Does post dural puncture headache exist in idiopathic intracranial hypertension? A pilot study

Background/ObjectiveOccurrence of post-dural puncture headache (PDPH) after diagnostic lumbar puncture (LP) for idiopathic intracranial hypertension (IIH) may seem very unlikely in clinical practice. Nevertheless, it has been suggested by several studies, mainly in sub-group analyses. We aimed to evaluate the prevalence of PDPH in an IIH population and determine any eventual predictive factors of PDPH occurrence.MethodsWe conducted a retrospective multiple-center observational study. All newly diagnosed IIH patients who met the International Classification of Headache Disorders (ICHD-3) or the Dandy modified criteria were included from three different French hospitals. They all underwent LP following the same process with the same type of needle. We recorded PDPH occurring within five days after LP, as defined by ICHD-3 criteria.ResultsSeventy-four IIH patients were recruited, of whom 23 (31%) presented with PDPH. Neither classical risk factors for PDPH such as body mass index, age or gender, nor cerebrospinal fluid opening pressure, or specific IIH features were associated with occurrence of PDPH.ConclusionPDPH can occur after LP in IIH patients. Clinicians should be aware of this possible event during the IIH diagnosis assessment and should not automatically reconsider IIH diagnosis. PDPH prevention using an atraumatic needle and dedicated PDPH treatment seem relevant in IIH patients.     

Pseudotumor cerebri syndrome in children: Clinical characteristic and re-classification

AimTo describe the clinical characteristics of children with pseudotumor cerebri syndrome (PTCS) who were diagnosed according to the modified Dandy criteria and to reclassify them according to the newly proposed diagnostic criteria by Freidman.MethodologyThis retrospective study included the period from January 2016-to July 2021.Results50 patients were included; 34 males and 16 females with a male to female ratio of 2.1:1. The average age at onset of symptoms was 8 years. Obesity was noticed in 6 (12%) patients; 34 (68%) had symptoms upon presentation. The most common presenting symptom was headache (28 patients; 56%), papilledema was present in 33 (66%) patients. Most patients (37; 74%) had an initial cerebrospinal fluid (CSF) pressure ≥280 mmH₂O. At last follow-up, papilledema resolved in 11/32 (34.3%) patients, and headache resolved in 17/23 (74%) patients. 22/50 (44%) patients fulfilled the definite criteria proposed by Freidman, 11/50 (22%) fulfilled the probable, 10/50 (20%) were categorized as possible, and 7 (14%) patients were categorized as unmet.ConclusionPTCS is a chronic condition. Managing patients who do not have papilledema or who do not meet the newly proposed higher CSF pressure is challenging. Although, applying the newly proposed criteria captured most of our patients, however, around one quarter were managed based on clinical experience. This study indicates a strong need for future guidelines tailored specifically for children, taking into consideration that the cut-off point of CSF pressure might not be similar for all populations.     

Cobalt hip prosthesis intoxication mimicking an autoimmune disease

Cobalt-containing hip prosthesis may cause systemic toxicity due to the release of cobalt from metal-on-metal (MoM) joint arthroplasty into the bloodstream. High cobalt blood levels can lead to a variety of clinical manifestations, mimicking other disorders, especially autoimmune, hematologic, and infectious diseases. Our purpose is to describe a clinical case of cobalt hip prosthesis intoxication mimicking an autoimmune disease, with systemic inflammation signs, arthro-myalgias unrelated to overt synovitis, and multiple autoantibody positivity. A 69-years-old woman presented with a 1-year history of right coxalgia, recurrent fever, arthro-myalgias, mediastinal and right iliac reactive lymphadenopathy. She underwent hip replacement surgery seven years earlier. The physical examination was unremarkable except for right hip pain. Laboratory tests showed markedly increased inflammatory indices and microbiological tests were all negative. Ultrasound-guided arthrocentesis of right hip yielded limpid fluid with negative cultures. Increased cobalt levels in plasma and urine showed metal intoxication. Magnetic resonance imaging with metal artifact reduction sequence (MARS) confirmed a periprosthetic mass as usually seen in reaction to metal debris. Prosthesis substitution was performed with a resolution of the clinical picture and normalization of cobalt levels.     

