Acquired (Non-Wilsonian) hepatocerebral degeneration

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Imaging features in Hirayama disease

Purpose: To evaluate the MR findings in clinically suspected cases of Hirayama disease. Materials and Methods: The pre and post contrast neutral and flexion position cervical MR images of eight patients of clinically suspected Hirayama disease were evaluated for the following findings: localized lower cervical cord atrophy, asymmetric cord flattening, abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior shifting of the posterior wall of the cervical dural canal and enhancing epidural component with flow voids. The distribution of the above features in our patient population was noted and correlated with their clinical presentation and electromyography findings. Observations: Although lower cervical cord atrophy was noted in all eight cases of suspected Hirayama disease, the rest of the findings were variably distributed with asymmetric cord flattening, abnormal cervical curvature, anterior shifting of the posterior wall of the cervical dural canal and enhancing epidural component seen in six out of eight (75%) cases. An additional finding of thoracic extension of the enhancing epidural component was also noted in five out of eight cases. Conclusion: Dynamic post contrast MRI evaluation of cervicothoracic spine is an accurate method for the diagnosis of Hirayama disease

Recommendations on the use of EEG monitoring in critically ill patients: consensus statement from the neurointensive care section of the ESICM.

OBJECTIVES: Recommendations for EEG monitoring in the ICU are lacking. The Neurointensive Care Section of the ESICM assembled a multidisciplinary group to establish consensus recommendations on the use of EEG in the ICU. METHODS: A systematic review was performed and 42 studies were included. Data were extracted using the PICO approach, including: (a) population, i.e. ICU patients with at least one of the following: traumatic brain injury, subarachnoid hemorrhage, intracerebral hemorrhage, stroke, coma after cardiac arrest, septic and metabolic encephalopathy, encephalitis, and status epilepticus; (b) intervention, i.e. EEG monitoring of at least 30 min duration; (c) control, i.e. intermittent vs. continuous EEG, as no studies compared patients with a specific clinical condition, with and without EEG monitoring; (d) outcome endpoints, i.e. seizure detection, ischemia detection, and prognostication. After selection, evidence was classified and recommendations developed using the GRADE system. RECOMMENDATIONS: The panel recommends EEG in generalized convulsive status epilepticus and to rule out nonconvulsive seizures in brain-injured patients and in comatose ICU patients without primary brain injury who have unexplained and persistent altered consciousness. We suggest EEG to detect ischemia in comatose patients with subarachnoid hemorrhage and to improve prognostication of coma after cardiac arrest. We recommend continuous over intermittent EEG for refractory status epilepticus and suggest it for patients with status epilepticus and suspected ongoing seizures and for comatose patients with unexplained and persistent altered consciousness. CONCLUSIONS: EEG monitoring is an important diagnostic tool for specific indications. Further data are necessary to understand its potential for ischemia assessment and coma prognostication