[Epicutaneous patch testing in delayed drug hypersensitivity reactions induced by antiepileptic drugs]. / Place du patch-test dans le diagnostic des réactions d'hypersensibilités retardées induites par les antiépileptiques.

INTRODUCTION: Antiepileptic drugs are widely used and are associated with numerous side effects including skin eruptions. Epicutaneous tests have been used with variable success in skin drug reactions. The purpose of this study was to evaluate the profitability of epicutaneous tests in delayed hypersensitivity reactions induced by antiepileptic drugs. METHODS: We analyzed all cases of allergic skin reactions to antiepileptic drugs notified in regional pharmacovigilance center of Sfax (Tunisia) between June 1, 2014 and April 30, 2016. The imputation score, determined using the French imputation method, should be at least doubtful. Patch-tests were performed in accordance with the general Europen network on Drug Allergy/European Academy of Allergy and Clinical Immunology (ENDA/EAACI) guidelines. Patch-tests were read according to the generally accepted criteria of the International contact dermatitis research group (ICDRG). RESULTS: In our study, 20 patients were included, among which 23 events were observed. The drug involved in delayed hypersensitivity reactions was carbamazepine in 11 cases, phenobarbital in 10 cases and valproic acid in 4 cases. The clinical reactions caused by the drug were classified as maculopapular exanthema (11 cases), DRESS syndrome (6 cases), Stevens-Johnson syndrome (2 cases), fixed drug eruption (2 cases) and erythroderma (2 cases). Patch-tests were positive in 19 patients (95 %). Cross-reactivity between antiepileptic drugs was observed in 4 cases: between valproic acid and carbamazepine in 2 cases between valproic acid and phenobarbital in 1 case and between phenobarbital and carbamazepine in 1 case. CONCLUSION: In this study, patch testing was a safe and useful method in confirming the culprit drug in delayed hypersensitivity reactions induced by antiepileptic drugs.

Drug-resistant schizophrenia-like psychosis associated with temporal non-anaplastic pleomorphic xanthoastrocytoma: unusual revealing symptom of a rare pathology.

Introduction and importance: Pleomorphic xanthoastrocytoma (PXA) was first described by Kepes et al. in 1979. Fewer than 200 cases have been reported in the literature. It generally involves the temporoparietal lobe. PXA has a favorable prognosis. The most reported clinical manifestation is epileptic seizures. Revealing psychiatric symptoms have an incidence varying from 50 to 78%. The most common symptoms encountered are anxiety disorders, depression, schizophrenia-like psychosis, cognitive dysfunction or even anorexia nervosa. Case presentation: Here, the authors report a new case of non-anaplastic pleomorphic xanthoastrocytoma revealed by a drug-resistant schizophrenia-like psychosis in a 26-year-old male patient known with epileptic seizures in whom these two pathologies were intertwined and had been evolving for 5 years. The postoperative course was uneventful, and positive symptoms of schizophrenia were relatively stabilized at discharge. Clinical discussion: Given the superficial hemispheric location of PXA, the most common clinical presentation is seizures. Psychiatric symptoms revealing brain tumors have an incidence varying from 50 to 78%. Most of these symptoms concern frontal and limbal tumors. In their case, the tumor was located in the right temporal lobe. Surgery was performed and postoperative course was uneventful even though there are conflicting reports regarding the importance of the surgical excision quality. Conclusion: PXA remains a rare and benign primary CNS tumor. Psychiatric disorders represent a rare revealing mode of this pathology, which must lead to neuroimaging in any patient carrying this type of symptoms.

A case of multiples liver metastases from a grade 2 brain meningioma: a rare entity with comprehensive literature review.

