Wazim Mohamed

Isolated inctracranial Whipple's disease-report of a rare case and review of the literature

INTRODUCTION Whipples disease (WD) is a rare multisystemic infectious disease that can involve a variety of organs namely the gastrointestinal tract, lymphatic system, heart and nervous system. Myorhythmia is a hallmark of WD. Isolated CNS involvement is very rare. CASE We present a 50 year-old African-American woman with rapid cognitive decline, visual hallucinations, insomnia, dysarthria, and gait unsteadiness. She subsequently developed pendular nystagmus and gaze paresis. Serial brain MRI scans showed T2 hyperintense lesions in the left striatum and right parahippocampal gyrus. FDG-PET scan showed marked increase of glucose uptake in the left putamen. Serum and CSF PCR for Tropheryma whipplei was negative. Stereotactic biopsy of the lesion and tissue PCR was consistent with WD. REVIEW OF LITERATURE A systematic review identified 24 cases of isolated intracranial presentation of WD since 1975. Cases with systemic and extracranial manifestations were excluded. DISCUSSION In patients with rapidly progressive cognitive decline with negative workup for common etiologies, there should be a high index of suspicion for WD. Diagnosis of WD remains a challenge as traditional methods commonly fail to culture T. whipplei. PET scans can help in identifying areas of inflammation that can be biopsied. Our case proves that a negative serum and CSF PCR should not exclude CNS WD and a brain biopsy of the lesion with PCR assay should be performed when possible.

Clobazam: An effective add‐on therapy in refractory status epilepticus

Refractory status epilepticus (RSE) is a medical emergency, with significant morbidity and mortality. The use and effectiveness of clobazam, a unique 1,5‐benzodiazepine, in the management of RSE has not been reported before. Over the last 24 months, we identified 17 patients with RSE who were treated with clobazam in our hospital. Eleven of the 17 patients had prior epilepsy. Fifteen patients had focal status epilepticus. Use of clobazam was prompted by a favorable pharmacokinetic profile devoid of drug interactions. Clobazam was introduced after a median duration of 4 days and after a median of three failed antiepileptic drugs. A successful response, defined as termination of RSE within 24 h of administration, without addition or modification of concurrent AED and with successful wean of anesthetic infusions, was seen in 13 patients. Indeterminate response was seen in three patients, whereas clobazam was unsuccessful in one patient. Clobazam averted the need for anesthetic infusions in five patients. Clobazam was well tolerated, and appears to be an effective and promising option as add‐on therapy in RSE. Its efficacy, particularly early in the course of SE, should be further investigated in prospective, randomized trials.

Early Access to a Neurologist Reduces the Rate of Missed Diagnosis in Young Strokes

BACKGROUND We hypothesized that the presence of an in-house neurologist or a neurology or emergency medicine (EM) residency is associated with a lower rate of missed stroke diagnosis and a greater use of thrombolytic therapy. METHODS The outpatient Young Stroke registry from our academic medical center was reviewed. Patients 16 to 50 years of age who presented with ischemic stroke were included. Information on presentation, acute therapy, and missed diagnosis was obtained. The presence of an EM or neurology residency at the presenting hospital was recorded. We also assessed whether hospital teaching status in these fields affected missed diagnosis rates, the use of thrombolysis, or stroke intervention. RESULTS Ninety-three patients were included. Thirteen patients were misdiagnosed. In hospitals with and without a neurology residency, the missed diagnosis rate was 6.3% versus 18.0%, respectively (P=.21). Two patients were misdiagnosed in hospitals with a neurology residency, but neither had neurology consultations in the emergency department. If these cases are removed from our analysis, the rate of missed diagnosis with and without a neurology residency is 0% versus 20.6%, respectively (P=.008). Acute stroke therapy was administered in 17.9% of patients seen with an EM residency, compared to 2.7% without an EM residency (P=.046). With and without a neurology residency, acute stroke therapy was administered in 25% versus 8.2% of cases, respectively (P=.055). CONCLUSIONS Young adults with ischemic stroke seen at hospitals with a neurology residency had a lower missed diagnosis rate. The presence of an EM resident or a neurology teaching program was associated with a greater use of acute stroke therapies. These results support initiatives to triage young adults with suspected acute stroke to hospitals with access to neurologic expertise in the emergency department.

