Posterior atlantoaxial fusion as treatment option for extracranial vertebral artery dissecting aneurysm: a case report and literature review.

Symptomatic extracranial vertebral artery (VA) dissection may need surgery. We describe such a case successfully treated with atlantoaxial fusion based on its rare dynamic angiographic findings. A 27-year-old woman suffered from repeated brainstem and cerebellar infarctions from a left extracranial VA dissecting aneurysm. Dynamic angiography showed the dissecting aneurysm of the V3 segment in the neutral head position, and deflation of the aneurysm during rightward head rotation. She underwent posterior atlantoaxial fusion, and the lesion was repaired with no subsequent ischemia. Posterior atlantoaxial fusion can be an option for some extracranial VA dissections with preserving its anterograde blood flow.

[Acute myelitis with Brown-Séquard syndrome complicated with subjective sudomotor laterality and ileocecal abscess without right abdominal pain].

A 47-year-old man presented with left shoulder pain and muscle weakness in the left limbs on November 2017. On the next day, he experienced dysesthesia of the right limbs and hypohidrosis of the left limbs and developed thermal hypoalgesia in right side of body and muscle weakness of the left upper and lower limbs progressed. He was diagnosed with acute myelitis and Brown-Séquard syndrome, based on cervical MRI scan. Muscle strength improved after steroid therapy and plasma exchange. He experienced complications of intraabdominal abscess in the right side during immunological therapy, although he only had a symptom of left abdominal pain, without pain in the right side. It is noteworthy that abdominal hypoalgesia can be associated with Brown-Séquard syndrome. Characteristically, MRI revealed bilateral lesions at the C3/4 cervical spine level. This report shows that in Brown-Séquard syndrome associated with bilateral spinal lesions, the abdominal visceral sensory pathway, in addition to the somatosensory pathway, could be impaired bilaterally, resulting in aggravation of abdominal hypoalgesia.

[A case of Bickerstaff brainstem encephalitis concomitant with axonal Guillain-Barre syndrome and ballism successfully treated with intravenous immunoglobulin treatment].

A 39-year-old man acutely developed diplopia, vertigo, unsteady gait, and disturbance of consciousness following an upper respiratory infection. Neurological examination showed ophthalmoplegia, facial paralysis, tetraplegia and loss of deep tendon reflexes. Babinski reflex was positive on the left and there were bilateral flexor withdrawal reflexes. He also developed ballism-like involuntary movements in all extremities, loss of proprioception predominantly on the left, and severe truncal ataxia. Anti-GQ1b IgG antibody was selectively elevated in serum, and CSF protein was elevated to 53 mg/dl with cell count of 12/mm3. Nerve conduction study showed decreased amplitude of compound motor action potentials in all extremities, and no response in facial muscles. Cranial MRI showed no abnormalities whereas EEG was severely abnormal with lack of posterior dominant rhythm and the presence of continuous diffuse theta-waves. This case presented clinical characteristics of three syndromes concurrently-Fisher syndrome, Bickerstaff brainstem encephalitis, and Guillain-Barré syndrome-that may be collectively called 'anti-GQ1b IgG antibody syndrome'. The unique feature of the present case was development of deep coma and ballism-like movements, associated with selective increase of serum anti-GQ1b IgG antibody. It is thus conceivable that anti-GQ1b IgG antibody might underlie the pathogenesis of all three conditions.

[A case presenting with both features of essential tremor and Parkinson tremor].

A 78-year-old woman had postural and action tremor in hands since age of late 20's, though without much difficulty in daily living. Since age 76, she has had an increasing difficulty in walking and postural balance, with some worsening of hand shaking as well. She also noted to have head shaking, involuntary movements in the mouth, and some difficulty in swallowing. Reportedly both of her parents and her brother had hand tremor. Neurologically she had resting, postural and action tremors in hands more on the left. She also had marked rigidity in the neck and moderate cogwheel rigidity in all limbs more on the left, moderate bradykinesia, markedly stooped posture with relatively wide base, slow and small-paced gait, and poor postural balance more posteriorly. Thus, it is most likely that the present case suffered from essential tremor and later developed Parkinson disease. Power spectrum analyses of surface electromyogram and accelerometer showed the peaks at 4.3 Hz for the resting tremor, and 3.1 and 5.2 Hz for the postural tremor. Furthermore, significant EEG-EMG coherence was seen at the peaks of 4.3 and 5.2 Hz, suggesting possible involvement of sensori-motor cortex for generation of both tremors. When the postural tremor was loaded with 500 g weight, the peak of power spectrum and EEG-EMG coherence showed the broader pattern with the maximal peak at 4.3 Hz, exactly the same frequency as that of the resting tremor. These findings may be explained by postulating that the 4.3 Hz peak of the resting tremor became apparent by loading because 5.2 Hz peak of the postural tremor was suppressed by the loading. The peak of the postural tremor at 3.1 Hz which was not detected by EEG-EMG coherence suggested that the tremor for 3.1 Hz was not involved to sensori-motor cortex for generation.