Introduction and importance: Meningiomas are primary benign extra-axial central nervous system neoplasms that originate in meningothelial cells. Extra-neurological metastases are quite rare and occur in 0.1% of cases. The main metastatic sites are usually the lungs, bones, pleura, mediastinum and lymph nodes. Hepatic locations are quite rare and account for ~3% of all extracranial metastases. The dissemination route is still a subject of debate. Suggested routes of dissemination include the venous system, lymph nodes, or even cerebrospinal fluid. The treatment is based on complete surgical excision or on radiosurgery and adjuvant radiotherapy in case of subtotal resection. Case presentation: The authors present the following clinical case of a 31-year-old healthy male patient with surgical history of meningioma excisions, who presents, 3 years later, evidence of liver masses on tomography and confirmed diagnosis of liver metastases from brain meningioma after biopsy with favorable outcomes after chemotherapy. Clinical discussion: The overall incidence of extra-neurological metastases of meningiomas remains low. The vast majority of these metastases concern those of high grade, namely WHO grade 2 and 3 as it was reported in our case. Hepatic metastases remain quite rare and account for ~3% of all extracranial locations. Due to these characteristics of having low recurrence and its rare frequency of metastasis to extracranial sites, the authors, through their case, will dig into the literature to dissect this rare entity. Conclusion: In the report of liver lesions, the first differential diagnosis in mind should be metastatic lesions, if there is a prior clinical history of primary tumors.

A case of adult cerebellar liponeurocytoma with atypical radiological features and long survival with literature review.

Cerebellar liponeurocytoma or lipomatous medulloblastoma is a rare oncological entity. Knowledge regarding the management and outcomes of these rare tumors are still evolving. Very few cases have been described previously in the literature. The authors report a case of a middle-aged woman operated on twice, 8 years apart, with uneventful postoperative follow-ups. Radiological characteristics were revealed atypically on the computed tomography scan and magnetic resonance imaging. Histopathological study supported a cerebellar liponeurocytoma with classic immunohistochemical features. Through this report, the authors aim to describe atypical radiological and histopathological features of this rare entity with good outcome by going through a comprehensive review of the existing literature.

Cystic lesions of the brain: Think of the hydatid cyst.

BACKGROUND: Hydatidosis is an endemic parasitosis in Tunisia that affect mostly the liver and the lung. Brain involvment is rare. AIM: To focus on diagnostic, therapeutic and evolutive characteristics of cerebral hydatidosis. METHODS: We report all cases of cerebral hydatidosis seen in the infectious diseases and neurosurgery departments between January 2013 and June 2020. RESULTS: Six cases of intracranial hydatid cyst were reported. The male to female ratio was 3:3. Age ranged from 3 to 60 years with a median age of 20,5 years. All patients lived in rural areas. The clinical symptomatology was progressive in 4 cases. It was dominated by headache (all cases). Brain imaging confirmed the diagnosis in all cases. The hydatid cyst was solitary and supratentorial in 3 cases. All the patients were operated. Albendazole was prescribed immediately after surgery, for 6 months in 5 cases and for 3 years in the case of disseminated hydatidosis. The outcome was favorable without recurrence in all patients with an average follow-up of 3,5 ± 0,5 years. CONCLUSION: Hydatid cyst of the brain is characterized by the severity of the neurological signs, the mandatory use of surgery because of life threatening and the excellent outcomes.

A Voxel-based lesion study on facial emotion recognition after circumscribed prefrontal cortex damage.

Previous studies have shown inconsistent findings regarding the contribution of the different prefrontal regions in emotion recognition. Moreover, the hemispheric lateralization hypothesis posits that the right hemisphere is dominant for processing all emotions regardless of affective valence, whereas the valence specificity hypothesis posits that the left hemisphere is specialized for processing positive emotions while the right hemisphere is specialized for negative emotions. However, recent findings suggest that the evidence for such lateralization has been less consistent. In this study, we investigated emotion recognition of fear, surprise, happiness, sadness, disgust, and anger in 30 patients with focal prefrontal cortex lesions and 30 control subjects. We also examined the impact of lesion laterality on recognition of the six basic emotions. The results showed that compared to control subjects, the frontal subgroups were impaired in recognition of three negative basic emotions of fear, sadness, and anger - regardless of the lesion laterality. Therefore, our findings did not establish that each hemisphere is specialized for processing specific emotions. Moreover, the voxel-based lesion symptom mapping analysis showed that recognition of fear, sadness, and anger draws on a partially common bilaterally distributed prefrontal network.