Which Comorbidities and Complications Predict Ischemic Stroke Recovery and Length of Stay

Objectives:Stroke is the second most common cause of death worldwide and can lead to significant disability and long-term costs. Length of stay (LOS) is the most predictive factor in determining inpatient costs. In the present study, factors that affect disability and LOS among ischemic stroke patients admitted to an urban community hospital and 2 university-based teaching hospitals were assessed. Methods:Data for consecutive patients with acute ischemic strokes were collected, by reviewing discharge diagnosis International Classification of Diseases codes. A data mining process was used to analyze admission data. Data regarding comorbidities and complications were abstracted by mining the secondary diagnoses for their respective International Classification of Diseases-9 codes. The primary outcome was LOS, calculated from the dates of admission and dates of discharge. The second outcome of interest was disability, which was evaluated by the modified Rankin score at the time of discharge. Results:LOS progressively increased with greater disability. Greater age and higher National Institute of Health Stroke Scale at admission were associated with both higher disability and longer LOS. Presence of congestive heart failure or chronic kidney disease, atrial fibrillation, other arrhythmias (preexisting or new onset), and development of acute renal failure were associated with greater LOS but not greater disability status. Patients with a previous stroke and those that developed urinary tract infection as a complication had higher disability. Conclusions:Greater age and higher National Institute of Health Stroke Scale at admission were associated with both higher disability and longer LOS. Congestive heart failure, CRF, presence of arrhythmias, and development of acute renal failure were associated with greater LOS. The development of urinary tract infection caused higher disability.

Teaching NeuroImages: “Subarachnoid hemorrhage” from decreased contrast elimination after therapeutic hypothermia

A 50-year-old woman underwent therapeutic hypothermia after a witnessed cardiac arrest and cardiac catheterization. A 24-hour head CT (figure 1) showed diffuse hyperattenuation in the subarachnoid space and was reported as subarachnoid hemorrhage. Repeat CT at 48 hours (figure 2) showed significant clearing of the “subarachnoid hemorrhage.”

Fulminant Reversible Cerebral Vasoconstriction Syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is increasingly being recognized as a diagnosis in patients presenting with thunderclap headaches. In the vast majority of the cases, the syndrome follows a benign course and patients recover completely without any significant therapeutic intervention. In the rarest of cases, RCVS follows a monophasic course with rapid worsening, severe neurological deficits, and poor outcomes. We present the case of a 25-year-old female who presented with headaches which were worsening over 1 week. She was admitted to the hospital and rapidly worsened to develop severe neurological deficits over the next day. Initial computed tomography scan showed areas of hemorrhage and multiple ischemic strokes. Computed tomography angiogram and a conventional cerebral angiogram both revealed multifocal vasoconstriction, highly suggestive of RCVS. Despite aggressive medical and surgical management, the patient continued to worsen and eventually died. Autopsy findings did not show evidence of vasculopathy or any other underlying disorder, further supporting the diagnosis of RCVS. The RCVS is usually a benign self-remitting condition which commonly affects young females and presents with an insidious onset of headaches. Rarely, it can have a fulminant course with devastating outcomes. This case illustrates an exceptionally uncommon clinical course of RCVS and the challenges in its treatment.

Late onset neuromyelitis optica mimicking an acute stroke in an elderly patient

Neuromyelitis optica spectrum disorder (NMOSD) was recently proposed as a unifying term for Neuromyelitis optica (NMO) and related syndromes to incorporate patients with NMO antibody but without the full clinical spectrum. NMO is a rare, demyelinating condition which predominantly affects females with a peak incidence in the third and fourth decade of life. We report a case of NMOSD in an elderly patient with extremely late onset (>80years) of disease. The patient presented with findings of sudden onset unilateral symptoms, which is extremely unusual for NMOSD. She had frequent relapses which were treated with high dose steroids and plasmapheresis but has not had any relapse since being started on immunosuppressive therapy and continues to do well. Our case also highlights the varied clinical presentations of NMOSD and we believe that the diagnosis of NMOSD should be considered in elderly patients in the appropriate clinical setting despite the presence of unconventional symptoms.

