Narrowing of the Parent Artery Angle Is Associated With Intracranial Aneurysm Growth.

OBJECTIVE: Although risk factors for intracranial aneurysm growth have been reported, studies investigating the influence of the parent artery angle are limited. In this study, we examined the relationship between intracranial aneurysm growth and parent artery angle narrowing by analyzing long-term follow-up magnetic resonance angiography data. METHODS: We retrospectively reviewed data of patients with untreated aneurysms and those treated by simple coil embolization, who were followed up by magnetic resonance angiography for over 24 months at the Steel Memorial Yawata Hospital between August 2007 and March 2023. We investigated the relationship of aneurysm growth with parent artery angle narrowing, age, sex, follow-up duration, previous subarachnoid hemorrhage, hypertension, smoking, aneurysm location, aneurysm type, maximum size, and neck size. RESULTS: A total of 180 aneurysms of 162 patients (women, n=113; untreated, n=136) were included. The median age at aneurysm diagnosis was 71 (63.8-76) years and the median follow-up duration was 69 (45-120) months. Among the 180 aneurysms, 41 (untreated, n=30; treated by simple coil embolization, n=11) showed growth during the follow-up period, with a risk of 4.4%/patient-year. In the univariable analysis, the parent artery angles on the initial and last follow-up images and angle change were significantly associated with aneurysm growth. However, in the multivariable analysis, the association remained significant only for angle change (odds ratio, 2.21; 95% confidence interval, 1.42-3.45). The cutoff value of parent artery angle change for intracranial aneurysm growth was -3.4°. CONCLUSION: Parent artery angle narrowing was significantly associated with intracranial aneurysm growth. This parameter may be useful for the monitoring of patients with unruptured intracranial aneurysms and may contribute to discerning the mechanism of intracranial aneurysm growth.

A case of hyperparathyroidism-associated parkinsonism successfully treated with cinacalcet hydrochloride, a calcimimetic.

BACKGROUND: Some metabolic disorders, including abnormal calcium metabolism, can develop and worsen parkinsonism. However, whether hyperparathyroidism can cause parkinsonism remains controversial. CASE PRESENTATION: An 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. CONCLUSIONS: In the present case, we speculate that hypercalcemia probably due to secondary hyperparathyroidism that had developed during steroid therapy deteriorated the parkinsonism.

A case of Parkinson's disease following dystonia.

Parkinsonism and dystonia are both disorders of the extrapyramidal motor system, and some patients exhibit a complex of the two symptoms. Although several reports have referred to the coexistence of these disorders as parkinsonian disorders with dystonia, in the majority of cases, dystonia appeared after parkinsonism. DAT-scan is useful for the early diagnosis of Parkinson's disease (PD) and other types of parkinsonism such as dementia with Lewy bodies. This case report describes a 67-year old woman diagnosed with axial dystonia without parkinsonism 6 years previously, which had worsened despite treatment with Botulinum toxin injections, and hindered the patient's gait. The patient visited the hospital because of gait disturbances and DAT-scan showed a levodopa transducer decrease in the putamen. A few weeks later, she was re-admitted to hospital and exhibited Parkinsonism. Levodopa improved the gait disturbances but axial dystonia was unchanged, and a clinical diagnosis of PD was made. In the authors' opinion, this was a rare case of parkinsonian disorders with dystonia, characterized by the development of PD after dystonia. DAT-scan may be helpful for the diagnosis of patients with parkinsonian disorders with dystonia.

Champagne bottle neck sign in a patient with Moyamoya syndrome.

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.

[Cerebral infarction and intracranial aneurysm related to the reactivation of varicella zoster virus in a Japanese acquired immunodeficiency syndrome (AIDS) patient].