Vertebral solitary bone plasmacytoma in a young adult with Trisomy 21: A case report.

Context: Solitary bone plasmacytoma (SBP) are rare lesions, accounting for less than 5% of all plasma cell proliferations. We describe a case of a 21-year-old female with Trisomy 21 presenting with cauda equina compression from an SBP. Findings: Solitary bone plasmacytoma (SBP) is a rare primary bone tumor. It is characterized by monoclonal proliferation of malignant plasma cells localized to a bone segment, without signs of systemic invasion. The vertebral location is the most common. It preferentially affects men during their 5th or 6th decade. Clinical relevance: We report the first association between solitary bone plasmacytoma and Trisomy 21.

Isolated metastatic dorsal spinal cord compression revealing prostatic adenocarcinoma.

Prostate cancer is the second common etiology of cord compression after lung cancer. Its slow natural history justifies an aggressive treatment. The fact that the metastatic lesion precedes the primary tumor remains rare. We report the case of a 86 year-old man who was admitted for heaviness of both lower limbs responsible for gait disorder. He had flaccid paraplegia. Spinal MRI showed an epidural lesion. Histology after surgery was compatible for a metastasis of prostatic adenocarcinoma. Spinal cord compression due to prostate cancer is correlated with poor prognosis. The fact that the metastatic lesion precedes the primary tumor remains rare.

The Lhermitte-Duclos disease: a rare bilateral cerebellar location of a rare pathology.

Dysplastic gangliocytoma or Lhermitte-Duclos disease is a rare disorder characterized by a slowly progressive unilateral tumour mass of the cerebellar cortex. It is probably hamartomatous, although the exact pathogenesis remains unknown. Lhermitte-Duclos disease was recently encountered to be part of a multiple hamartoma-neoplasia complex (Cowden's syndrome). It typically presents in young adults, although it has been encountered at all ages. We present the case of bilateral cerebellar location of this pathology in a 50-year-old man presented with a progressive onset and worsening of headaches accompanied by nuchal rigidity, photophobia and nausea awakening each morning. Upon physical examination, the patient was awake with a discrete right vestibular syndrome made of positive Romberg without nystagmus. Magnetic Resonance Imaging (MRI) was performed and revealed salient "tiger stripe" appearance of the bilateral cerebellar cortex relevant to a Lhermitte-Duclos disease.

Ruptured Central Nervous System Dermoid Cyst of Suprasellar Region Manifesting as Unusual Epileptic Seizure.

BACKGROUND: Central nervous system dermoid cysts are rare lesions derived from ectopic epithelial cells. They are slow-growing benign tumors but may cause significant morbidity through compression of neurologic and vascular structures and, rarely, rupture into the subarachnoid space. CASE DESCRIPTION: We present a rare case of a spontaneously ruptured intracranial dermoid cyst in a 32-year-old man presenting as new-onset epileptic seizures due to chemical meningitis caused by dissemination of fat or lipid droplets. CONCLUSIONS: The dermoid cyst is a rare entity, the rupture of which is exceptional and often spontaneous. It is manifested by a polymorphic and nonspecific clinical picture requiring the use of imaging. This is based on CT and especially MRI, which make it possible to positively diagnose the nature of the cyst and the rupture, specify the extent of the dissemination of the lipid content in the subarachnoid spaces, and detect possible complications such as hydrocephalus. It also makes it possible to carry out a precise topographic assessment to plan the therapeutic conduct and guide a possible surgical intervention.

Brain Metastasis of Pheochromocytoma: Diagnostic and Therapeutic Challenge.

