Headache-Related Characteristics of Biopsy-Confirmed Giant Cell Arteritis and the Relationship of Transmural Inflammation With Artery Tenderness and Chordal Thickening.

INTRODUCTION: Giant cell arteritis (GCA) is characterized by headaches, but few studies have examined the detailed characteristics of pathologically confirmed cases. We investigated the characteristics of GCA patients, particularly headache, and their correlation with pathological findings. METHODS: We retrospectively analyzed 26 patients (median age: 77.5 years, male: 38.4%) with GCA who underwent superficial temporal artery (STA) biopsy at the Japanese Red Cross Shizuoka Hospital between May 2001 and February 2022. All patients fulfilled the American College of Rheumatology and European League Against Rheumatism classification criteria for GCA. We focused on the relationship between clinical features, especially headache, and pathological findings. RESULTS: Twenty-four patients had unilateral, nonpulsatile, intermittent headaches. Transmural inflammation (TMI), a characteristic pathology of GCA, was present in 14 patients. Bivariate analysis revealed significant associations between the TMI and STA-related tenderness (odds ratio [OR]=11, 95% confidence interval [CI]=1.14 to 106.43, p=0.046) and the TMI and STA-related chordal thickening (OR=0.19, 95% CI=0.068 to 0.52, p=0.021). CONCLUSIONS: Headache in GCA patients was often unilateral, nonpulsatile, and intermittent. This study highlights the significant association of TMI with STA tenderness and ligamentous thickening, which has not been reported previously. Abnormal STA findings were significantly associated with pathological changes in GCA patients, emphasizing the importance of these lesions in predicting GCA.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) Following Adenovirus Meningitis After First Delivery: Case Report and Literature Review.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a group of central nervous system (CNS) demyelinating diseases caused by autoantibodies against myelin oligosaccharide protein (MOG), a myelin sheath component protein, and present with a variety of symptoms, including optic neuritis, myelitis, acute disseminated encephalomyelitis (ADEM), brainstem encephalitis, and corticobasal encephalitis. It is currently unknown at what point in life MOGAD can develop or how it can be triggered by autoimmune mechanisms. Here, we report a case of a mature woman who suffered from adenoviral meningitis one month after childbirth and developed MOGAD but was able to return to child rearing with high-dose methylprednisolone therapy. This case suggests that the risk of developing MOGAD early after childbirth may be increased. The case also suggested that adenoviral infection may be involved in the development of MOGAD.

Clinical profile of cluster headaches in Japan: low prevalence of chronic cluster headache, and uncoupling of sense and behaviour of restlessness.

INTRODUCTION: This study examined the clinical profile of cluster headaches in Japan and the potential presence of features peculiar to Japan. Most previous studies of cluster headaches have focused on Caucasian populations. METHODS: Subjects comprised 86 consecutive new cluster headache patients (68 males, 18 females; mean age, 38.4 ± 12.2 years; range, 17-73 years). Mean age at onset was 31.0 years and the ratio of males to females was 3.8:1. RESULTS: Chronic cluster headache was observed in 3.5% of patients. More than half of patients (68.9%) reported feelings of restlessness during headache episodes and 42.9% reported restless behaviour. Patients with uncoupling of feelings of restlessness and restless behaviour forced themselves to keep still. Similar findings were reported in a Taiwanese study. CONCLUSION: Japanese patients in this study showed a relatively low prevalence of chronic cluster headaches, and uncoupling of a sense of restlessness and restless behaviour. These features of cluster headache may be more common in Japanese and Taiwanese patients than in Caucasian patients.

[Cluster like headache in a patient with the Maffucci's syndrome].

Maffucci's syndrome is a rare congenital, nonhereditary mesodermal dysplasia characterized by soft tissue hemangiomas and multiple enchondromas. A 52 years old man was diagnosised as Maffucci's syndrome in his childhood. He complained of mild paraesthesia which gradually progressed to intolerable pain lasting one to two houres with rhinorrhea (cluster like headache). Magnetic resonance imaging showed a pituitary adenoma which invaded into right cavernous sinus. Serum hormone level was almost normal, therefore we diagnosed nonfunctional pituitary adenoma. The pharmacological preventive treatments for headache, such as loxoprofen, sumatriptan, lomerizine had no effect. On the other hand, verapamil showed moderate improvement of his headache, then the symptons was gradually getting better and finally he felt no pain. This case demonstrated occurence mechanism of cluster headache and preventive effect of verapamil associated with cavernous sinus.

Increase of serum vascular endothelial growth factors in wet beriberi: two case reports.

