Arnold-Chiari malformation with syrinx presenting as carpal tunnel syndrome: a case report

A 26-year-old administrative assistant presented with 3 years of left-hand dysesthesia involving primarily the first 3 digits. Her symptoms increased at night and with keyboard use. Through 12 visits to primary and specialty care physicians over 3 years, she experienced minimal improvement with splints and moderate improvement with gabapentin. On presentation, careful questioning revealed an abrupt onset of symptoms 3 years previously, related to a 2-week episode of gastritis associated with recurrent emesis. Examination revealed a negative Tinel sign over the median nerve at the wrist, decreased left biceps reflex, positive Spurling test, and decreased sensation over the palmar and dorsal surfaces of the left hand in the C5-6 distribution. The atypical onset of symptoms, poor response to therapy, and physical findings suggested the possibility of a radicular or central neurologic etiology for the patient's hand numbness. Magnetic resonance imaging demonstrated a Chiari I malformation with a syrinx extending from C2 to T10, with the greatest diameter at C4. Neurosurgical decompression led to a decrease in symptoms. A meticulous history and physical examination should be performed on patients with presumed carpal tunnel syndrome with an atypical onset of symptoms or response to therapy.     

小脑扁桃体延髓联合畸形型164例MR诊断

评价 Arnold- Chiari畸形 ( ACM)的 MR诊断价值。方法 :对我院 1990年 1月至 1999年 12月间 164例临床未明确诊断的 ACM 型患者进行 MR检查 ,总结并分析其影像表现。结果 :164例中 ,60 %脊髓萎缩 ( 99/164) ;80 %出现不同程度脊髓空洞 ( 12 3/164) ;71%延、颈髓交界处屈曲成角 ( 117/164) ;75%合并其他畸形 ( 12 3/164) ;70 %合并颅颈交界处骨结构畸形 ( 115/164)。结论 :MR是诊断 ACM的首选方法 ,并能准确查明其合并畸形、脊髓空洞范围及程度。     

Spontaneous Intracranial Hypotension: Headache With a Reversible Arnold-Chiari Malformation

Spontaneous intracranial hypotension is characterized by severe postural headache in the setting of low CSF pressure, usually attributed to a cryptic CSF leak. We report a patient whose prolonged refractory headache was characterized by the clinical symptoms of occipital neuralgia, but was also associated with the radiographic appearance of an Arnold-Chiari malformation, type I and low CSF pressure. After extensive diagnostic evaluation, CT cisternomyelography ultimately demonstrated a CSF leak at the C2 vertebral level. Symptomatic relief was sustained only with long-term theophylline administration. The apparent Arnold-Chiari malformation resolved with treatment of the low CSF pressure.     

Trigeminal neuralgia with chronic paroxysmal hemicrania: the CPH-tic syndrome

A case of chronic paroxysmal hemicrania-tic syndrome is described. The chronic paroxysmal hemicrania (CPH) was controlled with indomethacin and the trigeminal neuralgia with a glycerol blockade. The trigeminal neuralgia reappeared four years after the blockade and was then treated successfully with corticosteroids. Six months later, when indomethacin was stopped, the trigeminal neuralgia recurred and resolved again with corticosteroids. We suggest that the CPH-tic syndrome is due to periorbital venous vasculitis. To our knowledge this is the first reported case of this disorder.     

A型肉毒毒素治疗三叉神经痛和带状疱疹后神经痛的临床疗效

目的：观察A型肉毒毒素治疗三叉神经痛和带状疱疹后神经痛的临床效果。方法：选取33例三叉神经痛或带状疱疹后神经痛患者，进行疼痛区域A型肉毒毒素皮下或皮内注射治疗，评估患者治疗时、治疗2周后、治疗3个月后疼痛情况（NRS）、睡眠状况及生活质量（QOL），判断治疗效果，观察药物不良反应。结果：患者使用A型肉毒毒素治疗2周后、3个月后疼痛评分、睡眠评分显著低于治疗时（P<0.05），生活质量显著高于治疗时（P<0.05），治疗效果好，不良反应少。结论：A型肉毒毒素治疗三叉神经痛和带状疱疹后神经痛效果显著，可成为神经病理性疼痛治疗的一种新途径。     

A型肉毒毒素治疗三叉神经痛

目的:观察A型肉毒毒素治疗三叉神经痛(TN)的临床疗效。方法:选取57例TN患者,随机分为A、B 2组。A组28例患者口服卡马西平片治疗;B组29例在疼痛部位及板机点周围皮下注射肉毒素治疗。治疗后1,3及6个月时随访,行简式McGill疼痛问卷表(SF-MPQ)及生活质量评价量表(SF-36)评分,并观察不良反应。结果:治疗中脱失7例,A组3例,B组4例。与治疗前3个月SF-MPQ及SF-36平均分作为基础水平比较,治疗1,3及6个月后2组SF-MPQ评分明显下降,SF-36明显上升(P<0.01),B组表明更明显(P<0.05,P<0.01)。治疗过程中,A组出现不适患者多于B组。结论:A型肉毒毒素疼痛点皮下注射治疗TN发作作用高峰1～3个月,维持时间6个月,且临床疗效显著,不良反应轻微。     

