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Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake's pouch: two separate entities.

Cystic malformations of the posterior cranial fossa are all but arachnoid cysts contained within the general context of the Dandy-Walker complex and may be further classified in two groups on the basis of their embryological origin: anomalies of the anterior membranous area (AMA) and anomalies of the posterior membranous area (PMA). Whether the latter group of malformations can be regarded as separate entities is still quite controversial. The present authors give a detailed account of the various embryological stages in the formation of the posterior cranial fossa and its contents and propose the identification of two anomalies derived from a defect of the PMA: the mega cisterna magna (MCM) and the persisting Blake's pouch, a new entity with different MRI features from MCM. Criteria for their recognition are discussed, stressing the capital importance of a differential diagnosis in view of the radically different therapeutic approach.

Arachnoid Cysts↗

Predominantly upper limb weakness, enlarged cisterna magna, and borderline intelligence in a child with de novo mutation of the skeletal muscle alpha-actin gene.

We present a 10-year-old boy from nonconsanguineous parents of Libyan (Sephardi) Jewish origin. Mild dysmorphism, hypotonia, and clubfoot deformities were noted at birth. On follow-up, he had borderline intelligence and nonprogressive muscle weakness, predominantly in the upper extremities. Physical examination revealed mild facial weakness, a bell-shaped chest cavity, kyphosis, winging of the scapula, and hypotonia of the shoulder girdle. Muscle biopsy demonstrated prominent variation in fiber size and central nuclei and numerous subsarcolemmal particles on modified Gomori trichrome stains. Electron microscopy depicted areas of disrupted sarcomeres with abnormal aggregates. Brain magnetic resonance imaging showed mild widening of the lateral ventricles and an enlarged cisterna magna. Molecular DNA analysis by polymerase chain reaction (PCR) and direct sequencing revealed a de novo heterozygous missense mutation in the skeletal muscle alpha-actin gene (ACTA1) changing codon 348 from TCG serine to TTG leucine.

Actins↗

Decreased cardiovascular responses to cisterna magna NaCl injection in hypertensive rats.

Albino rats were made hypertensive by 1% NaCl in the drinking water for 4-5 months, systolic (SBP) and distolic blood pressure (DBP) were 164.0 +/- 10.1 mm Hg and 118.7 +/- 4.6 mm Hg respectively, vs. control rats whose SBP and DBP were 119.0 +/- 4.4 mm Hg and 86.8 +/- 4.3 mm Hg respectively. Psychosocial stress (1 hour daily for 4-5 months) only raised SBP to 140.0 +/- 5.2 mm Hg; DBP remained unaltered. One percent NaCl intake combined with psychosocial stress, increased SBP and DBP but not significantly beyond the level observed with single 1% NaCl administration. Formerly described control and hypertensive rats were anesthetized with sodium pentobarbital (40 mg/kg) and stereotaxically injected into de cisterna magna (i.c.) with 20 microliters of 1.5 M NaCl solution. During i.c. injection, intraarterial SBP, DBP and heart rate (HR) were continuously recorded. After i.c., 1.5 M NaCl injection, mean arterial pressure (MAP) increased 21.0 +/- 4.0 mm Hg and HR 51.0 +/- 5.0 beats/min in control rats. Rats made hypertensive by 1% NaCl intake showed a significantly lower increase of MAP, 11.0 +/- 1.8 mm Hg; HR increased 37.0 +/- 4.3 beats/min. Rats submitted only to psychosocial stress displayed a response similar to the one described in control rats. Hypertensive rats submitted to both 1% NaCl intake and psychosocial stress had a more intense reduction of the hypertensive and tachycardic response, 8.0 +/- 2.2 mm Hg and 20.0 +/- 3.2 beats/min respectively. Control i.c. injection with the same volume of saline (0.15 M NaCl) did not change significantly SBP, DBP or HR in a separate group of rats. Left ventricle weight (0.754 +/- 0.0333 g) was augmented in the 1% NaCl treated group (0.795 +/- 0.038 g), and increased its protein content by 13.1% (changes not statistically significant). The highest increase of the left ventricle weight (23.7% above control) with no change in its protein content was observed in rats submitted to 1% NaCl intake plus psychosocial stress. In conclusion, chronic high NaCl intake increased blood pressure; psychosocial stress acted as a weak stimulus for SBP and DBP increase, and central nervous system sodium chloride sensitivity for delivering a peripheral sympathetic discharge was found decreased in rats made hypertensive by a high salt intake.

Animals↗