BACKGROUND AND PURPOSE:The champagne bottle neck sign represents a rapid reduction in the extracranial ICA diameters and is a characteristic feature of Moyamoya disease. However, the clinical significance of the champagne bottle neck sign is unclear. We investigated the relationship between the champagne bottle neck sign and the clinical and hemodynamic stages of Moyamoya disease.

The champagne bottle neck (CBN) sign refers to a reduction in the diameter of the proximal portion of the internal carotid artery that resembles a CBN, and is a characteristic feature of Moyamoya disease. A 43-year-old woman with an infarction of the posterior limb of the left internal capsule was diagnosed with Moyamoya syndrome associated with Graves' disease. The CBN sign was observed bilaterally. Cerebral revascularization surgery was performed, including left-sided superficial temporal artery to middle cerebral artery anastomosis. During four years of follow-up, she maintained a euthyroid state and did not have any further cerebral ischemic events. The CBN signs remained unchanged on both sides during this time. This is the first report of the CBN sign in a patient with Moyamoya syndrome associated with Graves' disease.

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A 56-year-old man with oral and genital ulcers and folliculitis presented with distally dominant moderate weakness and impaired sensation with painful paresthesia in the right upper extremity. He presented with spastic paresis of the left lower extremity with Babinski sign. Tendon reflex was absent in the right upper extremity. Cerebrospinal fluid analysis showed mild mononuclear pleocytosis. MRI showed a solitary ring-enhanced lesion in the right pontine base with a high-intensity signal on diffusion-weighted image (DWI), and a decreased apparent diffusion coefficient (ADC) map (Picture A: Gd+T1WI, B: FLAIR, C: DWI, D: ADC map). Restricted diffusion indicated neuro-Behcet's disease (NBD) or abscess rather than metastatic tumor and glioma that commonly show unrestricted diffusion (1, 2). MRI also revealed compression of the right brachial plexus by an aneurysm of the right subclavian artery. The pontine lesion markedly improved 3 months after initiation of steroid therapy (Picture E: Gd+T1WI, F: FLAIR). A diagnosis of NBD was made on the basis of mucocutaneous and vascular manifestation and his response to steroid therapy, although

BackgroundSome metabolic disorders, including abnormal calcium metabolism, can develop and worsen parkinsonism. However, whether hyperparathyroidism can cause parkinsonism remains controversial.Case presentationAn 83-year-old woman with a history of right thalamic hemorrhage and drug-induced parkinsonism, was admitted due to worsening of parkinsonian symptoms including mask-like face, bradykinesia, freezing of gait, and rigidity. She had been diagnosed with autoimmune hepatitis and was being treated with prednisolone. Examinations revealed hypercalcemia (14.3 mg/dL) with an increased level of intact parathyroid hormone (iPTH) (361 pg/mL). Her symptoms were resistant to some additional anti-parkinsonian drugs; however, cinacalcet hydrochloride, a calcimimetic for the treatment of secondary hyperparathyroidism, normalized levels of serum calcium and iPTH, and remarkably improved her symptoms.ConclusionsIn the present case, we speculate that hypercalcemia probably due to secondary hyperparathyroidism that had developed during steroid therapy deteriorated the parkinsonism.