Integrated treatment for autonomic paraneoplastic syndrome improves performance status in a patient with small lung cell carcinoma: a case report

Abstract Background Paraneoplastic neurological syndromes (PNS) are rare disorders associated with cancer and are believed to be immune mediated. Patients with autonomic PNS suffer from variable combinations of parasympathetic and sympathetic failure. Autonomic PNS are usually associated with other PNS, such as encephalomyelitis and sensory neuropathy; however, autonomic symptoms may rarely manifest as PNS symptoms. Autonomic symptoms, therefore, may be overlooked in patients with cancer. Case presentation We described a 65-year-old Japanese man who was diagnosed with autonomic PNS due to small-cell lung carcinoma (SCLC) with Eastern Cooperative Oncology Group (ECOG) performance status 3, who suffered from orthostatic hypotension, and urinary retention needing a urethral balloon. Laboratory studies showed decreased levels of noradrenaline, and were positive for anti-ganglionic acetylcholine receptor antibody, type 1 antineuronal nuclear antibody, and sry-like high mobility group box 1 antibody. Nerve conduction evaluations and 123I-metaiodobenzylguanidine myocardial scintigraphy showed no abnormalities. Abdominal contrast-enhanced computed tomography revealed marked colonic distention. The patient’s autonomic symptoms resolved following integrated treatment (symptomatic treatment, immunotherapy, and additional chemotherapy) enabling the patient to walk, remove the urethral balloon, and endure further chemotherapy. ECOG performance status remained at 1, 10 months after admission. Conclusions Integrated treatment for autonomic PNS may improve autonomic symptoms and ECOG performance status of patients with cancer

Arterial Spin Labeling Imaging of a Giant Aneurysm Leading to Subarachnoid Hemorrhage following Cerebral Infarction

An 83-year-old Japanese man was admitted with dysarthria and right hemiparesis. He had had a large intracranial aneurysm on the left internal carotid artery 5 years before admission and had been followed up under conservative treatment. On admission, diffusion-weighted imaging revealed a hyperintense signal on the left anterior choroidal artery territory. Time-of-flight magnetic resonance angiography demonstrated poor visibility of the middle and anterior cerebral arteries and the inferior giant aneurysm, suggesting distal emboli from aneurysm thrombosis or a reduction of blood outflow due to aneurysm thrombosis. Arterial spin labeling (ASL) signal increased in the giant aneurysm, suggesting blood stagnation within the aneurysmal sac, and decreased in the left hemisphere. We diagnosed cerebral infarction due to aneurysm thrombosis, and started antithrombotic therapy. On day 2, he suddenly died of subarachnoid hemorrhage due to rupturing of the giant aneurysm. When thrombosis occurs in a giant aneurysm, increasing ASL signal within the aneurysm and decreasing ASL signal with poor visibility on magnetic resonance angiography in the same arterial territory may indicate the danger of impending rupture of the giant aneurysm

Progressive multifocal leukoencephalopathy associated with thymoma with immunodeficiency: a case report and literature review

Abstract Background The development of progressive multifocal leukoencephalopathy (PML) is associated with severe cellular immunosuppression. Good’s syndrome (GS) is a rare immunodeficiency syndrome related to thymoma, with the development of humoral as well as cellular immunosuppression; however, there are few reports of PML due to GS. One report suggested that the neurological symptoms of PML related to thymoma may be improved by a reduction of immunosuppressive therapy for myasthenia gravis (MG). It is therefore necessary to identify the cause of immunodeficiency in patients with PML to enable an appropriate treatment strategy to be adopted. Case presentation: A 47-year-old Japanese woman was admitted with aphasia and gait difficulty. She had an invasive thymoma that had been treated with repeated chemotherapy, including cyclophosphamide. She had also previously been diagnosed with MG (Myasthenia Gravis Foundation of America clinical classification IIa), but her ptosis and limb weakness had completely recovered. On admission, neurological examination revealed motor aphasia and central facial weakness on the right side. Laboratory studies showed severe lymphopenia, decreased CD4+ and CD8+ T cell and CD19+ B cell counts, and reduced levels of all subclasses of immunoglobulins, suggesting GS. Serology for human immunodeficiency virus (HIV) infection was negative. Brain magnetic resonance imaging showed asymmetric multifocal white matter lesions without contrast enhancement. Cerebrospinal fluid real-time polymerase chain reaction for JC virus was positive, showing 6,283,000 copies/mL. We made a diagnosis of non-HIV-related PML complicated with GS and probable chemotherapy-induced immunodeficiency. She then received intravenous immunoglobulin therapy, mirtazapine, and mefloquine, but died of sepsis 46 days after admission. Conclusions It is necessary to consider the possibility of immunodeficiency due to GS in patients with PML related to thymoma. Neurologists should keep in mind the risk of PML in MG patients with thymoma, even if the MG symptoms are in remission, and should thus evaluate the immunological status of the patient accordingly

