Rinsho shinkeigaku = Clinical neurology

Publisher: Nihon Shinkei Gakkai


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Publications in this journal

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    ABSTRACT: A 37-year-old woman presented with psychiatric symptoms. Cerebrospinal fluid analysis revealed pleocytosis and increased protein. The patient was diagnosed with limbic encephalitis on the basis of the clinical course. However, remarkable hyponatremia was noted throughout the clinical course, leading to a diagnosis of cerebral salt wasting syndrome (CSWS). The hyponatremia was alleviated by supplementation with sodium and water. The findings seen in this case indicate that differentiation between syndrome of inappropriate of antidiuretic hormone and CSWS is important in cases of hyponatremia accompanied by central nervous system disease.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(8):657-9.
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    ABSTRACT: A 52-year-old woman complained of the sudden onset of a left temporal headache, left neck stiffness and dizziness. Brain magnetic resonance imaging showed a high-intensity lesion in the right medial medulla. Dynamic cerebral angiography revealed vertebral artery dissection and compression at the C6 level due to a transverse process at the C6 level associated with rightward head rotation. Removal of bone and decompression of the vertebral artery were performed from the C5 to C6 levels. Intraoperasively, obstruction of blood flow due to a laryngeal cartilage that rotated with the passive rotation of the patient's head to the right was found. To the best of our knowledge this is the first reported case of vertebral artery occlusion due to a laryngeal cartilage associated with head rotation.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(7):589-92.
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    ABSTRACT: A 83 years old woman was slipped and injured with right femoral neck fracture. After three days from the fracture, she underwent an artificial head bone replacement operation. Immediately after surgery, she complained of chest discomfort, nausea and dyspnea. A few hours later, she became comatose. Brain CT showed no abnormality and clinical diagnosis of heart failure was made without pulmonary embolism on enhanced chest CT. Magnetic resonance imaging (MRI) of the brain next day showed multiple small patchy hyperintense lesion in bilateral hemispheres on diffusion-weighted images (DWI), producing a "star field pattern''. Based on Criteria of Gurd, this patient had one major criterion and four minor criteria. And according to the Criteria of Schonfeld, this patient had 5 points, consistent with clinical diagnosis of fat embolism. Because of these criteria, she was diagnosed as cerebral fat embolism syndrome. We started supported care and edaravon. Two weeks after surgery, her condition recovered and remaind to stuporous state even six month after surgery. We experienced a typical case of cerebral fat embolism, after bone surgery with diagnostic findings on MRI-DWI. Diagnosis of cerebral fat embolism syndrome requires a history of long bone fracture and/or replacing surgery with typical finding on MRI images, such as "star field pattern''.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(8):648-52.
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    ABSTRACT: Patient 1 was a 40-year-old man, who suffered from right leg myoclonus 1 week after an episode of fever and headache. Myoclonus disappeared 4 months after administration of clonazepam. Patient 2 was a 42-year-old man, who suffered from right leg myoclonus, attacks of speech arrest and a generalized tonic-clonic seizure. His symptoms disappeared after steroid-pulse therapy, but right leg myoclonus and episodic impairment of consciousness recurred within a month. He underwent another steroid-pulse therapy and his symptoms disappeared. In both patients, cerebrospinal fluid (CSF) study showed pleocytosis and elevated protein level, electrophysiological study showed cortical reflex by stimulation of the right tibial nerve, and brain MRI showed the high intensity area in the left parietal lobe. In addition, on electroencephalogram (EEG) spikes at vertex preceded myoclonic jerk of the right tibialis anterior muscle in both patients. These findings indicate that focal cortical reflex myoclonus was accompanied by acute central nervous system (CNS) infection. Furthermore, in both patients, autoantibody against glutamate receptor subunits ε2 was detected both in serum and CSF, which also suggest that autoimmune mechanism contributed in the pathophysiology of acute development of focal cortical reflex myoclonus.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(7):543-9.
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    ABSTRACT: We report a patient of 32-year-old female with central IgG4-related disease. She developed headache and visual disturbance. On examination, she revealed diabetes insipidus, retrobulbar neuritis, hyperreflexia and limb weakness. Her laboratory findings showed serum IgG4 elevation, pleocytosis and protein elevation in cerebrospinal fluid. Chest CT showed a nodular lesion in the S8 of the left lung. Cranial and spinal magnetic resonance images with gadolinium contrast material showed cranial, cervical and lumbosacral hypertrophic pachymeningitis associated with infundibulo-hypophysitis. Pathological findings of the left frontal dura mater revealed lymphoplasmacytic inflammatory cell infiltrate with dense fibrosis. IgG4 immunohistochemistry showed no IgG4 + plasma cells within the inflammatory infiltrate. During treatment with intravenous pulse methylprednisolone followed by oral prednisolone, she revealed recovery of visual acuity with improvement of hypertrophic pachymeningitis and normalization of serum IgG4. This is a first report of IgG4-related hypertrophic pachymeningitis which involved cranial, cervical and lumbosacral regions as well as infundibulo-hypophysitis in a young female.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(8):664-7.
