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actions or secondary bacterial infections in any of these children. Hirayama disease is a rare clinical entity that presents typically as a unilateral, slowly progressive arms weakness, mostly occurring in young men. We report a case of Hirayama disease in a 20-year-old man presenting with a 4-year history of progressive paresthesia starting in his left arm, progressing to the right arm 1 year later. Four months before the presentation, he experienced bilateral foot paresthesias. Examination revealed weakness of the abductor digiti minimi, hallux extension weakness, and postural tremor bilaterally. He had hypersensitivity to pinprick in both hands with ulnar and median distribution. Sensory examination in the legs was normal. He had a postural tremor in both hands, which worsened on neck flexion. Spinal fluid analysis, including oligoclonal band testing, was normal. Electromyography demonstrated bilateral chronic C7 and C8 radiculopathies. Laboratory tests were normal. Flexion-extension magnetic resonance imaging demonstrated laxity of the dura and ligamentum flavum, with compression of cervical cord, maximal at C5-C6 in neck flexion. Laxity of the posterior dura during neck flexion has been postulated to lead to asymmetric lower cervical cord atrophy. Involvement of all 4 limbs is rare, and the condition can be mistaken for progressive multiple sclerosis. Laxity of the posterior dura during neck flexion has been postulated to lead to asymmetric lower cervical cord atrophy. Involvement of all 4 limbs is rare, and the condition can be mistaken for progressive multiple sclerosis. Transcranial color-coded duplex sonography (TCCS) with and without ultrasound contrast agent has been used to diagnose cerebral venous thrombosis, however, no experience in the diagnosis of patients with cerebral venous thrombosis and with dural arteriovenous fistula (dAVF) by contrast-enhanced TCCS has been reported yet. The authors reported a 49-year-old male patient with straight sinus (StS) thrombosis and dAVF. Plain TCCS and contrast-enhanced TCCS demonstrated the direct presentation of the selected feeder and drainage veins of the dAVFs, arterialized venous flow waveform, and an enlarged optic nerve sheath diameter, indicating an increased intracranial pressure, and a filling defect in the StS even after administration of an ultrasound contrast agent. After intravascular thrombolysis and blockage of 2 feeders of the dAVF, TCCS still showed retrograde flow direction in the left basal vein and significantly elevated flow velocity in the StS, reflecting a high venous pressure caused by a high volume of arteriovenous shunt and severe stenosis in the StS. https://www.selleckchem.com/products/a-438079-hcl.html The patient's clinical manifestation did not substantially improve until 3 stents were implanted in the StS, and TCCS unveiled that the stents were well filled with flow signal, and the flow velocity in the StS dramatically decreased. TCCS could provide reliable data about the feeder and drainage veins of dAVF in our case. The extent of an increase in venous flow velocity may be closely associated with clinical manifestations, which may influence therapy. Moreover, TCCS possesses a unique advantage in terms of evaluating the patency of the stents compared with other neuroimaging techniques. TCCS could provide reliable data about the feeder and drainage veins of dAVF in our case. The extent of an increase in venous flow velocity may be closely associated with clinical manifestations, which may influence therapy. Moreover, TCCS possesses a unique advantage in terms of evaluating the patency of the stents compared with other neuroimaging techniques. Individuals with the inherited progressive microangiopathy Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts (CADASIL) most classically develop migraine with aura and recurrent subcortical ischemic infarcts with progressive cognitive decline, gait dysfunction, psychiatric disturbances culminating in early death. However, clinically important venous pathologies may not be anticipated by treating neurologists such as branch retinal vein occlusions (BRVOs). Herein we describe a case of CADASIL with a BRVO and a brief review of venous pathology in CADASIL. A 66-year-old man with CADASIL and clinical symptoms of chronic migraine with aura, episodic "CADASIL coma," recurrent subcortical ischemic infarcts and normal cognition presented with an asymptomatic superior BRVO. Retinal analysis by wide-field fluorescein angiography revealed dye extravasation and optical coherence tomography identified macular edema prompting a monthly regimen of intravitreal bevacizumab. Systemic investigations for prorepresent a structurally causative factor for retinal venous disease. However, these findings are not isolated to the retina as lower extremity varicose veins have associated with a family pedigree of CADASIL. Although presently it is uncertain whether those with CADASIL should undergo routine retinal screening, neurologists, and ophthalmologists, need to be cognizant of the extra-arterial manifestations of CADASIL to provide comprehensive clinical care. We aim to report 3 cases of central nervous system (CNS) vasculitides, in which high-resolution magnetic resonance vessel wall imaging (HR-VWI) findings were instrumental in the diagnosis and management. Case 1 A 41-year-old obese, smoker female with arterial hypertension presented with recurrent transient ischemic attacks. Computed topography angiography demonstrated bilateral middle cerebral artery (MCA) stenosis. HR-VWI revealed uniform enhancement and thickening of the arterial wall, suggestive of MCA vasculitis. The patient reported chronic calf rash that was biopsied and revealed unspecified connective tissue disease. With immunomodulation, patient remained asymptomatic and 6-month surveillance HR-VWI showed improved MCA stenoses.Case 2 A 56-year-old male with herpes simplex virus 1 encephalitis was treated with antiviral therapy and improved clinically. Two months later, the brain magnetic resonance imaging revealed new temporo-parietal edema and distal MCA hyperintense vessels. HR-VWI showed MCA concentric smooth contrast enhancement, that was attributed to postinfectious vasculitis and had resolved on follow-up HR-VWI.
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