Intractable hiccups as a presenting symptom of cerebellar hemangioblastoma. Case report

The authors report on a 52-year-old woman with a cerebellar hemangioblastoma who presented with a 2-year history of intractable hiccups. Computerized tomography scans and magnetic resonance images revealed a cerebellar hemangioblastoma with compression of the brainstem at the level of the medulla oblongata. The patient has been free of hiccups and has been neurologically intact since the day after total removal of the tumor. A review of the literature on medullary lesions presenting with intractable hiccups is provided.     

Hematomyelia caused by ruptured intramedullary spinal artery aneurysm associated with extramedullary spinal arteriovenous fistula--case report

A 53-year-old woman presented with a ruptured intramedullary aneurysmal dilatation fed by the anterior spinal artery associated with an arteriovenous malformation located in the ventral cervical spinal cord. She developed tetraparesis and respiratory dysfunction. The neurological deterioration was caused by hematomyelia due to the ruptured aneurysmal dilatation and progression of edema in the upper cervical spinal cord due to venous hypertension associated with additional hematoma in the medulla oblongata. Endovascular embolization of both C-1 and C-2 radicular arteries was performed with Guglielmi detachable coils, but components fed by small branches such as the radiculo-pial artery were not obliterated. Surgery was performed for extirpation of the arteriovenous malformation and cervical intramedullary hematoma, and excision of the aneurysmal dilatation through a transcondylar approach combined with vertebral artery transposition. Postoperatively, she overcame several complications such as pneumonia and endocarditis, and had only moderate weakness of the right upper and lower limbs. This case indicates that surgical intervention for high cervical intramedullary lesion may be very effective.     

Unusual spinal cord enlargement related to intramedullary hemangioblastoma

In a series of 60 intramedullary spinal cord tumors, there were eight cases of hemangioblastoma. Seven of these eight patients displayed evidence of diffuse spinal cord enlargement over multiple levels at a considerable range beyond the actual location of the tumor. One patient had a large intramedullary cyst that accounted for only part of the enlargement, and one had multiple tumor nodules. Six other patients had no obvious explanation for the diffuse cord enlargement. This phenomenon of extensive spinal cord enlargement appears to be unique to this type of tumor and not related to multiple tumors or extensive cyst formation. The cord enlargement seems to be related to edema and congestion of the spinal cord, perhaps secondary to the vascular shunt present in the hemangioblastomas. The possible pathophysiology and clinical importance of this observation is discussed.     

Acute brainstem dissection of syringomyelia associated with cervical intramedullary neurinoma

Intramedullary tumors and syringomyelia typically present with slowly progressing deficits. More rarely, they are characterized by acute presentation or worsening, at times mimicking other more common etiologies. The acute onset of syringomyelia is most likely attributable to an acute increase in cerebrospinal fluid and epidural venous pressure that results in impulsive fluid movement and, ultimately, in the rupture of the syrinx and dissection into the spinal cord or brainstem. Reported here is a case of acute presentation of a small cervical intramedullary neurinoma due to the upward dissection of its associated syrinx. Critical questions are: (1) how can a small tumor produce a large syrinx? and (2) in the absence of craniospinal interferences, which mechanism underlies the acute expansion of the cavity, resulting in a rapid onset? The authors examined the pathophysiology of syrinx formation and enlargement in intramedullary tumors and reviewed the literature, emphasizing the relationship between spinal cord movements and intramedullary pressure. On the basis of current pathogenetic concepts, the authors concluded that tumor-related syringomyelia might be caused by an association of mechanisms, both from within (obstruction of perivascular spaces; increase in extracellular fluid viscosity due to the tumor itself; intramedullary pressure gradients among different cord levels and between the cord and the subarachnoid space) and from without (the cerebrospinal fluid entering the tissue). All these factors may be amplified, as in the reported case, by a tumor located dorsally at the cervical level. Abnormal postures of the spine, such as a prolonged and excessive flexed neck position, may ultimately contribute to the acute dissection of the syrinx.     

原发性脊髓内黑色素瘤并脊髓空洞症1例

原发于椎管内的黑色素瘤十分罕见,自1906年首次发现以来,国内外均只见散在报道。有学者认为此病多发于中下段胸椎,而部位在髓内的较髓外硬膜下较少[1]。我院于2014年收治1例,现报道如下。     

Hiccups attributable to syringobulbia and/or syringomyelia associated with a Chiari I malformation: case report