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    ABSTRACT: A-47-year-old woman was admitted to our hospital because of eruption, severe arthritis, myalgia, pharyngalgia and the elevation of serum creatine kinase. She was diagnosed with dermatomyositis based on a biopsied specimen and clinical symptoms. Serum anti CADM-140 antibody was detected by immunoprecipitation. Pulse therapy with methylprednisolone and cyclophophamide could transiently improve myalgia and so on, but she died of rapidly progressive interstitial lung disease. Autopsy findings demonstrated diffuse alveolar damage and alveolar hemorrhage. It is said that patients with anti CADM-140 antibody show poor muscle symptoms and alveolar hemorrhage has been described very rarely. This patient is the rare case of dermatomyositis with anti CADM-140 antibody developing severe muscle symptoms, pharyngalgia and aleveolar hemorrhage in autopsy findings.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(5):408-12.
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    ABSTRACT: A 62-year-old man complained of resting tremor and posture tremor. In spite of presence of the tremor, other parkinsonian component was very mild. [(11)C]2β-carbomethoxy-3β-(4-fluorophenyl)-tropane ([(11)C]CFT) PET showed asymmetrical reduction of the uptake and [(11)C]raclopride PET showed slightly increased uptake in the striatum. Although he was diagnosed as having benign tremulous parkinsonism (BTP), anti-parkinsonian medications, including anti-cholinergic agent, dopamine agonist and l-dopa, were not effective for his tremor. His tremor gradually deteriorated enough to disturb writing, working, and eating. Because his quality of life (QOL) was disturbed by the troublesome tremor, deep brain stimulation of the subthalamic nucleus (STN-DBS) was performed. After STN-DBS, his tremor was dramatically improved. According to clinical course of our patient as well as previous reports, STN-DBS should be considered as a therapeutic option for BTP patients with severe tremor.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(6):511-4.
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    ABSTRACT: The patient was a 53-year-old male. He showed steppage gait at the age of 11 and equinus foot at 13. He walked unaided with shoe-insoles to support his heels. Atrial fibrillation and cardiac hypertrophy were found in his 30s, and ventricular tachycardia (VT) was observed at the age of 48. Electrophysiological studies were performed, but VT was not sustained, symptomatic, or showed signs of infra-Hisian block, and a pacemaker was not indicated. At 53, he was introduced to a neurologist because of tetraplegia after the first episode of syncope. A spinal MR showed ossification of posterior longitudinal ligament (OPLL) and central cervical cord injury. Furthermore, he presented not only contracture in his shoulder, elbow, and ankles but also atrophy in his scapulohumeral and gastrocnemius muscles. In accordance with a diagnosis of Emery-Dreifuss muscular dystrophy (EDMD), provocative testing of VT was carried out, and a cardiac resynchronization therapy defibrillator (CRT-D) was implanted. Later, a mutation analysis of the LMNA gene disclosed a known missense mutation of p.Arg377His, and we diagnosed him as EDMD2 (laminopathy). Contractures could be the clue to diagnose EDMD and indicate the need for pacemakers and defibrillators in patients with cardiac conduction disorders.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(6):489-94.
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    ABSTRACT: We report a case of complete Heerfordt syndrome accompanied by the involvement of small fiber neuropathy (SFN) manifesting as refracory facial pain. A 30-year-old man presented with pyrexia, a 2-week history of facial burning pain, and difficulty of mastication. After admission to our hospital, neurological examinations showed bilateral facial pain, trigeminal motor palsy, left facial nerve palsy, bilateral sensory neural deafness, uveitis and swelling of the parotid gland. Other examinations revealed bilateral hilar lymphadenopathy, high serum titer of angiotensin coenzyme, and no response in a tuberculin-tested, non-caseating epithelioid granuloma from lip biopsy, leading to the diagnosis of complete Heerfordt syndrome. Mandibular skin biopsy with immunostaining for PGP 9.5 showed SFN. High-dose corticosteroids proved somewhat effective against SFN as facial pain, but reducing the corticosteroid dose proved difficult, as symptoms were refractory to other immunosuppressants and pain-control drugs such as anti-epileptics and anti-depressants. The patient died of acute pancreatitis 3 years after disease onset. Autopsy showed no granuloma in hilar lymph node, trigeminal nerve, cranial base, nerve root, and muscle. SFN in this case probably represent a cause of refractory facial pain.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(7):585-8.