A 35-years-old right-handed man admitted to our hospital with a worsening of dysarthria, left facial palsy and left hemiparesis for 2 days. Acquired immunodeficiency syndrome (AIDS) was diagnosed when he was 28 years old. At that time, he also was treated for syphilis. After highly active antiretroviral treatment (HAART) was introduced at the age of 35 years old, serum level of human immunodeficiency virus (HIV) was not detected, but the number of CD4+ T cells was still less than 200/µl. He had no risk factors of atherosclerosis including hypertension, diabetes and hyperlipidemia. He had neither coagulation abnormality nor autoimmune disease. Magnetic resonance imaging (MRI) showed acute ischemic infarction spreading from the right corona radiate to the right internal capsule without contrast enhancement. Stenosis and occlusion of intracranial arteries were not detected by MR angiography. Although argatroban and edaravone were administered, his neurological deficits were worsened to be difficult to walk independently. Cerebrospinal fluid (CSF) examination showed a mild mononuclear pleocytosis (16/µl).　Oligoclonal band was positive. The titer of anti-varicella zoster virus (VZV) IgG antibodies was increased, that indicated VZV reactivation in the central nervous system (CNS), although VZV DNA PCR was not detected. Therefore, acyclovir (750 mg/day for 2 weeks) and valaciclovir (3,000 mg/day for 1 month) were administered in addition to stroke therapy. He recovered to be able to walk independently 2 month after the admission.Angiography uncovered a saccular aneurysm of 3 mm at the end of branch artery of right anterior cerebral artery, Heubner artery, 28 days after the admission. We speculated that VZV vasculopathy caused by VZV reactivation in CNS was involved in the pathomechanism of cerebral infarction rather than HIV vasculopathy in the case.

[Case of systemic myositis and subacute sensory neuropathy concomitant with signet-ring cell carcinoma].

A 72-year-old woman referred to our hospital because of slowly progressive (over 2 years) muscle weakness and paresthesias of the lower limbs. On neurological examination, weakness and muscle atrophies were noted in the distal upper limbs as well as the proximal lower limbs. She had also paresthesias of the legs. The level of creatinine phosphokinase (CK) was 126 IU/l. The magnetic resonance imaging demonstrated gadolinium enhancement of the nerve roots at the L4-S2 vertebrate levels. Nerve conduction study showed decreased compound muscle action potential and motor conduction velocity of tibial and peroneal nerves. Biopsy of the left biceps brachii muscle showed variations in fiber size, endomysial mononuclear cell infiltration and the findings like a rimmed vacuole. Although almost of her findings were in accord with clinical features of inclusion body myositis, strong inflammatory cellular influences allowed us to administer corticosteroid therapy. Because her weakness was well responded to steroid therapy, polymyositis was considered as differential diagnosis. Then, further examinations were investigated to search any occult neoplasm, and detected the early gastric cancer. Total gastrectomy was performed later, and the pathological diagnosis was made as a signet-ring cell carcinoma. To our knowledge, this is the first report of systemic myositis and subacute sensory neuropathy concomitant with signet-ring cell carcinoma. These symptoms might be occurred as a result of paraneoplastic syndrome associated with satellite effects of the signet-ring cell carcinoma.

[Usefulness of carotid ultrasonography for the early detection of moyamoya disease].

We report carotid ultrasonographic findings in moyamoya disease. A 44-year-old man was admitted to our hospital because of fever, headache and nausea. Neurological examination showed neck stiffness and Kernig's sign but he was otherwise normal. Brain computed tomography showed hemorrhage in the right thalamus and the lateral ventricle. Conventional carotid ultrasonography (CCU) detected marked narrowing of the right internal carotid artery (ICA) at the proximal portion without arteriosclerosis, which fulfilled the criteria of bottle neck sign, namely, the ratio of diameter of proximal portion of ICA to that of the distal portion of common carotid artery (CCA) was less than 0.5. Additionally, CCU as well as transoral carotid ultrasonography (TOCU) showed the diameter of the ICA to be smaller than that of the external carotid artery (ECA) (diameter reversal sign). These signs strongly suggested moyamoya disease. Cerebral angiography confirmed the occlusions of intracranial ICA and moyamoya vessels. Bottle neck sign and diameter reversal sign of the carotid artery on carotid ultrasonography are useful for the early detection of moyamoya disease.