OBJECTIVE AND IMPORTANCE: Approximately 20 to 50% of patients with syringomyelia associated with Chiari malformations exhibit cranial nerve or cerebellar symptoms. However, hiccups represent a rare clinical manifestation of this disorder. We report a case of intractable hiccups resulting from syringobulbia associated with a Chiari I malformation, which was successfully treated with foramen magnum decompression. CLINICAL PRESENTATION: We report the case of a patient who presented with syringomyelia and syringobulbia associated with a Chiari I malformation, manifested as intractable hiccups and neurological deficits. Magnetic resonance imaging scans demonstrated syringobulbia in the dorsal medullary region and a large cervical syrinx from C2 to C6-C7, associated with a Chiari I malformation. INTERVENTION: Foramen magnum decompression and a C1 laminectomy were performed. One month later, the intractable hiccups disappeared and the neurological symptoms demonstrated improvement. CONCLUSION: Postoperative magnetic resonance imaging scans demonstrated enlargement of the subarachnoid space in the posterior fossa and disappearance of the syringobulbia. There has been no recurrence of intractable hiccups and syringobulbia in 6 months after surgery. Magnetic resonance imaging of the brainstem is an important diagnostic procedure for intractable hiccups, because syringobulbia associated with a Chiari malformation represents a surgically treatable disorder, although the incidence is low.     

颈髓髓内血管网织细胞瘤的诊断及显微外科治疗

目的探讨颈髓髓内血管网织细胞瘤的诊断及显微外科治疗。方法分析26例患者的MRI表现及其显微外科手术治疗的效果。其中男19例，女7例，年龄17～55岁。结果根据肿瘤在MRI上的表现可分为囊肿型12例，实体型14例，均行肿瘤全切除术。术后患者神经系统症状好转者17例，7例无改善，2例加重。结论颈髓MRI能对颈髓髓内血管母细胞瘤作出定位诊断，并可将其分型，以利选择不同的手术方法。颈髓髓内、即使累及延髓的血管母细胞瘤宜行积极手术治疗；根据肿瘤类型不同选择不同手术方法，在手术显微镜下沿正确的界面进行分离，先离断动脉后处理静脉，避免分块切除而力争全切，是减少术中出血和避免神经功能损害的关键。     

Intramedullary spinal cord metastasis from renal cell carcinoma mimicking intramedullary hemangioblastoma

A rare case of intramedullary spinal cord metastasis from renal cell carcinoma mimicking intramedullary hemangioblastoma was described. A 57-year-old man had a nephrectomy for renal cell carcinoma in the left kidney 5 years ago, and no recurrence or metastasis was found in computed tomography and bone scintigraphy. Later, the patient was presented as having bilateral shoulder pain and severe palsy of bilateral upper and lower extremities, and a solitary tumor in the intramedullary spinal cord was found at the C4 level. Excision of the tumor was performed and the intraoperative pathological diagnosis suspected the tumor to be hemangioblastoma. However, a final pathological examination revealed characteristics consistent with metastasis of renal cell carcinoma. Although the patient’s neurological condition and neuralgia initially slightly deteriorated postoperatively, they then gradually improved. Twenty-two months after the operation, a follow-up magnetic resonance imaging showed no recurrence of intramedullary spinal cord tumor, and there were no other metastases found in other organs.     

Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis

BACKGROUND: Syringomyelia is a common intramedullary lesion associated with spinal arachnoiditis and obstruction of the foramen magnum such as in Chiari's malformation. Disturbance of cerebrospinal fluid flow around the spinal cord has an important role in the development of syringomyelia due to spinal arachnoiditis; however, the exact mechanisms have not been clarified. The purpose of this retrospective study is to understand the clinical features and pathomechanisms of syringomyelia secondary to spinal arachnoiditis and to provide the current choice of surgical treatment in this difficult clinical entity. METHODS: Clinical and radiological findings in 15 patients with syringomyelia associated with spinal arachnoiditis who underwent surgical treatment in our institutes between 1982 and 2000 were reviewed. All patients presented with paraparesis or tetraparesis on admission. RESULTS: Magnetic resonance imaging (MRI) or computed tomography-myelography revealed that the syrinx predominantly existed at the thoracic levels. Five patients showed complete block of the thoracic subarachnoid space by conventional myelography. T2-weighted MRI showed diffuse intramedullary hyperintensity at the level of arachnoiditis. As the first surgical treatment, 10 patients underwent syringo-peritoneal shunt placement. Three patients were treated with a syringo-subarachnoid shunt, and 2 patients were treated with a ventriculoperitoneal shunt. Eight patients required further shunting operations for syringomyelia 2 months to 12 years after the first surgery. Neurologic improvement was obtained in 9 patients (60%) with decreased size of the syrinx. One patient remained stable; 5 patients showed gradual deterioration. CONCLUSIONS: The syrinx originated from the thoracic levels where severe adhesion of the subarachnoid space was present. The mechanisms of syrinx formation may be based on the increased interstitial fluid in the spinal cord. Shunting procedures were effective in some population of the patients. Decompression procedures of the spinal subarachnoid space may be an alternative primary surgical treatment except for patients with longitudinally extensive arachnoiditis.     

Intractable hiccup caused by spinal cord lesions in demyelination disease

Abstract

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.     

