Risk factors for the development of secondary intracranial hypertension in acute cerebral venous thrombosis.

PURPOSE: Intracranial hypertension (IH) can complicate cerebral venous thrombosis (CVT), potentially causing permanent visual loss. Current knowledge on risk factors for the development of IH following CVT is scarce. We applied a compound classifier (CSF opening pressure > 25 cmH2O, papilledema, or optic disc protrusion on MRI) as a surrogate for IH and studied the predictive value of thrombus location, the number of thrombosed segments, and thrombus volume. METHODS: We prospectively included 26 patients with acute CVT and complete MRI data. IH was defined by CSF opening pressure > 25 cmH2O, papilledema, or optic disc protrusion on MRI. Using high-resolution contrast-enhanced venography, we determined the thrombus location, number of thrombosed segments, and thrombus volume. We analyzed their association with IH by logistic regression, their predictive power by the area under the receiver operating characteristic curve, and their association with CSF opening pressure by linear regression. RESULTS: IH occurred in 46% of CVT patients and was associated with higher thrombus volume (AUC 0.759, p = 0.025) and superior sagittal sinus thrombosis both alone (OR 2.086, p = 0.049) and combined with transverse sinus thrombosis (OR 2.014, p = 0.028). Effects in patients presenting CSF opening pressure > 25 cm H2O and the compound classifier were consistent. Thrombus volume > 4 ml was the single most important predictor of higher CSF opening pressure (ß = 0.566, p = 0.035), increasing IH risk. CONCLUSION: Larger thrombus volume, dominant transverse sinus occlusion, and extensive superior sagittal combined with transverse sinus thrombosis were associated with IH. Thrombus volumetry might identify patients at risk for IH and direct further clinical evaluation.

Type 2a fibre rhabdomyolysis in myoadenylate deaminase deficiency.

A 31-year-old woman developed an acute, potentially fatal rhabdomyolysis of undetermined origin. Muscle biopsy revealed selective lysis involving exclusively type 2a fibers. Myoadenylate-deaminase (MAD) deficiency was proven by a negative histochemical reaction as well as by an enzymatic biochemical determination. The significantly greater energetic dependence of type 2a fibres on MAD explains their selective damage. The patient's mother also suffers from a similar muscle disease of still unclarified origin.

Morphometric study of muscle in congenital idiopathic club foot.

In congenital idiopathic club foot gross morphological changes cannot be assessed in muscle tissue by conventional histopathological techniques. Since, however, recent studies have indicated the presence of neuromuscular anomalies with preponderance of Type 1 fibres in this condition, we have performed histochemical, morphometric and electron microscopic examinations in muscle biopsies of 23 patients with congenital idiopathic club foot deformity. The age of the patients varied between 6 weeks and 12 years, respectively. Muscle biopsy was taken mainly from the flexor group of the affected leg(s) during the surgical correction of the anomaly. No gross pathological changes could be found by histochemical analysis. Morphometric study, however, disclosed abnormalities in the composition of the fibre types. The most prominent change was the percentual increase in Type 1 and decrease in Type 2 fibres in almost all the cases. Electron microscopically, only minor fine structural changes could be found. Since these changes could be assessed soon after birth just as in the later course, they cannot be regarded as the consequence of the abnormal position of the leg. More probably, Type 1 fibre predominance is related etiologically to the congenital club foot deformity.