Clinical spectrum of the pseudotumor cerebri in children: Etiological,clinical features,treatment and prognosis

Objective: Pseudotumor cerebri (PTC) is a clinical condition characterized by signs and symptoms of increased intracranial pressure, such as headache and papilledema. Our aim was to investigate the etiological and clinical features of pseudotumor cerebri (PTC) in children. Materials and method: We performed a comprehensive analysis of epidemiology, diagnostic work-up, therapy, and clinical follow-up in 42 consecutive patients. Results: Totally 42 patients diagnosed with PTC [27 (64.3%) females and 15 (35.7%) males] were included in the study. The average age of the symptoms onset was 10.79 ± 3.43 years (range from 12 months to 17 years). Obesity was found in eleven (26.2%) of them. Two of the patients had familial mediterranean fever, two of them had posttraumatic PTC. The following diseases were one patient, respectively; mycophenolate mofetil-induced PTC, hypervitaminosis A induced PTC, corticosteroid induced withdrawal due to nephritic syndrome, use of oral contraceptives, Guillain–Barre syndrome, urinary tract infection, varicella-zoster virus infection and dural venous sinus thrombosis associated with otitis media. The most common symptom was headache, recorded in 76.2% of the patients. All patients were treated medically. Three patients in our group also required a ventriculoperitoneal shunt. Conclusion: Pseudotumor cerebri is an avoidable cause of visual loss, both in adults and children. Pre-pubertal obese girls are more common. Medical therapy appeared to be successful in treating pediatric PTC in most patients. Nevertheless, despite adequate treatment, children can rarely experience loss of visual field and acuity; thus, prompt diagnosis and management are important.     

Haemophilic Pseudotumour: Surgical Management of a Rare Case

Haemophilia is a common cause of genetically inherited bleeding disorders. Pseudotumours occur in 1–2 % of persons with severe forms of haemophilia. These are a result of repeated haemorrhage into soft tissues, subperiosteum or a site of bone fracture with inadequate resorption of the extravasated blood. There are a number of therapeutic alternatives for this dangerous condition: surgical removal, percutaneous management, irradiation, embolization etc. In this case report, we describe the natural history, clinical course and successful surgical management of a patient with haemophilia who presented with a massive pseudotumour. We also briefly review the relevant literature on the various therapeutic modalities that have been implemented in the management of this rare complication. Though surgeons may be averse to operate on haemophiliacs, primary surgical management as done in our case may prove to be the definitive treatment option for such patients.     

Pseudotumor cerebri syndrome in a patient with narcolepsy type 1

Type 1 narcolepsy (NT1) is a chronic primary disorder of hypersomnolence characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations and disrupted nocturnal sleep.NT1 is linked to hypothalamic hypocretin deficiency, strongly associated with Human Leukocyte Antigen (HLA) marker DQB1*06:02 and of probable autoimmune origin. NT1 is usually associated with increased rates of overweight and obesity, and sometimes with increases in overnight blood pressure and increased rates of hypoventilation with raised CO₂ levels overnight. Many of these are predisposing factors for pseudotumor cerebri syndrome (PTCS).We present a case of a young girl with both NT1 and PTCS that responded well to treatment with acetazolamide after early identification, with improvement of headache and resolution of hypoventilation.     

Isolated Spinal Neurocysticercosis : Unusual Ocular Presentation Mimicking Pseudotumor Cerebri

Spinal intradural cysticercosis is a rare manifestation of neurocysticercosis. We report a unique patient who showed visual symptoms and normal imaging of the brain caused by isolated spinal neurocysticercosis. A 59-year-old male patient was admitted to the emergency unit with a history of severe headache and progressive blurred vision. Brain computed tomographic scanning and magnetic resonance imaging showed normal cerebral anatomy without hydrocephalus. The fundoscopic evaluation by an ophthalmologist showed bilateral papilledema. Perimetry studies revealed visual field defects in both eyes. With the diagnosis of pseudotumor cerebri, a lumbar tap was attempted; however, we could not drain the cerebrospinal fluid in spite of appropriate attempts. Lumbar magnetic resonance imaging revealed multilevel intraspinal lesions that were confirmed histologically to be neurocysticercosis. An intraoperative lumbar puncture revealed an increased opening pressure and cytochemical analysis showed elevated cerebrospinal fluid protein level. The headache resolved immediately after surgery. However, the visual symptoms remained and recovered only marginally despite antihelminthic medications after six months of operation.     

肥大细胞对特发性眼眶炎性假瘤纤维化的作用研究

目的：探讨肥大细胞在特发性眼眶炎性假瘤纤维化中的作用。方法：将分离纯化的大鼠骨髓肥大细胞和同源大鼠眼眶成纤维细胞,分别进行单独和共育培养。观察肥大细胞和成纤维细胞共育对成纤维细胞在形态和数量上的影响。结果：肥大细胞和成纤维细胞共育能够显著增强成纤维细胞的增殖活性,共育组中单位面积内的成纤维细胞数较对照组显著增加,差异具有统计学意义P﹤0.01）。结论：肥大细胞对眼眶成纤维细胞的增殖具有显著的促进作用,提示其在特发性眼眶炎性假瘤纤维化过程中发挥着重要作用。