[Concurrence of myasthenia gravis, polymyositis, thyroiditis and eosinophilia in a patient with type B1 thymoma].

We presented a 43-year-old Japanese woman who acutely developed weakness of all extremities and difficulty in swallowing and drooping of eyelids, characterized by easy fatigability at the end of December, 2005. On general physical examination, she had moderate goiter. No cervical lymphadenopathy, cardiac murmur, or skin rash was noted. Neurologically, she had blepharoptosis, more on the right, only in the upright position with easy fatigability and marked weakness in the neck flexor, trunk, and all limb muscles much more proximally than distally. She had neither muscular atrophy nor upper motor neuron sign. Laboratory data showed slight leukocytosis with eosinophilia (up to 31%), and serum creatine kinase was markedly increased to over 2,000 IU/l. TSH receptor antibody (11.9%) and anti-acetylcholine receptor antibody (46.6 nmol/L) were also increased. Edrophonium test was positive. Electrophysiologically, muscle evoked potentials by repetitive motor nerve stimulation showed 13% and 50% waning in abductor pollicis brevis and deltoid muscle, respectively, at low frequency and no waxing at high frequency. Needle EMG showed fibrillation potentials and positive sharp waves in proximal muscles. Polymyositis was diagnosed by muscle biopsy which showed infiltration of lymphocytes in the endomysium and around non-necrotic muscle fibers. Upper arm muscle MRI showed multifocal high signal intensity lesions on T2-weighted images which were likely related to myositis. This finding is atypical for polymyositis. X-ray and CT of chest showed a mass lesion in the left pulmonary hilum, which was histologically diagnosed as type B1 thymoma. Thus, the present case had myasthenia gravis, polymyositis, thyroidititis and eosinophilia associated with type B1 thymoma. After the thymectomy, corticosteroid administration and immunoadsorption therapy, clinical symptoms and all laboratory abnormalities markedly improved.

[Case presenting both clinical features of progressive supranuclear palsy and amyotrophic lateral sclerosis].

A 70-year-old Japanese male farmer, born and living in Kyoto prefecture, developed gait disturbance, with tendency to fall at age 68, and muscle atrophy and weakness of the right shoulder and arm a year and half later. All symptoms have been progressive ever since. The patient manifested marked dementia, parkinsonism associated with limitation of ocular movements in all directions with slow saccade, loss of startle reflex, asymmetric muscle atrophy and weakness in shoulder girdles and upper limbs with hyperreflexia, and positive Babinski reflexes. The needle electromyogram showed evidence of active denervation. Brain MRI showed cortical atrophy in the frontal and temporal lobes, and midbrain tegmentum. Cerebral blood flow image on SPECT suggested hypoperfusion in the frontal, temporal and parietal cortices and basal ganglia bilaterally. Thus, it is most likely that the present case suffered from clinical features of amyotrophic lateral sclerosis (ALS) and progressive supranuclear palsy at the same time. Relation to the ALS/Parkinsonism/Dementia complex reported from Kii peninsula and Guam was discussed.

[Remission of lymphomatosis cerebri induced by corticosteroid and high-doses intravenous methotrexate].

Lymphomatosis cerebri (LC) is a rare form of primary central nervous system lymphoma characterized by subacute progressive dementia and unsteady gait. MRI study of LC typically reveals diffuse leukoencephalopathy without contrast enhancement. The clinical presentation and MRI features of LC can resemble infectious, inflammatory, toxic or vascular leukoencephalopathy. Hence diagnosis of LC is easily mistaken for other, more common diseases. In this report, we present a case of a 55-year-old man presenting with subacute progressive dementia and ataxic gait. Brain MRI showed diffuse hyperintense lesions in the cerebral white matter of both hemispheres, left amygdala, brainstem and cerebellar peduncles on FLAIR image. No contrast-enhanced lesion was observed. Cerebrospinal fluid analysis showed elevated levels of soluble interleukin-2 receptor and ß2-microglobulin. Based on MRI findings and 123I-IMP SPECT, stereotactic biopsy targeting white matter of the left medial temporal lobe was performed (day 0). On the day after the brain biopsy, corticosteroid therapy was initiated and improved the patient's cognitive function and gait disturbance. Pathological diagnosis of large B-cell lymphoma was obtained on day 9. High-dose intravenous methotrexate chemotherapy was started on day 14 and led to complete remission by day 52. This case highlighted the importance of brain biopsy for diagnosis of LC. This report raises a possibility that timely and proper treatment leads to a favorable outcome of LC that has been regarded as an intractable disease with poor prognosis.