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- 183 Intracranial arachnoid cysts and hemorrhage. [Journal Article]
- Neurosurgery 2014 Aug.:220.
Intracranial arachnoid cysts are a relatively common finding on neuroimaging studies in the pediatric population (either incidental or symptomatic). Incidental lesions usually have a benign course and have been managed expectantly. On occasion, these cysts have presented with hemorrhage. We reviewed the clinical presentation, radiographic findings, management decisions, and surgical outcome of a single institution series of patients with intracranial arachnoid cysts who presented with hemorrhage.Retrospective chart review was conducted on all patients at Boston Children's Hospital who were diagnosed with an intracranial arachnoid cyst with associated hemorrhage. Clinical presentation, physical examination, radiographic findings, surgical intervention, complications, and postoperative outcomes were examined.Between 1993 and 2014, 16 patients were diagnosed with an intracranial arachnoid cyst with subdural or intracyst hemorrhage at Boston Children's Hospital. Average age at presentation was 8 years and 81% were male. Of these patients, 62.5% had a history of recent head trauma. Two patients had a previous craniotomy for cyst fenestration. The most common presentation was headache, present in all but 1 patient (94%). Other common symptoms and findings included nausea and vomiting (62%), lethargy (25%), and papilledema (25%). Two patients had subtle weakness on presentation. Surgery was offered to all patients and performed in all but 1 patient, whose parents refused surgery for personal reasons. All patients who received surgery had resolution of the initial symptoms on subsequent follow-up. Two patients had a small, asymptomatic extra-axial fluid collection on follow-up imaging studies. Three patients (18.7%) subsequently required subdural-peritoneal shunt placement. One patient went on to require 3 shunt revisions. All patients who received a shunt had complete resolution of subdural hemorrhage.Intracranial arachnoid cysts can rarely present with hemorrhage. Surgery is safe and effective in these patients, and the possibility of a permanent shunt should be discussed with the patient and the family in advance.
- A study of the causes of bilateral optic disc swelling in Japanese patients. [Journal Article]
- Clin Ophthalmol 2014.:1269-74.
To investigate the etiology of bilateral disc swelling in Japanese.Using Kitasato University's Department of Neuro-Ophthalmology medical records and fundus photographs of the period December 1977 through November 2010, we retrospectively identified 121 outpatients who had been initially confirmed with bilateral disc swelling.The most common cause of the bilateral disc swelling was increased intracranial pressure (ICP) (59%); followed by pseudopapillitis (16%); uveitis (8%); hypertensive retinopathy (5%); bilateral optic neuritis, acute disseminated encephalomyelitis (ADEM) and optic disc drusen (all at 2% each); and leukemia (1%). Unknown etiology accounted for 6% of the cases.Although increased ICP is the most common etiology for bilateral disc swelling, it can also be triggered by a variety of other causes. Pseudopapillitis is the most important progenitor of bilateral disc swelling in Japanese.
- Clinical profile and outcome of cerebral venous sinus thrombosis at tertiary care center. [Journal Article]
- J Neurosci Rural Pract 2014 Jul; 5(3):218-24.
