Ricardo Reisin

Immune reconstitution inflammatory syndrome associated with PML in AIDS: A treatable disorder

An HIV-1-infected patient with progressive multifocal leukoencephalopathy presented clinical deterioration and contrast-enhancing lesions on brain nuclear MR after the initiation of highly active antiretroviral therapy (HAART). Brain biopsy identified an inflammatory reaction compatible with immune reconstitution inflammatory syndrome. Treatment with corticosteroids and transient suppression of HAART led to marked neurologic improvement.

Nonconvulsive Status Epilepticus in the Elderly: A Case–Control Study

Summary:  Background: Nonconvulsive status epilepticus (NCSE) is a usually underdiagnosed and potentially treatable cause of altered awareness in the elderly. To assess etiologies, associations with other medical problems, and prognosis of NCSE in a population aged >75 years we performed a nested case–control study.

Two-dimensional speckle tracking echocardiography for early detection of myocardial damage in young patients with Fabry disease.

Fabry disease (FD) is characterized by left ventricular hypertrophy (LVH). Conventional echocardiography is not sensitive enough to perform the preclinical diagnosis To assess whether longitudinal myocardial strain of the left ventricle (LV), using speckle tracking, is useful to detect early myocardial involvement in FD. Forty‐four patients with FD who were diagnosed with genetic testing were prospectively included and were compared to a sex‐matched control group. They were divided into three groups: 22 with LVH (Group I), 22 without LVH (Group II), and 22 healthy volunteers (Group III). LV longitudinal strain was measured from the apical views. An ANOVA test was used for multiple comparisons for variables with a normal distribution, and a Kruskal–Wallis test was used for variables with non‐Gaussian distribution. Longitudinal LV strain was different in the three groups: it was ≥−15% in at least one segment in all Group I patients, in 50% of patients of Group II and in no patient of Group III. Seventy percent of the segments with abnormal strain in Group II were located in the basal regions (32/46). These findings show that the presence of at least one strain value ≥−15% demonstrates subclinical myocardial dysfunction in patients with preclinical FD. Longitudinal myocardial LV strain measured with speckle tracking is a useful tool to detect early myocardial involvement in young patients with FD. This information allows the detection and treatment of myocardial dysfunction at an early stage, which is of high clinical importance.

Central retinal artery occlusion and ophthalmoplegia following spinal surgery

Visual loss and ophthalmoplegia are very infrequent complications after spinal surgery.1,2 Visual loss may be caused by ischaemic optic neuropathy, central retinal artery or vein occlusion, or occipital stroke.2,3 Previous reports have attributed this complication to patient positioning, intraoperative blood loss, and controlled hypotension or shock.2–5 Associated risk factors include anaemia, prolonged surgical time, bradyarrhythmia, hypertension, diabetes, smoking, vascular disease, and increased blood viscosity.2,6 Ophthalmoplegia after spinal surgery is even more unusual than visual loss, and only few reports exist in the literature.6–8 Moreover, magnetic resonance image (MRI) studies to differentiate between cavernous sinus thrombosis and direct compression of orbital contents have not been previously described. We therefore report two patients who developed this unusual combination of ophthalmoplegia and central retinal artery occlusion (CRAO) after spinal surgery. A 62 year old male ex-smoker underwent a L2–L3 posterior spinal decompression and segmental instrumentation for lumbar stenosis and scoliosis, in prone position with ocular protection (gauze swab and tape). The surgery lasted 2 hours and 45 minutes. Before the procedure blood pressure was 140/60 mm Hg and during operation it was maintained at 90/60 mm Hg. Just after surgery he complained of visual loss and left ocular and nasal pain. Examination revealed left palpebral oedema, local erythema, blindness, and total ophthalmoplegia of the left eye. Left pupil was dilated and …

Different pain patterns in patients with vertebral artery dissections

Spontaneous or traumatic cervical artery dissections are an increasingly recognized cause of stroke in young adults.1 The internal carotid artery (ICA) is more frequently involved than the vertebral artery (VA).1 Headache and neck pain are common and suggestive symptoms of VA dissection (VAD).2 However, their clinical characteristics and their relationship with focal neurologic symptoms have not been extensively analyzed. We prospectively analyzed 10 consecutive patients admitted to our hospital with diagnosis of VAD from 1995 to 2003. In all cases, the dissection was confirmed by magnetic resonance angiography (MRA), conventional angiography, or both. The presence of headache, neck pain, or both at the time of the dissection, their characteristics, and their association with neurologic signs and symptoms were registered by direct interview. The history of headache was classified according to the International Headache Society criteria.3 Headache at the time of arterial dissection was classified into that occurring before, during …

Role of EMSE and STESS scores in the outcome evaluation of status epilepticus

Status epilepticus (SE) is a severe neurological condition with significant morbidity and mortality. A reliable tool for prognosis is needed to take decision regarding treatment strategies. We compared 2 available prognostic scores of outcome: the Status Epilepticus Severity Score (STESS) and the Epidemiology-based Mortality score in SE (EMSE). We included 46 patients with SE evaluated out the last 5years in our hospital. We excluded patients with postanoxic encephalopathy or incomplete data. Among the 46 patients with SE, in-hospital mortality was 28%. The receiver operating characteristic (ROC) curve for predicting of death by STESS had an area under the curve (AUC) of 0.80 with cutoff point ≥4. The best EMSE variable combination to predict mortality was EMSE-AEL using an optimized cutoff point of 34 (age/etiology/loss of consciousness) with an area under the ROC of 0.79. The STESS and EMSE would be useful tools to predict in-hospital mortality in SE.

