Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Area Postrema Syndrome-Like Symptoms without Medulla Oblongata Lesions.

INTRODUCTION: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently described steroid-responsive meningoencephalomyelitis positive for cerebrospinal fluid (CSF) anti-GFAP antibody. Area postrema syndrome (APS) involves intractable hiccups, nausea, and vomiting, which is caused by medulla oblongata (MO) impairment. APS is a characteristic symptom of aquaporin-4 (AQP4) autoimmunity, and it helps to differentiate between AQP4 and GFAP autoimmunity. Conversely, although 6 cases of autoimmune GFAP astrocytopathy with APS and MO lesions have been reported, the association between GFAP autoimmunity and APS is unclear. We report the case of a patient with autoimmune GFAP astrocytopathy presenting with APS-like symptoms without MO lesions and discuss the mechanisms underlying the symptoms. METHODS: CSF anti-GFAP antibody was detected using cell-based assays and immunohistochemical assays. RESULTS: A 54-year-old Japanese man developed persistent hiccups, intermittent vomiting, fever, anorexia, and inattention. Brain magnetic resonance imaging (MRI) showed periventricular lesions with radial linear periventricular enhancement, suggesting autoimmune GFAP astrocytopathy. However, no obvious MO lesions were identified on thin-slice images. Spinal cord MRI revealed hazy lesions with patchy enhancement along the cervical and thoracic cord. CSF analysis demonstrated inflammation, with positive results for anti-GFAP antibodies. Anti-AQP4 antibodies in the serum and CSF were negative. Esophagogastroduodenoscopy revealed gastroparesis and gastroesophageal reflux disease, and vonoprazan, mosapride, and rikkunshito were effective only against persistent hiccups. Steroid therapy was initiated, allowing clinical and radiological improvements. Repeated MRIs demonstrated no obvious MO lesions. CONCLUSION: This report suggests that autoimmune GFAP astrocytopathy presents with APS-like symptoms without obvious MO lesions. The possible causes of hiccups were gastroparesis and cervical cord lesions. Gastroesophageal reflux disease was not considered a major cause of the hiccups. Intermittent vomiting appeared to be associated with gastroparesis, cervical cord lesions, and viral-like symptoms. Testing for anti-GFAP antibodies should be considered in patients with APS-like symptoms in the context of typical clinical-MRI features of autoimmune GFAP astrocytopathy.

Hiccup-like segmental myoclonus in thoracic compressive myelopathy: a case report.

STUDY DESIGN: A case report. OBJECTIVES: This study discusses a case of spinal segmental myoclonus caused by thoracic myelopathy, mimicking hiccup spasms. Spinal myoclonus caused by thoracic myelopathy is extremely rare. It can be misdiagnosed as chronic intractable hiccups due to similar clinical manifestations. SETTING: Korea University Anam Hospital, Seoul, Republic of Korea. METHODS: A 42-year-old man presented with a history of involuntary jerky movement of the upper abdominal wall muscles that had been continuing for over 3 years. A neurological examination, brain computed tomography and electroencephalogram did not reveal a cause of the symptoms. Electromyography was performed on the abdominal muscles and the findings revealed were compatible with spinal myoclonus. The spinal myoclonus had started in the abdominal muscles, with a spinal magnetic resonance imaging revealing a disc protrusion compressing the anterior spinal cord. RESULTS: The cause of the spinal myoclonus was determined to be spinal myelopathy due to mild T7 disc protrusion. The patient refused surgical or invasive interventions and was conservatively treated with clonazepam. The symptoms were reported to be less frequent following the treatment. CONCLUSION: Compressive myelopathy developed from disc protrusion may cause spinal myoclonus mimicking as hiccup spasms.

Intractable hiccup caused by spinal cord lesions in demyelination disease.

OBJECTIVE: This study aimed to summarize the clinical features of patients who presented intractable hiccup (IH) without brain and medulla oblongata (MO) lesions. METHOD: This study included six patients who were diagnosed with inflammatory demyelinating myelitis, categorized as neuromyelitis optica (NMO), multiple sclerosis (MS), and myelitis. Patients who presented IH with cervical lesions but without MO lesions were also included. Clinical profiles, laboratory data, and magnetic resonance imaging findings were analyzed. RESULTS: Three out of six patients were diagnosed with NMO, whereas the remaining three were diagnosed with acute myelitis, recurrent myelities, and MS, respectively. The duration of hiccup was from 2 to 23 days (average = 9.33 ± 8.64 days). Five patients (83.33%, patients 1-5) had long segmental lesions and one had a patchy lesion. None of these patients had any MO lesions. Half of them were successfully treated with high-dose methylprednisolone combined with gamma-aminobutyric acid (GABA) inhibitor. CONCLUSION: IH occurred in patients without MO lesion. However, the mechanism remained unclear. Immune factors of demyelinating neuropathy stimulated the hiccup reflex arch. Cervical cord lesions may activate the hiccup center. In general, IH can be controlled by IVMP combined with GABA inhibitor. Unilateral phrenic nerve block may elicit no effect.

