Kearns Sayre syndrome: a rare etiology of complete atrioventricular block in children (case report).

Kearns Sayre syndrome is a rare mitochondrial abnormality first described in 1958, characterized by a triad associating progressive external ophthalmoplegia, ptosis, and pigmentary retinopathy with progressive alteration of cardiac conduction, which determines the vital prognosis of this entity. Here we report the case of a 13-year-old child of consanguineous parents who consults for recurrent syncope. The clinical exam found bilateral ptosis with complete atrioventricular block on electrocardiogram. The ophthalmological exam found pigmentary retinopathy. The patient underwent successful implantation of a double chamber pacemaker within 24 hours of admission, with an uneventful postoperative course. This case report highlights the interest of systematically assessing cardiac complications in children with mitochondrial disease such as Kearns Sayre syndrome, especially since cardiac involvement is the major prognostic factor in this disease.

Cerebrospinal fluid fistula in a patient with chronic constipation related to an autonomic dysfunction and revealed by bacterial meningitis - A case report.

BACKGROUND: Cerebrospinal fluid (CSF) fistula represents a rare neurosurgical entity that can be defined as a communication between the subarachnoid space and nasal fossa or less commonly the ear cavity. It can be spontaneous without an evident etiology or secondary following a skull base surgery or trauma. The early diagnosis of spontaneous forms remains a challenge as clinical signs (e.g., unilateral rhinorrhea) can be absent or neglected by patients and can result in meningitis. CASE DESCRIPTION: Here, we report the case of a 31-year-old man with chronic constipation complicated by chronic intracranial hypertension, and resulting in rhinorrhea with bacterial meningitis. The etiological assessment of chronic constipation retained an autonomic dysfunction with sympathetic hyperactivity (e.g., pure autonomic failure) as an underlying cause. Beta-2 transferrin testing associated with cerebral magnetic resonance imaging and computed tomography scan confirmed the diagnosis and localization of the fistula at the cribriform plate. The patient underwent an endoscopic endonasal approach with a repair of fistula. He presented with recurrent rhinorrhea 17 months later which required a surgical revision along with CSF diversion with a ventriculoperitoneal shunt. CONCLUSION: Although rare, autonomic dysfunction can result in chronic constipation in young patients, with intermittent or permanent intracranial hypertension, leading to CSF leaks. The early identification and treatment of the underlying etiology may prevent severe complications and improve the management and outcome of CSF fistula patients.

COVID-19 and SARS-Cov-2 Infection: Pathophysiology and Clinical Effects on the Nervous System.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is an infectious disease caused by SARS-Cov-2, resulting in severe acute respiratory syndrome, with high potential of spreading and infecting humans worldwide. Since December 2019, when the virus was identified in humans, the literature on COVID-19 has grown exponentially and extrarespiratory symptoms including neurologic symptoms are increasingly highlighted. METHODS: Given the high and increasing number of publications reporting neurologic involvements of SARS-Cov-2, we thought that providing an update for neurologic complications of COVID-19 would be useful for physicians and especially young trainees in neurology and neurosurgery. Indeed, in this review we discuss several neurologic aspects reported in the literature to date including the evidence and pathways of neuroinvasion in COVID-19 and the main neurologic disorders reported in the literature to date, as well as future perspectives and the potential long-term consequence of current neuroinfection in COVID-19 patients. RESULTS: Currently, there is convincing evidence that SARS-CoV-2, the etiologic agent of COVID-19, can affect the nervous system, with damage and neurologic alterations. These neurologic disorders are grouped into several categories, ranging from nonspecific and moderate symptoms such as headache, myalgia, and hyposmia to severe symptoms including cerebrovascular disease and intracranial infections. Severe neurologic symptoms such as acute cerebrovascular disease occur only in a minority of patients with usual risk factors and are associated with poor outcome. However, most COVID-19 patients exhibit only minor or mild neurologic symptoms. CONCLUSIONS: Management of COVID-19 patients should include early clinical, radiologic, and laboratory neurologic assessment, with a close follow-up, especially in severe forms. Future studies should assess late and long-term consequences of current COVID-19 patients with neurologic involvement.