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    ABSTRACT: A 48-year-old woman with no previous neurological diseases was transferred to our hospital because of sudden-onset unconsciousness. On arrival, she showed consciousness disturbance (E1V1M3 on the Glasgow Coma Scale), tetraplegia, right conjugate deviation and bilateral pathological reflexes. These symptoms resulted in a NIH stroke scale score of 32. Brain diffusion-weighted MR imaging (DWI) showed multiple hyper-intense lesions, and MR angiography revealed occlusions of the basilar artery (BA) and superior branch of the right middle cerebral artery (MCA). Transthoracic echocardiography disclosed a 51 × 24 mm myxoma in the left atrium. These findings led to diagnosis of acute ischemic stroke due to embolization from cardiac myxoma. Thrombolytic therapy with intravenous tissue plasminogen activator (IV tPA) was started 120 min after onset because there were no contraindications for this treatment. However, the symptoms did not resolve, and thus endovascular therapy was performed immediately after IV tPA. Angiography of the left vertebral artery initially showed BA occlusion, but a repeated angiogram resulted in spontaneous recanalization of the BA. However, the left posterior cerebral artery remained occluded by a residual embolus. Subsequently, occlusion found in the superior branch of the right MCA was treated by intra-arterial local thrombolysis using urokinase and thrombectomy with a foreign body retrieval device, but the MCA remained occluded. DWI after endovascular therapy showed new hyper-intense lesions in the bilateral medial thalamus and left occipital cortex. Clinically, neurological status did not improve, with a score of 5 on the modified Rankin Scale. IV tPA can be used for stroke due to cardiac myxoma, but development of brain aneurysms and metastases caused by myxoma is a concern. Given the difficulty of predicting an embolus composite from a thrombus or tumor particle, aspiration thrombectomy may be safer and more effective for stroke due to cardiac myxoma to avoid delayed formation of brain aneurysms and metastases.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(6):502-6.
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    ABSTRACT: Most scalp neuralgias are supraorbital or occipital. Although they have been considered idiopathic, recent studies revealed that some were attributable to mechanical irritation with the peripheral nerve of the scalp by superficial anatomical cranial structures. Supraorbital neuralgia involves entrapment of the supraorbital nerve by the facial muscle, and occipital neuralgia involves entrapment of occipital nerves, mainly the greater occipital nerve, by the semispinalis capitis muscle. Contact between the occipital artery and the greater occipital nerve in the scalp may also be causative. Decompression surgery to address these neuralgias has been reported. As headache after craniotomy is the result of iatrogenic injury to the peripheral nerve of the scalp, post-craniotomy headache should be considered as a differential diagnosis.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(5):387-94.
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    ABSTRACT: A 42 years-old male noticed alopecia in 2006. Three years later, he had loss of hair in the whole body and weakness in the left upper limb. Prednisolone treatment failed to alleviate these symptoms. In 2011, he was diagnosed multifocal acquired demyelinating sensory and motor neuropathy and treated by intravenous immunoglobulin (IVIg), which improved muscle strength eventually. Three years later, he had remitting weakness of the limbs, and thus underwent again IVIg with amelioration of both alopecia and weakness in the limbs. Association of multifocal acquired demyelinating sensory and motor neuropathy and alopecia, and their improvement by immunomodulatory treatment strongly suggest a role of immunological pathomechanism.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(6):507-10.
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    ABSTRACT: We report two cases of cerebral venous thrombosis as a complication of nephrotic syndrome. No urine protein or kidney disease was noted in either case. The patients were diagnosed with nephrotic syndrome after admission to our hospital. Case 1: The patient was a 46-year-old man. He experienced headache and vomiting the day after he drank heavily. Contrast brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed a defect in the transverse sinus, straight sinus, and superior sagittal sinus. His blood was hemo-concentrated, and blood test results indicated high D-dimer and fibrinogen levels and decrease of antithrombin III. Case 2: The patient was an 89-year-old woman. After the diarrhea lasted suffering from ischemic colitis, she developed left hemiplegia and headache. Brain CT revealed hematoma in the subcortical region of the right frontal lobe and a high signal in the straight sinus. The superior sagittal sinus showed high-signal intensity on T1-weighted MRI and mild high-signal intensity on T2-weighted MRI. High fibrinogen levels were detected in the blood. Patients with nephrotic syndrome have a thrombotic tendency; both venous thrombosis and arterial thrombosis may occur. In the literature, the number of published cases of cerebral venous thrombosis was 10-fold that of cerebral artery thrombosis as a complication of nephrotic syndrome in individuals aged <20 years. In adults, however, the number of cerebral venous thrombosis was 2-fold that of cerebral artery thrombosis cases were reported. Nephrotic syndrome shows a thrombotic tendency, but cerebral venous thrombosis may develop as a result of another thrombotic factor. Management of life along with the conventional treatment of nephrotic syndrome is important.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(6):495-501.