Medullary neurosarcoidosis presenting with intractable hiccoughs.

Hiccoughs (singultus) are a complex physiological process characterized by sudden brief involuntary contractions of the diaphragm. They most commonly occur from peripheral mechanisms that result in diaphragmatic irritation, but also occur from brain stem lesions such as that seen in Wallenberg's syndrome. They are uncommon in sarcoidosis and it is remarkably rare when hiccoughs are the presenting symptom of neurosarcoidosis. We report a patient with sarcoidosis who presented with intractable hiccoughs due to an inflammatory medullary lesion. Evaluation revealed an enhancing lesion in the dorsomedial medulla that resolved after aggressive immune-modulating therapy. In the absence of a clear peripheral lesion that would potentially affect the diaphragm, the diagnosis of neurosarcoidosis involving the brainstem should be considered in patients with sarcoidosis.

Intractable hiccup caused by medulla oblongata lesions: a study of an autopsy patient with possible neuromyelitis optica.

We report the first autopsy verification of medulla oblongata lesions involving bilateral nucleus tractus solitarius (NTS) as a cause of intractable hiccup in an autopsy patient. The female patient first developed pain and weakness in the lower limbs and urinary incontinence at age 48, and was given a diagnosis of myelitis. Intractable hiccup was accompanied by urinary retention on the third attack. She died of respiratory failure when the fifth attack occurred at age 51. Autopsy disclosed severe involvement of the medulla oblongata and entire spinal cord. Optic nerve lesions were also identified unexpectedly. Dual involvement of the optic nerve and spinal cord, necrotic spinal cord lesions involving not only myelin but also neurons and axon, and marked extension of the spinal cord lesions in both the longitudinal and transverse directions suggested the diagnosis of neuromyelitis optica rather than multiple sclerosis. Although animal experiments have shown that NTS is a critical structure in the hiccup reflex, we demonstrated for the first time the involvement of the NTS in an autopsy patient with intractable hiccup.

Lesional location of lateral medullary infarction presenting hiccups (singultus).

BACKGROUND: Hiccups are an infrequent result of lateral medullary infarction. Their importance may be underestimated and they can cause distress, exhaustion, and aspiration. Hiccups in lateral medullary infarction remain poorly understood OBJECTIVE: To evaluate the relation between the lesional loci of lateral medullary infarction and hiccups. METHODS: 51 patients with lateral medullary infarction were investigated by magnetic resonance imaging within three days of the onset of infarction. Seven of the 51 patients developed hiccup. RESULTS: All patients with hiccups had middle level lateral medullary lesions, including two with lower level lesions and four with upper level lesions. In the middle level lateral medullary lesions, dorsolateral lesions were most often involved. All patients with lateral medullary infarction presenting with hiccups also had vertigo, dizziness, nausea, vomiting, and dysphagia. CONCLUSIONS: The observations suggest that middle level and dorsolateral lesion locations in lateral medullary infarction frequently induce hiccups.

An unusual cause of hiccup: costal exostosis. Treatment by video-assisted thoracic surgery.

The described case is of a 15-year-old boy who presented with a persistent hiccup and repeated episodes of left-sided chest pain. At computed tomography scan an exostosis originating from the costo-chondral junction of the left 4th rib was seen. The tip of the exostosis reached the external surface of the pericardium. Removal of a 2 cm rib segment including the implantation basis of the exostosis was achieved by video-assisted thoracic surgery. Symptoms disappeared after surgery. This report shows an exceptional symptom of costal exostosis.

Hiccups.

Hiccups result from a variety of causes and serve no known physiologic function. Although most episodes are time-limited, hiccups may become protracted, with serious consequences. Assessment of hiccups entails a focused history and physical examination coupled with selected laboratory tests. If a correctable malady is discovered, treatment should address the underlying disorder. However, if the cause remains unknown, therapeutic options include nonpharmacologic and pharmacologic measures. Since hiccups are common, this review provides a practical approach to the management of this bothersome symptom.

Intractable hiccup due to multiple sclerosis: MR imaging of medullary plaque.

We describe a 31-year-old man with clinically definite multiple sclerosis who presented with intractable hiccup for one month prior to admission. The responsible lesion was confirmed by magnetic resonance imaging to be a plaque in the ventral paramedian portion of the medulla oblongata.