Beriberi is a disease caused by thiamine deficiency resulting in peripheral neuropathy and myocardial dysfunction. Increases in vascular endothelial growth factor (VEGF) are seen in polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, called POEMS syndrome. We present herein two cases of wet beriberi accompanied by a moderate increase in VEGF level. Serum VEGF decreased after treatment in both cases. Our experience with these cases suggests that beriberi should be considered in the differential diagnosis of polyneuropathy with a moderate increase in serum VEGF, and that the serum VEGF level may be a therapeutic marker for beriberi.

Giant cell arteritis: clinical features of patients visiting a headache clinic in Japan.

OBJECTIVE: The first symptom of giant cell arteritis (GCA) is usually a headache. Japan has a low prevalence of GCA, and clinical features of this disorder have not been fully investigated. We conducted a retrospective evaluation of clinical features in patients with giant cell arteritis who visited a headache clinic in Japan. METHODS: Clinical and demographic data were obtained from clinical examinations, face-to-face interviews, and hospital records. PATIENTS: Subjects comprised 19 patients (9 men, 10 women). RESULTS: Mean age at disease onset was 78.1 ± 4.8 years (range, 71-86 years). Seventeen of 19 patients (89.5%) had consulted other medical institutions before consulting our hospital, but only 2 of those patients had been diagnosed with GCA at these medical institutions. Manifestations at disease onset included headache (89.5%), ear pain (5.3%), and jaw pain (5.3%). Ocular manifestations were reported in 2 patients (10.5%). No loss of vision occurred. One patient showed trigeminal nerve palsy involving the third division of the nerve. Jaw claudication was observed in 3 patients (15.8%). Concomitant polymyalgia rheumatica was seen in 3 patients (15.8%). No patient showed upper respiratory tract symptoms, arm claudication, or aortic aneurysms. CONCLUSION: Although most patients had consulted other medical institutions before consulting our hospital, they were not diagnosed with GCA at these institutions. Infrequent clinical findings of GCA and lack of symptoms other than headache may contribute to the high rate of unrecognized and misdiagnosed cases of GCA.

Ischemic stroke associated with cough and cold preparation containing methylephedrine and supplement containing Chinese herbal drugs.

Methylephedrine is generally harmless and is contained in many cough and cold preparations. Likewise, Chinese herbal drugs are considered to be effective and to have few side effects. A 32-year-old woman experienced ischemic stroke attributed to concomitant administration of a cough and cold preparation containing methylephedrine and a supplement containing Chinese herbal drugs. Computed tomography and magnetic resonance imaging of the brain showed acute infarctions bilaterally in the cerebellum. Conventional angiography and magnetic resonance angiography showed transient stenosis of the left vertebral artery. These findings suggested vasospasm or dissection, presumably related to hypertension and/or angiitis or vasoconstriction of large cerebral arteries leading to local thrombosis as a result of stasis and sympathomimetic-induced platelet activation. Combining methylephedrine and Chinese herbal drugs might carry a risk of stroke.

Relapse of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome without increased level of vascular endothelial growth factor following successful autologous peripheral blood stem cell transplantation.

We report a patient with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome who experienced relapse after successful treatment with autologous peripheral blood stem cell transplantation (auto-PBSCT). Interestingly, although vascular endothelial growth factor (VEGF) levels were positively correlated with disease activity before and after auto-PBSCT, as expected, levels did not increase at the time of relapse. The patient was treated with doxorubicin, dexamethasone, and diuretics, followed by thalidomide, and remains clinically stable at one year after treatment. This case suggests that VEGF level may not always be indicative of relapse of POEMS syndrome, and that thalidomide is both useful and safe for patients with relapse following successful treatment with auto-PBSCT.

Legionnaires' disease with hypoperfusion in the cerebellum and frontal lobe on single photon emission computed tomography.

A 59-year-old man was admitted for further investigation of headache. Neurological examination revealed memory loss, disorientation, and bilateral intention tremor. Legionella pneumophila antigen was detected in the urine. Brain magnetic resonance diffusion-weighted images showed marked hyperintensity in the splenium of the corpus callosum without other abnormalities. Single photon emission CT with Tc-99m hexamethyl-propyleneamine oxime showed multi-focal hypoperfusion in the brain, involving mainly the cerebellum and frontal lobe. This is the first report demonstrating cerebellar and frontal lobe hypoperfusion without corresponding MRI abnormalities in a patient with central nervous system Legionnaires' disease.

Efficacy of autologous peripheral blood stem cell transplantation in POEMS syndrome with polyneuropathy.

POEMS syndrome (POEMS) is a rare multi-system disease which is typically associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. We report herein a case of POEMS with progressive polyneuropathy resistant to high-dose intravenous immunoglobulin therapy who experienced improvement in polyneuropathy after the administration of high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. Improvements in polyneuropathy remain despite recognition of monoclonal gammopathy by immunofixation as of 1 year after treatment. We demonstrated that autologous peripheral blood stem cell transplantation can improve clinical symptoms and signs due to polyneuropathy in POEMS even without achieving complete remission.