James E Eaton, Ipek Oguz, Habeeb Kazimuddin, Francesca Bagnato
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引用次数: 0
Abstract
Introduction: We present the case of a gentleman who developed rapidly progressive vision loss, ophthalmo-paresis, and flaccid quadriparesis in the context of severe intracranial hypertension. We reviewed the available cases in the literature to increase awareness of this rare clinical entity.Case Report:A 36-year-old man developed rapidly progressive vision loss, ophthalmo-paresis, and flaccid quadriparesis. He had an extensive workup, only notable for severe intracranial hypertension, >55 cm of H 2 O. No inflammatory features were present, and the patient responded to CSF diversion. Few similar cases are available in the literature, but all show markedly elevated intracranial pressure associated with extensive neuroaxis dysfunction. Similarly, these patients improved with CSF diversion but did not appear to respond to immune-based therapies.
Conclusions: We term this extensive neuroaxis dysfunction intracranial hypertension associated with poly-cranio-radicular-neuropathy (IHP) and distinguish it from similar immune-mediated clinical presentations. Clinicians should be aware of the different etiologies of this potentially devastating clinical presentation to inform appropriate and timely treatment.
导言:我们介绍了一名男性患者的病例,该患者在严重颅内高压的背景下出现了快速进展性视力下降、眼球震颤和弛缓性四肢瘫。我们回顾了现有文献中的病例,以提高人们对这一罕见临床实体的认识。病例报告:一名 36 岁的男子出现了快速进展性视力下降、眼球震颤和弛缓性四肢瘫。他接受了广泛的检查,唯一值得注意的是严重的颅内高压,>55 cm H2O。没有炎症特征,患者对脑脊液引流术反应良好。类似病例在文献中很少见,但都显示颅内压明显升高,伴有广泛的神经轴功能障碍。同样,这些患者在接受脑脊液转移治疗后病情有所好转,但似乎对基于免疫的疗法没有反应:我们将这种广泛的神经轴功能障碍称为颅内高压伴多颅神经病(IHP),并将其与类似的免疫介导型临床表现区分开来。临床医生应了解这种具有潜在破坏性的临床表现的不同病因,以便为适当和及时的治疗提供依据。
期刊介绍:
The Neurologist publishes articles on topics of current interest to physicians treating patients with neurological diseases. The core of the journal is review articles focusing on clinically relevant issues. The journal also publishes case reports or case series which review the literature and put observations in perspective, as well as letters to the editor. Special features include the popular "10 Most Commonly Asked Questions" and the "Patient and Family Fact Sheet," a handy tear-out page that can be copied to hand out to patients and their caregivers.