Primary Spinal Epidural Abscesses Not Associated With Pyogenic Infectious Spondylodiscitis: A New Pathogenetic Hypothesis.

Introduction: Spinal epidural abscess (SEA) incidence is rising. However, most series do not differentiate between SEAs associated with pyogenic infectious spondylodiscitis (PS) and SEAs limited to the epidural space. Methods: We retrospectively reviewed the records and radiological images of all patients admitted to our institutions with a diagnosis of SEA not associated with PS between January 2013 and December 2018. Results: We found three males and four females; five of the seven were intravenous drug users. All patients presented with pain: in six, it was associated with acute motor and sensory deficits, while one had only pain and paresthesias. Staphylococcus aureus was cultured from abscesses and/or from multiple blood cultures in four patients. Abscesses were localized to the cervical spine in one patient, thoracic in three, lumbar in one, and in two, the SEAs involved multiple segments. All patients but one underwent urgent open surgery. This patient had a multisegmental abscess and was successfully treated by percutaneous aspiration when pain became intractable. After abscess evacuation, the neurological deficits improved in all patients except one. The patients that were treated without spine instrumentation did not develop delayed kyphosis or instability at follow-up. Conclusion: Patients with SEAs not associated with PS are likely to present with pain and motor deficits, appear to benefit from urgent abscess evacuation, and seem to be less dependent on spine instrumentation to avoid delayed spinal deformities compared to SEA associated with PS. Finally, the lack of initial involvement of bone and intervertebral disks may suggest that at least some of the SEAs without PS originate from infection of epidural lymphatic vessels that are not present inside those structures.

Minimally symptomatic posterior reversible encephalopathy in Guillain-Barré syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a rare occurrence in patients with Guillain-Barré syndrome (GBS). Two patients with neuroradiological evidence of PRES without central nervous system (CNS) symptoms were recently reported. We present the case of a GBS patient with minimal CNS symptoms and magnetic resonance imaging findings consistent with PRES. PRES in GBS might be an underestimated condition. It should be suspected in GBS patients in the presence of even mild CNS symptoms. A timely PRES diagnosis along with early correction of autonomic system dysfunction in GBS patients is recommended to prevent possible dangerous CNS complications.

Reversible cerebral vasoconstriction syndrome possibly induced by etoricoxib.

Etoricoxib is a newer cyclooxygenase (COX)-2 inhibitor anti-inflammatory drug with a favorable safety profile. However, several randomized trials have provided evidence of an increased risk for acute myocardial infarction associated with the use of COX-2 inhibitors. Fewer data are available concerning the risk for ischemic stroke associated with COX-2 inhibitors. Although sporadic classes of drug-induced reversible cerebral vasoconstriction syndrome (RCVS) have been reported, this was not the case for etoricoxib. We report a patient who developed thunderclap headache, reversible cerebral arterial vasoconstriction, high blood pressure, and ischemic stroke (ie, RCVS) with recent exposure to etoricoxib. Although the association is hypothetical, the authors suggest consideration of RCVS in hypertensive patients presenting with headache, focal deficits, and evidence of cerebral ischemia during COX-2 inhibitors use.

Brain white matter impairment in congenital adrenal hyperplasia.

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an inherited recessive disorder of adrenal steroidogenesis. Past reports suggested that brain white matter could be involved in CAH. OBJECTIVE: To detect the presence, and possible changes over time, of brain white matter abnormalities in patients with CAH. DESIGN: Neurological examination and brain magnetic resonance imaging (MRI) that were repeated in 12 patients after a mean interval of 11 years. SETTING: Pavia, northern Italy. Patients Twenty-two patients with CAH. MAIN OUTCOME MEASURES: Evaluation of clinical neurological findings and brain MRI T2-weighted images. RESULTS: Ten (45%) of 22 patients with CAH had white matter abnormalities (diffuse in 4 cases, focal in 3 cases, and both diffuse and focal in 3 cases) on MRI. The MRI findings never changed over repeated assessments. CONCLUSIONS: Subclinical brain white matter involvement is frequent in CAH. This might be due to hormonal imbalance during brain development or corticosteroid treatments. Our study findings indicate that a relationship with demyelinating diseases can also be suggested. Diagnosis of CAH should be suspected in young subjects with brain MRI white matter abnormalities that are not otherwise explicable.

Long-term follow-up of an adolescent who had bilateral striatal necrosis secondary to Mycoplasma pneumoniae infection.

We describe the long-term follow-up of a patient with bilateral striatal necrosis associated with Mycoplasma pneumoniae infection occurred in adolescence. In the literature there are no longitudinal studies of such a rare condition. Our patient, 4 years after the onset of an acute and reversible akinetic-rigid syndrome, showed a severe obsessive-compulsive disorder, cognitive decline, and a neuropsychological profile characterized by signs of deficient executive functioning. The clinical picture that emerged in our patient is suggestive of a frontosubcortical dementia which might be considered a major long-term sequela of the bilateral selective striatal necrosis and consequent dysfunction of frontostriatal connections.

Muscle MRI in adult-onset acid maltase deficiency.

We report the spectrum of muscle involvement on magnetic resonance imaging in 11 patients with a molecularly confirmed diagnosis of adult-onset acid maltase deficiency at different clinical stages. Muscle magnetic resonance imaging showed a selective progressive pattern of muscle involvement with a constant involvement of the adductor magnus and semimembranosus at the early stage of the disease and a later fatty infiltration of the long head of the biceps femoris, semitendinosus and of the anterior thigh muscles. In the advanced phases a selective sparing of sartorius, rectus, and gracilis muscles and peripheral portions of the vastus lateralis was also evident. Muscle strength and magnetic resonance imaging findings were positively correlated. The results suggest that muscle magnetic resonance imaging may provide valuable diagnostic guidance for the assessment of accurate selective muscular involvement in acid maltase deficiency and may help monitor the progression of the disorder. Further control studies in a larger cohort are needed to evaluate the specificity of these findings.

Quantitative MR evaluation of body composition in patients with Duchenne muscular dystrophy.

The aim of this study was to propose a quantitative MR protocol with very short acquisition time and good reliability in volume construction, for the evaluation of body composition in patients affected by Duchenne muscular dystrophy (DMD). This MR protocol was compared with common anthropometric evaluations of the same patients. Nine boys affected by DMD, ranging in age from 6 to 12 years, were selected to undergo MR examination. Transversal T1-weighted spin-echo sequences (0.5T; TR 300 ms, TE 10 ms, slice thickness 10 mm, slice gap 1 mm) were used for all acquisitions, each consisting of 8 slices and lasting just 54 s. Whole-body examination needed an average of nine acquisitions. Afterwards, images were downloaded to an independent workstation and, through their electronic segmentation with a reference filter, total volume and adipose tissue volumes were calculated manually. This process took up to 2 h for each patient. The MR data were compared with anthropometric evaluations. Affected children have a marked increase in adipose tissue and a decrease in lean tissue compared with reference healthy controls. Mean fat mass calculated by MR is significantly higher than mean fat mass obtained using anthropometric measurements ( p<0.001). Our MR study proved to be accurate and easy to apply, although it was time-consuming. We recommend it in monitoring the progression of the disease and planning DMD patients' diet.