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    ABSTRACT: Multiple mitochondrial DNA (mtDNA) deletions usually occur secondarily to a mutation in one of the enzymes involved in mtDNA maintenance, such as polymerase γ, which is encoded by the nuclear polymerase γ1 gene (POLG1) and POLG2. Patients with multiple mtDNA deletion disorders show clinical heterogeneity of symptoms, in addition to usually seen progressive external ophthalmoplegia (PEO). We conducted clinical, histological and genetic analyses of two affected sisters in a family with the autosomal dominant inheritance pattern of PEO. A 73-year-old woman (patient 1) with congenital hypogonadism and PEO developed L-dopa responsive parkinsonism about the age of 60. Neurological examination revealed mild proximal muscle weakness and polyneuropathy too. Her 69-year-old sister (patient 2) also showed PEO, parkinsonism and polyneuropathy. Histopathological studies of biopsied muscle specimens from patient 1 revealed numerous ragged red fibers as well as fibers with increased succinate dehydrogenase activity and decreased cytochrome c oxidase activity. Multiple mtDNA deletions were detected, both by Southern blot and long-range PCR assays of total DNA from the biopsied muscle specimens. A systemic mutational analysis in both sisters revealed a heterozygous p.Y955C (c.2864A>G) mutation in POLG1. This is the first Japanese family identified with this mutation. We reviewed cases with this mutation highlighting a wide phenotypic spectrum of this disorder.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(5):417-22.
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    ABSTRACT: A 73-year-old man was admitted to our hospital because of a decrease in spontaneity. His medical history included two stroke episodes, probably related to hypertension. Brain MRI on admission demonstrated acute infarction in the right caudate nucleus and left putamen. Intravenous infusion of a low molecular-weight heparin added to oral antiplatelets was started. Following admission, he developed a low grade fever and severe inflammatory reaction. The focus of infection was not evident, and none of the antibiotics tried were effective. Ten days after admission, he developed right hemiparesis, and an additional brain MRI showed new multiple infarctions. We also determined the presence of a high MPO-ANCA titer (57 EU), and we diagnosed the patient's condition to be ANCA-associated vasculitis (AAV). Steroid therapy improved his inflammatory reaction and stroke recurrence was not observed. We suggest that vasculitis should be considered as a potential risk factor for repeated small infarctions with fever of unknown origin, especially those of perforating artery territories.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(5):429-33.
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    ABSTRACT: Transient neurological attacks (TNAs) are attacks with temporary (< 24 h) neurological symptoms, and reported in 1975. Ischemic cerebral infarction often occurres after transient neurological symptoms which are not sometimes involved in transient ischemic attack (TIA) criteria. The purpose of this study was to clarity transient neurological symptoms before the onset of ischemic stroke. Consecutive acute stroke and TIA patients within 7 days of onset between July 2011 and December 2012 were studied. Questionnaire survey of transient neurological symptoms was done and we investigated the history of transient neurological symptoms and divided the patients into three groups, TIA group (history of TIA), TNAs group (history of TNAs), and no past history group. We compared clinical characteristics, the rate of posterior circulation lesions, and number of attacks among three groups. 184 patients (male 112 patients, mean 73 years old) were enrolled into the present study. TNAs group had 13 (7%) patients, and TIA had 20 (11%). Neurological symptoms of TNAs group were 5 (38%) transient dizziness, 3 (23%) disturbance of consciousness, 2 (23%) weakness of legs, 1 (8%) sensory disturbance of legs, 1 (8%) gait disturbance, and 1 (8%) sensory disturbance of lips. Five (38%) TNAs group had multiple attacks. In 33 transient neurological symptom patients, 17 (52%) patients did not have hospital visit, and 7 (21%) patient did not have antiplatelet and anticoagulant drug. TNAs group more frequently had hyperlipidemia, diabetes mellitus, and posterior circulation lesions among three groups. 20% posterior circulation lesion patients had TNAs history, and posterior circulation might be examined in TNAs patients.
    Rinsho shinkeigaku = Clinical neurology 01/2014; 54(6):480-3.