Alpha 3 ganglionic acetylcholine receptor antibody associated refractory status epilepticus

Autoimmune encephalitides are a group of syndromes that may present with subacute onset of disorientation, personality changes, memory loss and most prominently seizures. Earlier reports of this entity were from patients with paraneoplastic limbic encephalitis, however autoimmune etiologies have also been described in patients with non-paraneoplastic encephalitis [1]. The Alpha 3 ganglionic acetylcholine receptor autoantibody (a3-AChR Ab) mainly causes autoimmune dysautonomia that is either subacute or insidious in onset. A direct relationship betweenantibody titer and severity of dysautonomia occurs in both experimental animals and patients [2]. Most of these cases have been paraneoplastic (mainly adenocarcinomas) however a few cases have been linked to other autoimmune disorders such as systemic lupus erythematosus (SLE) and Sjogren’s syndrome. Patients who are seropositive for a3-AChR Ab may present with less common neurological manifestations such as peripheral neuropathies, encephalopathy and subacute neuropsychiatric presentations. This report presents a unique case of refractory new-onset status epilepticus associated with a3-AChR antibody which responded to immunotherapy.

Efficacy of Cilostazol in Prevention of Delayed Cerebral Ischemia after Aneurysmal Subarachnoid Hemorrhage: A Meta-Analysis

OBJECTIVES Cilostazol, a selective inhibitor of phosphodiesterase 3, may reduce symptomatic vasospasm and improve outcome in patients with aneurysmal subarachnoid hemorrhage considering its anti-platelet and vasodilatory effects. We aimed to analyze the effects of cilostazol on symptomatic vasospasm and clinical outcome among patients with aneurysmal subarachnoid hemorrhage (aSAH). PATIENTS AND METHODS We searched PubMed and Embase databases to identify 1) prospective randomized trials, and 2) retrospective trials, between May 2009 and May 2017, that investigated the effect of cilostazol in patients with aneurysmal aSAH. All patients were enrolled after repair of a ruptured aneurysm by clipping or endovascular coiling within 72hours of aSAH. fixed-effect models were used to pool data. We used the I2 statistic to measure heterogeneity between trials. RESULTS Five studies were included in our meta-analysis, comprised of 543 patients with aSAH (cilostazol [n=271]; placebo [n=272], mean age, 61.5years [SD, 13.1]; women, 64.0%). Overall, cilostazol was associated with a decreased risk of symptomatic vasospasm (0.31, 95% CI 0.20 to 0.48; P<0.001), cerebral infarction (0.32, 95% CI 0.20 to 0.52; P <0.001) and poor outcome (0.40, 95% CI 0.25 to 0.62; P<0.001). We observed no evidence for publication bias. Statistical heterogeneity was not present in any analysis. CONCLUSION Cilostazol is associated with a decreased risk of symptomatic vasospasm and may be clinically useful in the treatment of delayed cerebral vasospasm in patients with aSAH. Our results highlight the need for a large multi-center trial to confirm the observed association.

Cerebral Abscess following Mechanical Thrombectomy for Ischemic Stroke: Report of a Case and Review of Literature

Cerebral infections have been reported after endovascular interventions such as embolization and coiling. Such complications are extremely rare and only one other case has been reported in a patient who underwent an endovascular therapy for ischemic stroke. We report a 32-year-old woman, who presented to our hospital with headaches lasting four weeks after an endovascular intervention for ischemic stroke via mechanical thrombectomy. Further investigations revealed a cerebral abscess in the area of the infarct. She was effectively treated with antibiotics in combination with stereotactic drainage and was discharged after she made a good recovery. A review of literature on cerebral abscesses after minimally invasive procedures such as endovascular intervention was also done and is being presented in this paper. A cerebral abscess can occur rarely after endovascular interventions. A high degree of suspicion is important in identifying patients with an abscess and appropriate treatment can prevent significant morbidity or even death.