Evaluation of cerebral blood flow in older patients with status epilepticus using arterial spin labeling

Introduction: Although older patients with status epilepticus (SE) have a high mortality rate and poor outcome, it is difficult to perform emergent electroencephalography (EEG) to diagnose SE in community hospitals. Arterial spin labeling (ASL) is a non-invasive magnetic resonance imaging (MRI) technique that can rapidly assess cerebral blood flow (CBF). Further, ASL can detect increased CBF in the ictal period. Therefore, ASL may be a useful tool for diagnosing SE in older patients. However, its effectiveness in this population is unknown. Methods: We retrospectively investigated differences in CBF abnormalities between older patients (≥70 years) and non-older patients (<70 years) with SE using ASL. Participants were diagnosed with convulsive status epilepticus (CSE) or non-convulsive status epilepticus (NCSE) based on symptoms, brain MRI, and EEG. Results: ASL detected CBF abnormalities in 40% of older patients with CSE or NCSE. Rates of CBF abnormalities in older patients were not significantly different compared with that in non-older patients. Conclusions: ASL did not detect a higher rate of CBF abnormalities in older patients, but may help physicians diagnose SE in older patients in a community hospital setting if emergent EEG cannot be immediately performed. Keywords: Status epilepticus, Arterial spin labeling, Older patient, Non-convulsive status epilepticus, Magnetic resonance imagin

LSVT-BIG Improves UPDRS III Scores at 4 Weeks in Parkinson’s Disease Patients with Wearing Off: A Prospective, Open-Label Study

The efficacy of LSVT-BIG for advanced Parkinson’s disease (PD) patients with wearing off remains to be determined. Therefore, we evaluated whether LSVT-BIG improves motor disability in eight PD patients with wearing off. Unified Parkinson’s Disease Rating Scale (UPDRS) scores, daily off time, and mobility assessments were evaluated during the “on” time before and after the LSVT-BIG course. LSVT-BIG significantly improved UPDRS III scores at 4 weeks and UPDRS II scores in the “off” state at 12 weeks, with no changes in the other measures. The findings suggest that LSVT-BIG may be an effective therapy for advanced PD patients with wearing off

Unilateral moyamoya disease with ipsilateral carotid canal hypoplasia: A case report

A 41-year-old Korean man was admitted to our hospital with a 1-day history of headache and abnormal left visual field. Neurological examination revealed left homonymous hemianopia and difficulty in fine movement of the left finger. Laboratory studies indicated hyperlipidemia and hyperhomocysteinemia. Diffusion-weighted imaging showed a hyperintense signal on the right posterior cerebral-middle cerebral artery watershed area. Computed tomography angiography and carotid angiography revealed the bottle neck sign on the right internal carotid artery (ICA) with occlusion of the distal right ICA, but not the left ICA. Carotid angiography showed moyamoya vessels as well as occlusion of the distal right ICA. The right carotid canal was hypoplasic. We diagnosed the patient with unilateral moyamoya disease and carotid canal hypoplasia. This case demonstrates that unilateral carotid canal hypoplasia may co-exist with ipsilateral moyamoya disease. A unilateral bottle neck sign may indicate the presence of carotid canal hypoplasia. Evaluation of the bony carotid canal may be useful when it is necessary to distinguish between secondary vascular stenosis due to arteriosclerosis and MMD. Keywords: Moyamoya disease, Carotid canal, Bottle neck sig