Dural arteriovenous fistula in a case of dementia with bithalamic MR lesions T2-weighted MRI of the brain revealed bithalamic hyperintense lesions (figure, A) in a 73-year-old man with a 5-month history of dementia. Although the tentative diagnosis was metabolic encephalopathy or glial tumors, we finally suspected dural arteriovenous fistula (AVF) 1 based on bithalamic vasodilatation on enhanced MRI. Cerebral angiography demonstrated a dural AVF with retrograde flow through the vein of Galen (figure, B). After transarterial embolization, dementia improved accompanied by disappearance of bithalamic abnormal intensities (figure, C). Hypertension of the vein of Galen resulting from a dural AVF should be considered in dementia cases with characteristic bithalamic lesions.
2A. Matsumura, MD, M. Oda, MD, T. Hozuki, MD, T. Imai, MD, PhD, and S. Shimohama, MD, PhD, Sapporo, Japan Disclosure: The authors report no disclosures.
We conducted spinal MR imaging on a 35-year-old man with Lhermitte's sign that had manifested over the previous 4 years. He had consumed more than 500 ml of whisky daily for at least 10 years. However, he did not show any evidence of severe liver disease with hepato-systemic blood shunting. Neurologic examination revealed markedly depressed sense of vibration in the feet and mild spasticity in the lower limbs, together with Lhermitte's sign. MR imaging revealed abnormal signal intensity in the posterior column spanning the whole length of the upper cervical cord, which is consistent with Lhermitte's sign.
Objective We performed a longitudinal study to elucidate the correlation between respiratory insufficiency and respiratory biomarkers, including diaphragmatic compound muscle action potential (DCMAP), at the initiation of noninvasive ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). Methods The patients were assessed at least every six months. Additional assessments were performed at the start of respiratory therapy when the patients met the criteria for the initiation of NIV. Each assessment consisted of a full neurological examination, a phrenic nerve conduction study, respiratory function tests, and nocturnal pulsed oximetry. Patients We enrolled 43 patients with either definite or probable ALS as defined by the revised El Escorial criteria. Results The patients were divided into two groups according to the timing of the initiation of respiratory therapy. Seventeen patients (group A) met the criteria for NIV initiation when their DCMAP remained normal. Twenty-six patients (group B) met the criteria when their DCMAP decreased below normal limits. Although respiratory function parameters were significantly worse in group B compared with group A at NIV initiation, more than 80% of the patients in both groups developed nocturnal desaturation during sleep. Conclusion DCMAP is not always a reliable indicator for determining the optimal timing for NIV initiation during the progression of respiratory insufficiency in ALS. Physicians should be aware of the risk of respiratory insufficiency during sleep in patients with ALS.
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