Dynamic CT features of hepatic abscesses

Forty hepatic abscesses were examined with dynamic computed tomography (CT). A "double target sign," consisting of a hypodense central area surrounded by first a hyperdense ring and then a hypodense zone, seems to be highly suggestive of abscess formation. In 12 cases, the hepatic parenchyma surrounding the lesion demonstrated transient hyperdensity after contrast injection, possibly due to localized hepatic venous obstruction secondary to acute hepatic inflammation. This is similar to the appearance of an arterioportal fistula.     

The efficacy of the ketogenic diet-1998: a prospective evaluation of intervention in 150 children

OBJECTIVE: The ketogenic diet is a high-fat, low-protein, low-carbohydrate diet developed in the 1920s for the treatment of children with difficult to control seizures. Despite advances in both the pharmacotherapy and the surgery of epilepsy, many children continue to have difficult-to-control seizures. This prospective study sought to determine the ketogenic diet's effectiveness and tolerability in children refractory to today's medications. METHODS: One hundred fifty consecutive children, ages 1 to 16 years, virtually all of whom continued to have more than two seizures per week despite adequate therapy with at least two anticonvulsant medications, were prospectively enrolled in this study, treated with the ketogenic diet, and followed for a minimum of 1 year. Seizure frequency was tabulated from patients' daily seizure calendars and seizure reduction calculated as percentage of baseline frequency. Adverse events and reasons for diet discontinuation were recorded. RESULTS: The children (mean age, 5.3 years), averaged 410 seizures per month before the diet, despite an exposure to a mean of 6.2 antiepileptic medications. Three months after diet initiation, 83% of those starting remained on the diet and 34% had >90% decrease in seizures. At 6 months, 71% still remained on the diet and 32% had a >90% decrease in seizures. At 1 year, 55% remained on the diet and 27% had a >90% decrease in seizure frequency. Most of those discontinuing the diet did so because it was either insufficiently effective or too restrictive. Seven percent stopped because of intercurrent illness. CONCLUSIONS: The ketogenic diet should be considered as alternative therapy for children with difficult-to-control seizures. It is more effective than many of the new anticonvulsant medications and is well tolerated by children and families when it is effective.     

Incidental heterotopic pregnancy demonstrated on magnetic resonance imaging

We report a case of an assisted pregnancy in an asymptomatic woman who was found to have an extrauterine mass on ultrasound and MRI. This complex mass had equivocal imaging features and was found to be a ruptured ovarian ectopic pregnancy at surgery. This case illustrates that vigilance is required regarding the possibility of coexisting ectopic and intrauterine pregnancy following assisted conception, even in entirely asymptomatic cases.     

The screen for cognitive impairment in psychiatry (SCIP) is associated with disease severity and cognitive complaints in major depression

Objective: To assess the relationship between the Screen for Cognitive Impairment in Psychiatry (SCIP) score and illness severity, subjective cognition and functioning in a cohort of major depressive disorder (MDD) patients.

Methods: Patients (n?=?40) diagnosed with MDD (DSM-IV-TR) completed the SCIP, a brief neuropsychological test, and a battery of self-administered questionnaires evaluating functioning (GAF, SDS, WHODAS 2.0, EDEC, PDQ-D5). Disease severity was evaluated with the Hamilton Depression Rating Scale (HDRS) and the Clinical Global Impression (CGI).

Results: Age and sex were associated with performance in the SCIP. The SCIP-Global index score was associated with disease severity (r?=??0.316, p?<?.05), the SDS, a patient self-assessment of daily functioning (r?=??0.368, p?<?.05), and the EDEC subscales of patient-reported cognitive deficits (r?=??0.388, p?<?.05) and their functional impacts (r?=??0.335, p?<?.05). Multivariate analysis adjusted for age and sex confirmed these tests are independent predictors of performance in the SCIP (CGI-S, F_[3,34]?=?4.478, p?=?.009; SDS, F_[3,34]?=?3.365, p?=?.030; EDEC-perceived cognitive deficits, F_[3,34]?=?5.216, p?=?.005; EDEC-perceived impacts of functional impairment, F_[3,34]?=?5.154, p?=?.005).

Conclusions: This study confirms that the SCIP can be used during routine clinical evaluation of MDD, and that cognitive deficits objectively assessed in the SCIP are associated with disease severity and self-reported cognitive dysfunction and impairment in daily life.     

Marrow transplantation with or without donor buffy coat cells for 65 transfused aplastic anemia patients

Sixty-five multiply transfused patients with severe aplastic anemia were given cyclophosphamide followed by grafts anemia were given cyclophosphamide followed by grafts from HLA-identical siblings. The effect of the administration of viable donor buffy coat cells following the marrow inoculum was evaluated with regard to graft rejection and survival. Results in 43 patients so treated are presented along with those in 22 concurrent patients given marrow alone. Most patients given buffy coat had positive in vitro tests of sensitization indicating a high risk for graft rejection, while all but one of the patients given marrow alone had negative tests. Thirty of the 43 (70%) patients given marrow and buffy coat are alive between 10 and 61 mo (median 36) after grafting; 4 died after graft rejection and 6 with acute or chronic graft-versus-host disease (GVHD). Eleven of the 22 (50%) patients given marrow alone are alive between 29 and 65 mo (median 52); 7 died after graft rejection and 3 with GVHD. The addition of buffy coat cell infusions to the marrow inoculum reduced the risk of rejection and increased survival in the currently reported transfused patients when compared to patients grafted before 1976. However, there was an increased risk of chronic GVHD. Recipients of marrow from female donors survived slightly better (73%) than recipients of male marrow (58%).     

Exploring the utility of whole‐exome sequencing as a diagnostic tool in a child with atypical episodic muscle weakness

The advent of whole‐exome next‐generation sequencing (WES) has been pivotal for the molecular characterization of Mendelian disease; however, the clinical applicability of WES has remained relatively unexplored. We describe our exploration of WES as a diagnostic tool in a 3½‐year old female patient with a 2‐year history of episodic muscle weakness and paroxysmal dystonia who presented following a previous extensive but unrevealing diagnostic work‐up. WES was performed on the proband and her two parents. Parental exome data was used to filter potential de novo genomic events in the proband and suspected variants were confirmed using di‐deoxy sequencing. WES revealed a de novo non‐synonymous mutation in exon 21 of the calcium channel gene CACNA1S that has been previously reported in a single patient as a rare cause of atypical hypokalemic periodic paralysis. This was unexpected, as the proband's original differential diagnosis had included hypokalemic periodic paralysis, but clinical and laboratory features were equivocal, and standard clinical molecular testing for hypokalemic periodic paralysis and related disorders was negative. This report highlights the potential diagnostic utility of WES in clinical practice, with implications for the approach to similar diagnostic dilemmas in the future.     

A neurophysiological-metabolic model for burst suppression

Burst suppression is an electroencepholagram (EEG) pattern in which high-voltage activity alternates with isoelectric quiescence. It is characteristic of an inactivated brain and is commonly observed at deep levels of general anesthesia, hypothermia, and in pathological conditions such as coma and early infantile encephalopathy. We propose a unifying mechanism for burst suppression that accounts for all of these conditions. By constructing a biophysical computational model, we show how the prevailing features of burst suppression may arise through the interaction between neuronal dynamics and brain metabolism. In each condition, the model suggests that a decrease in cerebral metabolic rate, coupled with the stabilizing properties of ATP-gated potassium channels, leads to the characteristic epochs of suppression. Consequently, the model makes a number of specific predictions of experimental and clinical relevance.