Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a non-arterio-atherosclerotic, non-amyloidotic arteriopathy affecting preferentially the small arteries and arterioles of the brain. The morphologic hallmark is the presence of a characteristic granular alteration of the arterial media that ultrastructurally corresponds to the accumulation of electron-dense material surrounding the smooth muscle cells. Although the presence of this granular osmiophilic material (GOM) was originally described as limited to brain vessels, identical electron microscopic findings have been demonstrated in the media of peripheral tissue arteries, allowing for a pathologic diagnosis of the disease by a simple skin, muscle or nerve biopsy. We report some atypical features identified in our CADASIL patients that broaden the phenotypic expression of this disease. Firstly, we identified a cortical infarct in an otherwise typical CADASIL patient. Secondly, we observed GOM in skin arteries of a 30-year-old man with hemiplegic migraine, the son of a woman who had died with CADASIL. This confirms that it may be possible to diagnose the disease at a preclinical stage by the ultrastructural evaluation of peripheral tissue biopsy material, particularly for individuals for whom there is a supporting family history. Thirdly, ultrastructural examination of the skin, and subcutaneous and striated muscle of an unrelated and apparently sporadic patient with neuropathologic and neuroradiologic evidence of CADASIL in meningeal and cerebral vessels failed to reveal diagnostic lesions in peripheral arteries. Thus, the possibility of a false-negative pathologic diagnosis in patients with a clinicoradiologic diagnosis of CADASIL, if one relies solely on a peripheral tissue biopsy, does exist. Additionally, we have identified heat shock proteins (Hsp70 and alphaB crystallin) and ubiquitin in the vascular myocytes of affected arteries. B crystallin also seemed to be deposited extracellularly, which suggests that GOM also might be immunoreactive for alphaB crystallin.
The collaborative care model (CoCM) has substantial support for improving behavioral health care in primary care. However, large-scale CoCM adoption relies on addressing operational and financial implementation challenges across health care settings with varying resources. An academic medical center serving socioeconomically and racially diverse patients implemented the CoCM in seven practices. A smartphone application was introduced to facilitate CoCM care management during depression treatment (app-augmented CoCM). App features included secure texting, goal/appointment reminders, symptom monitoring, and health education material. A nonrandomized convenience patient sample (N = 807) was enrolled in app-augmented CoCM and compared with patients in standard CoCM (N = 3,975). Data were collected on clinical contact frequency, engagement, and clinical outcomes. App-augmented CoCM patients received more health care team contacts (7.9 vs. 4.9, p < .001) and shorter time to follow up compared with the standard CoCM sample (mean = 11 vs. 19 days, p < .001). App-augmented CoCM patients had clinical outcomes similar to the standard CoCM group (47% vs. 46% with ≥50% depression improvement or score <10), despite app-augmented patients having more prior depression treatment episodes. Further, the app-augmented group with greater app engagement demonstrated increased behavioral health appointment compliance, including more completed appointments and fewer no shows, and greater depression symptom improvement than those with less app engagement. App-augmented CoCM may improve patient engagement in treatment and provide opportunities to implement key CoCM elements without overburdening practice resources. CoCM sustainability and scalability in primary care may be enhanced by using this technology.
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