pulsed radiofrequency treatment, and the patient's intractable hiccups were successfully managed without complication.
Diaphragm ; Hiccup* ; Humans ; Lung Neoplasms ; Lung* ; Male ; Middle Aged ; Phrenic Nerve ; Pulsed Radiofrequency Treatment ; Ultrasonography

BACKGROUND: Herpes zoster of the head and neck commonly presents with Ramsay Hunt syndrome. However, vesicular eruptions may occur on the pharyngeal or laryngeal area with multiple lower cranial-nerve (CN) palsy. CASE REPORT: We report on the case of a 54-year-old man with herpes zoster of the pharynx and larynx with multiple CN palsy and persistent hiccups. He initially developed progressive dysphagia, hoarseness, and persistent hiccups (CN IX and X). After admission, Dizziness, hearing impairment, and peripheral facial palsy (CN VII and VIII) were complicated. The results of a polymerase chain reaction test of saliva and vesicular fluid from the ear and throat were strongly positive for varicella zoster virus. The progression of CN palsy was in an ascending sequence. CONCLUSION: We suggest that the sequence of CN palsy may be either ascending or descending, depending on the initial site of involvement.
Cranial Nerve Diseases ; Cranial Nerves ; Deglutition Disorders ; Dizziness ; Ear ; Facial Paralysis ; Head ; Hearing Loss ; Herpes Zoster Oticus ; Herpes Zoster ; Herpesvirus 3, Human ; Hiccup ; Hoarseness ; Humans ; Laryngitis ; Larynx ; Middle Aged ; Neck ; Paralysis ; Pharynx ; Polymerase Chain Reaction ; Saliva

No abstract available.
Hiccup* ; Myoclonus

Our case had hiccups arising in an adolescent with the attention deficit and hyperactivity disorder (ADHD) and conduct disorder (CD) after adding aripiprazole treatment to extended-release methylphenidate. Actually, antipsychotics are also used in the treatment of hiccups, but studies suggest that they can cause hiccups as well. Within 12 hours of taking 2.5 mg aripiprazole added to extended-release methylphenidate at a dose of 54 mg/day, 16-year-old boy began having hiccups in the morning, which lasted after 3–4 hours. As a result, aripiprazole was discontinued and methylphenidate was continued alone because we could not convince the patient to use another additional drug due to this side effect. Subsequently, when his behavior got worsened day by day, his mother administered aripiprazole alone again at the dose of 2.5 mg/day at the weekend and continued treatment because hiccup did not occur again. But when it was administered with methylphenidate on Monday, hiccup started again next morning and lasted one hour at this time. In conclusion, we concluded that concurrent use of methylphenidate and aripiprazole in this adolescent led to hiccups.
Adolescent* ; Antipsychotic Agents ; Aripiprazole* ; Conduct Disorder* ; Cytochrome P-450 CYP2D6 ; Hiccup* ; Humans ; Male ; Methylphenidate* ; Mothers

Neuromyelitis optica spectrum disorder (NMOSD) may present with area postrema syndrome, which is characterized by intractable vomiting and hiccups. Hyponatremia is common in NMOSD and is mostly associated with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). In contrast to SIADH, cerebral salt wasting syndrome (CSWS) causes hyponatremia, which is associated with severe natriuresis and extracellular volume depletion in patients with cerebral disease. To our knowledge, hyponatremia associated with CSWS has not been reported in a patient with NMOSD. Here, we describe a NMOSD presenting with hyponatremia, which may be caused by CSWS following area postrema syndrome.
Area Postrema ; Hiccup ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome ; Natriuresis ; Neuromyelitis Optica* ; Vomiting ; Wasting Syndrome*