Thrombosis of the cerebral venous sinuses (CVST) is an uncommon form of stroke, usually affecting young individuals. Clinical features of CVST are diverse, and for this reason, high degree of clinical suspect is mandatory to diagnose the conditions.This study was conducted over a period of 1 year (Jan 2011 to Dec 2011). This was a retrospective, observational, and noninterventional study. This study was conducted in the Department of Medicine at a tertiary care teaching center. Total 50 patients where diagnosis of CVST was confirmed by computed tomography/magnetic resonance imaging brain venogram were included in this study. All patients with diagnosis of CVST were treated according to the standard protocol and guidelines.The mean and standard deviation were obtained. The Chi-square test was used to analyze the data and P < 0.05 was considered as statistically significant.Of total 50 patients with diagnosis of CVST, 21 (42%) were males and 29 (58%) were females with 39 ± 10 years and 29 ± 7 years, respectively. Total 45 (90%) patients presented with symptoms of headache and vomiting, 13 (26%) had seizures, 12 (24%) had hemiplegia, and 19 (38%) had fever. A total of 13 (26%) patients had papilledema on fundoscopy. Total 9 (31%) out of 29 patients had diagnosis of CVST during peripartum period. Total 12 (24%) patients had hyperhomocysteinemia. Total 23 (46%) patients had sagittal sinus thrombosis, 10 (20%) had multiple sinus thrombosis, 16 (32%) had sigmoid/transverse sinus thrombosis. There was 1 (2%) patient who had bilateral cavernous sinus thrombosis, who presented with bilateral proptosis, conjunctival congestion, and external ophthalmoplegia with a history of acute or chronic maxillary and sphenoid sinusitis. Total 38 patients had evidence of infection in the form of fever, paranasal sinus (PNS) infections, Chronic suppurative otitis media (CSOM). Total 19 (38%) patients had a history and evidence of dehydration. Total 8 (16%) patients died during the course of treatment and 42 (84%) were discharged with partial and/or total recovery. Three (6%) patients required neurosurgical intervention in the form of decompressive craniotomy. Eight (16%) patients died with cerebral edema with transtentorial herniation. The mean age of death in male was significantly greater than in female patients with P < 0.02. Majority of patients succumbed had sigmoid, transverse, and/or multiple sinus involvement. Patients with multiple sinus thrombosis had greater case fatality rate.The current study highlights the burden of CVST in the study population with headache and vomiting, which was the most common presenting complaint. The superior sagittal sinus thrombosis was the most common and bilateral cavernous sinus thrombosis was the uncommon affection in CVST. One third of female population was affected in peripartum period. The infection and/or dehydration was the most commonly associated precipitating event for development of CVST and more than one fifth of the population had evidence of hyperhomocysteinemia. Mortality was more in patients with affection of sigmoid, transverse, and/or multiple sinus involvement in male patients and superior sagittal sinus thrombosis in female patients. The treatment of CVST has to be aggressive as morbidity and mortality is relatively minimal compared with the arterial stroke.
- Correlation Between Papilledema Grade and Diffusion-Weighted Magnetic Resonance Imaging in Idiopathic Intracranial Hypertension. [JOURNAL ARTICLE]
- J Neuroophthalmol 2014 Jul 3.
To explore the relationship between diffusion-weighted magnetic resonance imaging (DWI) hyperintensity of the optic nerve head (ONH) and papilledema grade in patients with idiopathic intracranial hypertension (IIH).A retrospective chart review was conducted of patients with definitively diagnosed IIH by clinical examination and visual field (VF) analysis who underwent orbital magnetic resonance imaging (MRI) within 4 weeks of diagnosis. A neuroradiologist masked to the diagnosis assessed the results of DWI for each eye independently and graded the signal intensity of the ONH into none, mild, and prominent categories. DWI grading was compared with papilledema grade and visual field mean deviation (VFMD) by Spearman rank correlation analysis and t-tests.Forty-two patients were included in the study. A statistically significant difference (P = 0.0195) was found between papilledema grade and patients with prominent DWI findings (n = 16; mean papilledema grade 3.75 ± 1.25) vs mild or no ONH hyperintensity (n = 26; mean papilledema grade 2.79 ± 1.24) at the time of initial diagnosis. DWI hyperintensity of the ONH at diagnosis was also found to be significantly correlated with the degree of papilledema at follow-up (ρ = 0.39, P = 0.0183) but not with VFMD.We found a significant correlation between the severity of papilledema and ONH hyperintensity on DWI in patients with IIH but not with VF loss or other visual parameters. These findings may offer insight into the pathophysiology of papilledema in IIH and provide a surrogate marker for the presence and severity of papilledema.