Neuro-otological and peripheral nerve involvement in Fabry disease

Fabry disease (FD) is an X-linked lysosomal storage disease, with multisystemic glycosphingolipids deposits. Neuro-otological involvement leading to hearing loss and vestibular dysfunctions has been described, but there is limited information about the frequency, site of lesion, or the relationship with peripheral neuropathy. The aim was to evaluate the presence of auditory and vestibular symptoms, and assess neurophysiological involvement of the VIII cranial nerve, correlating these findings with clinical and neurophysiological features of peripheral neuropathy. We studied 36 patients with FD with a complete neurological and neuro-otological evaluation including nerve conduction studies, quantitative sensory testing (to evaluate small fiber by warm and cold threshold detection and cold and heat pain), vestibular evoked myogenic potentials, videonistagmography, audiometry and brainstem auditory evoked potentials. Neuro-otologic symptoms included hearing loss (22.2%), vertigo (27.8%) or both (25%). An involvement of either cochlear or vestibular function was identified in most patients (75%). In 70% of our patients the involvement of both cochlear and vestibular function could not be explained by a neural or vascular mechanism. Small fiber neuropathy was identified in 77.7%. There were no significant associations between neuro-otological and QST abnormalities. Neuro-otologic involvement is frequent and most likely under-recognized in patients with FD. It lacks a specific neural or vascular pattern, suggesting multi-systemic, end organ damage. Small fiber neuropathy is an earlier manifestation of FD, but there is no correlation between the development of neuropathy and neuro-otological abnormalities.

Brain MRI findings in children and adolescents with Fabry disease

OBJECTIVE To evaluate the presence of white matter and hemorrhagic lesions in brain MRI of children and adolescents with Fabry disease (FD). METHODS Brain MRI studies in 44 consecutive children and teenagers (20 boys, mean age 14.6 years, range 7-21 years) were evaluated using classic sequences as well as, GRE-weighted images, for white matter lesions (WML) and chronic microbleed detection. All patients lacked history of stroke or TIA. Brain MRI findings in 46 consecutive children and adolescents without FD, referred for the evaluation of headaches (36 females, mean age 14.1 years, range 7-21 years) were evaluated as a control group. Additionally, we assessed the clinical manifestations of FD. RESULTS Seven children (15.9%) with FD had brain MRI evidence of asymptomatic WML (5 girls, mean age 14.8 years, range: 13-20 years) compared with 3 children (6.5%) in the control group (p = 0.01). Brain abnormalities in patients with FD revealed WML, deep gray matter and infratentorial involvement. Three patients presented two lesions each. None of the children showed microbleeds. Regarding clinical manifestations, 90.9% of the patients had signs or symptoms of FD. CONCLUSION We identified asymptomatic white matter brain lesions in 15.9% of children with FD without clinical history of stroke. FD is a treatable disorder that should be routinely included in the differential diagnosis of both symptomatic and asymptomatic brain lesions in children and adolescents. The detection of brain lesions may foster earlier treatment.

BLOOD PRESSURE EVOLUTION IN YOUNG PATIENTS WITH ACUTE ISCHEMIC STROKE: A new model for understanding the natural course of spontaneous hypertension?

ABSTRACT Background: Approximately 80% of patients suffering an acute ischemic stroke develop transient hypertension. The physiopathological mechanism remains unclear. Due to the lack of vascular risk factors, young adults could be a useful model for understanding blood pressure (BP) evolution in this setting. Methods: Patients between 18 and 55 years old admitted with an acute ischemic stroke were included. BP was evaluated during the following 48 h. Hypertension was defined as: systolic blood pressure (SBP) ≥140 mmHg or diastolic blood pressure (DBP) ≥90 mmHg. Patients were divided into two groups: RF group included those who had a previous vascular risk factor and/or other medical condition known to affect the autonomic function; noRF group included patients without any of the previously stated conditions. Results: Thirty-nine patients were included: mean age: 44.26 years old, 21 were males (53.8%). Mean SBP and DBP at admission were 139.77 ± 5.35 mmHg (range: 90–243) and 84.44 ± 3.02 mmHg (range: 60–128), respectively; 43.58% patients were hypertensive at admission. SBP decreased significantly during the first 48 h (p = 0.044) for the entire population. RF group has higher SBP (p = 0.009) and DBP (p = 0.011) at admission than the noRF group. Conclusion: Most patients were normotensive at admission and BP fell spontaneously despite BP at admission. Young patients without medical conditions that could alter the autonomic system function could be a useful model for understanding acute hypertension in ischemic stroke.

Multiple cranial nerve palsies due to perineural invasion

A 69-year-old man presented with a recent history of painless diplopia and left eye conjunctival injection. He had a cutaneous adenosquamous carcinoma removed from the left forehead 3 years before. The examination showed complete left ophthalmoplegia, exophthalmos, ptosis, and ipsilateral V1 hypoesthesia. Brain MRI revealed thickening and contrast enhancement of the external wall of the left cavernous sinus (figure 1). Because of skin cancer history, biopsy specimens of the previously resected tumor were reassessed, exhibiting neoplastic perineural spread (figure 2). Cavernous sinus invasion due to centripetal perineural spread from a supposedly removed skin cancer has been previously reported.1,2