Trigeminal neuralgia secondary to giant Virchow-Robin spaces: A case report with neuroimaging

Virchow-Robin spaces are pial-lined, interstitial, fluid-filled structures that accompany penetrating arteries and arterioles as they enter the cerebral substance. Occasionally they may enlarge and become giant Virchow-Robin spaces (GVRS) and produce mass effect. Various neurological symptoms have been described in association with GVRS, however, trigeminal neuralgia has not yet been reported in this context. We present a case of trigeminal neuralgia secondary to dorsal pontine giant Virchow-Robin spaces (GVRS) and highlight the diagnostic radiologic features. Routine 1.5 T MRI sequences were sufficient to diagnose the GVRS and a diffusion tensor imaging (DTI) study revealed distortion of the intrinsic trigeminal pathway. This study highlights the utility of routine MRI to study the intrinsic anatomy of the trigeminal pathway in pathological conditions.     

原发性三叉神经痛微血管减压术中出现5次心跳骤停1例报告

患者,女性,81岁,身高155 cm,体质量51 kg,以“左侧面部间断疼痛3年,加重1周”主诉入院。入院胸部CT:慢性支气管炎、肺气肿征象,双肺散在纤维硬结;心电图(ECG):窦性心律67次/min;心脏B超:左室舒张功能受损,收缩功能正常,未见明显血流异常,左室射血分数(LVEF)62%;其他常规检查无明显异常。     

Familial trigeminal neuralgia: case reports and review of the literature

The paroxysmal facial pain of trigeminal neuralgia is usually idiopathic, but familial cases have been described. We describe a family with apparent autosomal dominant transmission of trigeminal neuralgia. Our cases and a review of the literature suggest that the etiology of trigeminal neuralgia may be vascular compression of the fifth cranial nerve. Autosomal dominant vascular and epileptic disorders are reviewed, and possible relationships to familial trigeminal neuralgia are considered.     

Trigeminal Neuralgia as the Sole Symptom of Pancreatic Cancer

Trigeminal neuralgia (TN) is a condition characterized by brief electric shock‐like pains in the topography of the trigeminal nerve. The most common cause of this disorder is the compression of the trigeminal nerve root by tortuous or aberrant vessels. In this report, we describe a patient who presented due to paroxysmal and excruciating facial pain that was found to be secondary to pancreatic cancer.     

Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia

OBJECTIVE: To characterize the presenting symptoms among persons with radiographic evidence of Chiari malformation (CM) type I in an effort to help the primary care provider determine accurate diagnosis and treatment. METHODS: The symptoms of CM can often be vague and ambiguous, leading to misdiagnosis. Patients who had radiographic evidence of CM with or without syringomyelia were prospectively studied. The detailed symptom data were collected from patient reports at initial examination to determine if there was a pattern of symptoms typically attributable to the CM. RESULTS: Analysis of presenting symptoms among 265 patients demonstrated a distinct pattern of symptom constellation. This study reports a prospective analysis of presenting symptoms among 265 patients with radiographic evidence of CM with or without syringomyelia and defines a common presentation pattern. Recognition of typical symptoms attributable to CM can result in appropriate diagnostic workup, accurate diagnosis, and timely treatment, leading to improved patient outcomes.     

Nasal Sumatriptan as Adjunctive Therapy for idiopathic Trigeminal Neuralgia: Report of Three Cases

We examine the effect of nasal sumatriptan as an adjunctive therapy in 3 patients with idiopathic trigeminal neuralgia refractory to carbamazepine (CBZ) and found that this therapy might be suitable for patients for whom the CBZ dose cannot be increased, who are under poor pain control, and who are not candidates for nerve blocks or surgery.     

Vascular compression as a potential cause of occipital neuralgia: a case report

Vascular compression is a well-established cause of cranial nerve neuralgic syndromes. A unique case is presented that demonstrates that vascular compression may be a possible cause of occipital neuralgia. A 48-year-old woman with refractory left occipital neuralgia revealed on magnetic resonance imaging and computed tomographic imaging of the upper cervical spine an atypically low loop of the left posterior inferior cerebellar artery (PICA), clearly indenting the dorsal upper cervical roots. During surgery, the PICA loop was interdigitated with the C1 and C2 dorsal roots. Microvascular decompression alone has never been described for occipital neuralgia, despite the strong clinical correlation in this case. Therefore, both sectioning the dorsal roots of C2 and microvascular decompression of the PICA loop were performed. Postoperatively, the patient experienced complete cure of her neuralgia. Vascular compression as a cause of refractory occipital neuralgia should be considered when assessing surgical options.