- An archaeologic dig: A rice-fruit diet reverses ECG changes in hypertension. [REVIEW]
- J Electrocardiol 2014 Jun 4.
In 1940, a young German refugee physician scientist at Duke University in Durham, North Carolina began to treat patients with accelerated or "malignant" hypertension with a radical diet consisting of only white rice and fruit, with strikingly favorable results. He reported rapid reduction in blood pressure, rapid improvement in renal failure, papilledema, congestive heart failure and other manifestations of this previously fatal illness. This treatment was based on his theory that the kidney had both an excretory and a metabolic function, and that removing most of the sodium and protein burden from this organ enabled it to regain its normal ability to perform its more important metabolic functions. It was also effective in "ordinary" hypertension, in the absence of the dramatic vasculopathy of the accelerated form. The results were so dramatic that many experienced physicians suspected him of falsifying data. Among these results was the normalization of the ECG changes seen with hypertension. This paper reviews his published experience with this radical therapy, its controversial rise to fame, and its decline in popularity with the advent of effective antihypertensive drugs. It features the ECG changes seen in this then fatal disease, and the reversal of these changes by the rice diet. This treatment, though very difficult for the patient, produced effects which make it equal or superior to current multi-drug treatment of hypertension. A poorly known but important observation was that patients who were able to follow the regime, and who were slowly guided through a gradual modification of the diet over many months, were able to transition into a very tolerable low fat, largely vegetarian diet, while leading a normal, active life, without medications, indicating that the disease state had been permanently modified.
- Point-of-Care Ultrasonography for the Identification of 2 Children With Optic Disc Drusen Mimicking Papilledema. [Journal Article]
- Pediatr Emerg Care 2014 Jul; 30(7):505-7.
We present 2 cases of asymptomatic patients who were found to have raised and blurred optic discs on physical examination, suggestive of papilledema. Evaluation in the emergency department revealed 2 well-appearing children with normal vital signs and neurologic evaluation results, without symptoms of increased intracranial pressure. Point-of-care ocular ultrasonography was performed on both children, demonstrating calcification at the optic nerve, which is diagnostic of optic disc drusen. Optic disc drusen is caused by the deposition of calcified axonal debris and is often buried within the optic disc in pediatric patients. It can cause some changes in visual acuity and visual fields, but patients who are otherwise asymptomatic can be easily diagnosed through point-of-care ultrasound, thereby sparing patients an aggressive workup if their clinical picture is otherwise reassuring.
- Visual outcomes and headache following interventions for idiopathic intracranial hypertension. [REVIEW]
- J Clin Neurosci 2014 Jun 25.
The optimal surgical management for medically refractory idiopathic intracranial hypertension (IIH) is not well established. Few studies have directly compared headache and visual outcomes across treatment modalities. A systematic analysis of case series was conducted to compare therapeutic efficacies among currently available interventions. The electronic databases from EMBASE (1980-17 September 2013), Medline (1980-17 September 2013), Cochrane databases, and references of review articles was searched. All publications reporting headache and visual outcomes following intervention for IIH were included. A total of 457 manuscripts were selected and full text analysis produced 30 studies with extractable data. All studies constituted Class III evidence. Overall, 332 patients treated by optic nerve sheath fenestration (ONSF), 287 by lumboperitoneal shunt (LPS), 61 by ventriculoperitoneal shunt (VPS), and 88 by dural venous sinus stenting, were identified. Visual acuity improved in 49.3%, 56.6%, 67.2% and 84.6% of patients following VPS, LPS, ONSF, and stent placements, respectively. Resolution of papilledema was noted in 59.9% to 97.1%. Postoperative headache improved in 36.5%, 62.5%, 75.2%, and 82.9% of patients treated with ONSF, VPS, LPS, and stenting, respectively. Shunt revision was more frequent for LPS compared to VPS (46% versus 36%; p<0.2). Among the LPS revisions, 87.5% occurred within the first 12months following initial surgery. Our pooled analysis indicated an overall similar improvement in visual outcomes across treatment modalities, and a modest improvement in headache following cerebrospinal fluid shunting and endovascular stent placement. Based on currently available literature, there is insufficient evidence to recommend or reject any treatments modalities for IIH.