脊髓内肿瘤伴全长空洞的诊治体会

目的探讨髓内肿瘤伴脊髓全长空洞的诊断、治疗方法及空洞形成机制.方法对6例确诊为髓内肿瘤伴脊髓全长空洞的患者行显微镜下肿瘤摘除术,空洞不予处理.结果经3年的随访,6例均疗效明显,肿瘤摘除完整,空洞自行缩小或消失,症状改善.病程越短,疗效越好.结论脊髓全长空洞发生率可能比文献报道高.空洞是由于肿瘤本身的梗阻引起,显微外科技术能最大限度地保留脊髓功能,空洞不需处理.     

Three cases of intramedullary spinal hemangioblastoma: The role of intraoperative histological diagnosis

Three cases of intramedullary spinal hemangioblastoma are presented. Since the tissue adjacent to the neoplasm often shows a gliosis resembling an astrocytoma, a misinterpretation in the intraoperative histological diagnosis may lead to an unnecessary exstirpation of the reactive tissue. It is recommended that biopsies be performed in cases in which a hemangioblastoma being considered. This is necessary in order to get specimen from the hemangioblastoma itself for the intraoperative histological evaluation.     

An extramedullary spinal cord tumor associated with syringomyelia: a case report

A 53-year-old woman presenting weakness in the left hand for ten years was diagnosed seven years ago as having juvenile muscular atrophy of the unilateral upper extremity. Afterwards, her left forearm and hand gradually became weaker. A neurologist pointed out syringomyelia in the film of MR imaging. However, the cause of syringomyelia was not demonstrated until gadolinium-DTPA-enhanced MR imaging disclosed a spinal tumor. Through an operation it was found that it was an intradural extramedullary tumor at C7 level. Histological diagnosis of the tumor was meningioma. Only 12 reports of syringomyelia associated with extramedullary intraspinal tumors were found. Among them each tumor in three cases was diagnosed by delayed metrizamide computed tomography, only one case by MR imaging. Though in our case myelography, postmyelographic CT, intravenous enhanced CT and MR imaging were performed, gadolinium-DTPA-enhanced MR imaging was eventually most useful in diagnosing this tumor. The pathophysiologic mechanism by which the syrinx fills, and the differentiation in MR imaging between syrinx and tumor cyst were reviewed.     

Hemorrhagic intramedullary hemangioblastoma of the cervical spinal cord presenting with acute-onset quadriparesis: Case report and review of the literature

Context

Hemangioblastomas of the spinal cord are uncommon vascular tumors. Patients commonly present with subtle neurologic findings that are thought to represent growth of the lesion over time. Hemorrhage of an intramedullary hemangioblastoma presenting as acute neurologic deficit is an extremely rare occurrence. Although the cervical spine is the most common location for hemangioblastoma of the spinal cord, there have been no previously published cases in the literature of intramedullary hemorrhage from such a lesion.

Findings

A 22-year-old woman with a previously undiagnosed spinal cord hemangioblastoma presented with sudden-onset dense quadriparesis due to intramedullary hemorrhage in the cervical spinal cord. The patient did not have any clinical findings of von-Hippel Lindau disease. Laminoplasty from C5 to T2 and posterior midline myelotomy for resection of the intramedullary tumor with hematoma evacuation were completed without complication.

Conclusion

Intramedullary hemangioblastoma of the spinal cord is uncommon, and hemorrhage from a cervical spinal cord lesion has not previously been reported. Symptoms from these usually indolent lesions are commonly associated with tumor growth, edema, or associated syrinx, whereas devastating acute neurologic deficit from hemorrhage is exceedingly rare. Microsurgical resection should be done in cases of symptomatic lesions and considered in isolated symptomatic lesions without the known diagnosis of von Hippel-Lindau disease.     

Syringosubarachnoid shunt for noncommunicating syringomyelia associated with spinal lipoma: a case report

Noncommunicating syringomyelia may complicate repeated untethering procedures in patients with spinal lipomas. Surgical intervention is indicated when syringomyelia is symptomatic. However, the preferred surgical procedure for noncommunicating syringomyelia associated with spinal lipomas has not been established. The authors report a case of noncommunicating syringomyelia associated with a spinal lipoma which was successfully treated with a syringosubarachnoid (S-S) shunt. The patient was a 9-year-old girl with a past history of an excision of a dermal sinus at the age of three months and an untethering of the spinal cord at the age of five. She developed a gait disturbance in the year prior to surgery, and a magnetic resonance imaging (MRI) study demonstrated syringomyelia from the second thoracic vertebra to the fourth lumbar vertebra. S-S shunt surgery successfully addressed the syringomyelia without adverse events, and a follow-up MRI study showed reduction of the size of the lesion. S-S shunt placement was therefore considered to be an effective treatment option for noncommunicating syringomyelia associated with a spinal lipoma.