BACKGROUND: Pheochromocytoma brain metastasis is extremely rare. Few cases have been reported in the literature. Therefore, diagnosis and effective treatment of these lesions are difficult. CASE DESCRIPTION: Here, we report the case of 29-year-old woman, who was operated on for a right adrenal pheochromocytoma. Fourteen months later, a posterior fossa tumor was diagnosed. Radiologic findings mimicked an extra-axial lesion. The radiologic differential diagnosis was difficult even using magnetic resonance imaging spectroscopy. The patient was operated on through a retrosigmoid approach. Local recurrence occurred 6 months after first surgery. The patient died 1 month later, despite a second operation. CONCLUSIONS: Although the occurrence of brain metastases in pheochromocytoma is not prevalent, patients presenting with suggestive features need to be carefully considered. Magnetic resonance imaging can help in differential diagnosis with a primary brain tumor. There are no established guidelines for the treatment of pheochromocytoma brain metastasis, for which the prognosis remains dismal, despite effective surgery.

[Pediatric esthesioneuroblastoma: an exceptional malignant lesion (a case study and literature review)]. / L'esthésioneuroblastome pédiatrique: une lésion maligne exceptionnelle (à propos d'un cas et revue de la littérature).

Esthesioneuroblastoma (ENB) is a rare malignant tumor accounting for 3% of all sinonasal cancers. It arises from the olfactory epithelium and usually affects subjects aged 30-50 years. It is uncommon in children. It is often diagnosed late because tumor remains confined to its original site for long and prognosis depends on locoregional extension (in particular to the brain and the orbital regions). We report the case of a 3-year old child with sphenoidal esthesioneuroblastoma discovered after early onset blindness. This study aims to highlight the clinical, radiological, anatomopathological, therapeutic and prognostic peculiarities of this disease while insisting on the importance of early diagnosis affecting prognosis. Unfortunately, diagnosis is still pejorative due to high recidivism rates as well as to the occurrence of distant metastases (in particular lung and bone metastases).

Applicability, Safety, and Cost-Effectiveness of Improvised External Ventricular Drainage: An Observational Study of Tunisian Neurosurgery Inpatients.

OBJECTIVE: External ventricular drainage (EVD) is an emergent neurosurgical procedure. Many commercial sets are available for EVD that are not always obtainable in all hospitals. The aim of our study was to describe new techniques to perform EVD using simple improvised materials to check the real-world applicability of the same device in the management of acute hydrocephalus and its effectiveness and safety. METHODS: We illustrated 2 techniques for a "do it yourself" improvised EVD device using materials available even in non-neurosurgery-dedicated operating rooms. We performed an observational study in our institution (April 2015 to December 2016). We included all patients presenting with acute hydrocephalus and requiring EVD. RESULTS: During a 20-month period, the new EVD device was used as a lifesaving solution for 33 patients. Good outcomes were noted in 11 of the 33 patients (33%). The EVD was complicated by fatal meningitis in 4 of the patients (12%). Malfunction occurred in 6 patients. The new EVD device costs less than US$20 for the first technique and less than US$10 for the second technique. In contrast, the cost of a standard EVD set ranges from US$170 to US$380 in Tunisia. CONCLUSIONS: The new EVD device has the potential to improve the quality of efficiency of care in difficult economic times that have changed the medical landscape, because it is both easy to make and cost-effective. Because it is an inexpensive technique, it could also be suitable for low-income countries, where neurosurgery is not yet the first and foremost health priority.

Ewing's sarcoma of the cranial vault: a case report.

Ewing's sarcoma is a malignant tumor that mainly affects young patients. It represents 10% of primary malignant tumors of the bone and 3% of malignant tumors of the child. Cranial localization is extremely rare representing less than 1% of all the localizations. We report a case of a 10-year-old girl who presented with an intracranial hypertension syndrome with left parietal mass of progressive installation. The X-ray skull showed a lytic lesion with irregular margins involving the left parietal bone. Brain magnetic resonance imaging revealed extensive parietal bone destruction involving both the inner and outer tables. The girl was operated in emergency. Histological examination concluded to Ewing's Sarcoma. The resection was incomplete (R1). The girl received induction's chemotherapy. The cerebral scanner evaluation showed no abnormalities. Then, she received consolidation's chemotherapy with concomitant local radiation therapy. Currently, the girl is in complete remission with a seven-month decline.