Silent cerebral ischemic lesions after carotid artery stenting with distal cerebral protection.

OBJECTIVE: To evaluate by MR diffusion weighted image (DWI-MR) the presence of new ischemic cerebral lesions after carotid artery stenting (CAS) and distal cerebral protection, in patients with internal carotid artery (ICA) stenosis >70. METHODS: Sixty-seven CAS were performed under cerebral protection with a distal filter. Mean age of the patients was 68.3 years (range 37-86) and 42 patients (62.7%) were symptomatic. An EZ filter device was used in all cases. The mean length of the procedure was 22.2 minutes (range 8-110). All patients had a cerebral MRI done in the 3 days before CAS and a DW-MR (eco planar single shot, b=1000 mm2/seconds) was done the day after. RESULTS: As a consequence of the CAS, three transient ischemic attacks were observed. There was one minor stroke (1.5%) on day 21, but no major stroke, death or myocardial infarction in a 30-day period. DW-MRI after CAS showed 26 new silent ischemic lesions in 11 asymptomatic patients (16.4%). In six, they were multiple (range 2-5). Lesions were mainly seen in the ipsilateral medial cerebral artery (21); four in the posterior fossa, and one in the contralateral medial cerebral artery. DISCUSSION: Although the use of distal cerebral protection was safe, new cerebral ischemic lesions, supposedly embolic, were observed in 16.4% of the patients. Although without clinical consequences in our series, their moderate high incidence should promote the investigation of safer techniques and devices.

Hereditary vacuolar internal anal sphincter myopathy causing proctalgia fugax and constipation: a new case contribution.

Hereditary anal sphincter myopathy is rare. We present a family with one affected member with proctalgia fugax, constipation and internal anal sphincter hypertrophy. Ultrastructural findings show vacuolization of smooth muscle cells without the characteristic polyglucosan inclusion. Further relief of symptoms was obtained using an oral calcium antagonist. Based on clinical presentation, endosonography and morphological findings, we consider our case is a histological variant of the vacuolar myopathy originally described.

Infantile systemic hyalinosis: a clinicopathological study.

Infantile systemic hyalinosis (ISH) is a presumed autosomal recessive connective tissue condition. Symptoms usually begin at birth or shortly thereafter, and are characterized by pain when handled, painful and swollen joints and, later on, dermal anomalies, diarrhea, failure to thrive and recurrent infections, which usually lead to death around the age of 2. The skin has generally diminished elasticity with small pearly papules appearing on neck, ears, coccygeal region, and face. We present two unrelated patients with ISH, with specific focus on clinical and pathologic studies. In the first patient the diagnosis was made several years after she died, in a retrospective study of her clinical file. On ultrastructural examination both patients showed an accumulation of fibrillogranular material in the extracellular matrix with long-spacing collagen of 90 nm. The first child died at the age of 1(1/2) years and the second at 3 years.