- Neurocysticercosis presenting as focal hydrocephalus. [Journal Article]
- BMJ Case Rep 2014.
A 40-year-old man presented with a 2-month history of headache, nausea and vomiting, with generalised seizures for the past 15 days. On examination he had bilateral papilloedema, visual acuity was 6/6 in both eyes but perimetry showed right homonymous inferior quadrantanopia. His MRI showed numerous small cystic lesions with eccentric nodules, diffusely distributed in bilateral cerebral and cerebellar hemispheres. There was also focal hydrocephalus involving occipital and temporal horns of the left lateral ventricle leading to its selective dilation. Stool examination showed ova of Taenia solium. He was treated with albendazole, prednisone and sustained release sodium valproate for 1 month. His headache resolved and he is free of seizures. Repeat perimetry at 1 month also showed resolution of visual field defect.
- RETINAL TOXICITIES OF CANCER THERAPY DRUGS: Biologics, Small Molecule Inhibitors, and Chemotherapies. [JOURNAL ARTICLE]
- Retina 2014 Jul; 34(7):1261-1280.
To review reported retinal side effects from current cancer therapy drugs.Retinal toxicities from ophthalmologic or oncologic case reports, case series, and clinical trials were identified by a systematic literature search using Lexicomp and PubMed.Four biologics, 8 small molecule inhibitors, and 17 traditional chemotherapy agents had reported retinal side effects. For biologics, interferon alpha 2b was associated with retinopathy, denileukin diftitiox with pigmentary retinopathy, ipilimumab with a Vogt-Koyanagi-Harada-like syndrome, and trastuzumab with retinal ischemia. For small molecule inhibitors, v-raf murine sarcoma viral oncogene homolog B (BRAF) inhibitors were associated with uveitis, mitogen-activated protein kinase/extracellular signal-regulated kinase inhibitors with pigment epithelium detachments, and tyrosine kinase inhibitors with macular edema. Steroid antagonists were associated with crystalline retinopathy and macular edema. Nitrosoureas, platinum analogs, and cytosine arabinoside were associated with retinal vascular occlusions. Antimicrotubular agents were associated with cystoid macular edema but without fluorescein leakage. Retinoic acid derivatives were associated with impaired night vision, and mitotane was associated with a pigmentary retinopathy and papilledema.Certain agents used in the treatment of systemic cancer are associated with ocular complications. Awareness of these complications will allow early detections and maybe reversal of some of the ocular problems.
- A Rare presentation of neurobrucellosis in a child with Recurrent transient ischemic attacks and pseudotumor cerebri (A case report and review of literature). [Journal Article]
- Iran J Child Neurol 2014; 8(2):65-9.
Brucellosis is a multi-system infectious disease that presents with various manifestations and complications. Neurobrucellosis is an uncommon but serious presentation of brucellosis that can be seen in all stages of the disease. High index of suspicion, especially in endemic areas is essential to prevent morbidity from this disease. The case was an 11- year -old female patient who was admitted with a severe headache that was worsening over a period of 2 months. The day after each attack, she experienced transient right hemiparesia that was lasting less than one hour (TIA) as well as blurred vision and bilateral papilledema. Laboratory findings revealed serum agglutination Wright test positive at 1/320 and 2ME test positive at 1/160. A lumbar puncture showed a clear CSF with increased opening pressure (32 cmH2O), CSF examination was within normal range (pseudotumor cerebri).To our knowledge, there has been no report for recurrent TIA in pediatric neurobrucellosis in the base of pseudotumor cerebri. In endemic areas like Iran, unexplained neurological signs or symptoms should be evaluated for brucellosis.