RESUMEN
Introduction Intracranial hypotension can occur for many reasons, including trauma, surgery, congenital defects, or spontaneous rupture of the dura mater. Symptoms appear long before cerebrospinal fluid (CSF) leaks are diagnosed. Treatment procedures include a variety of conservative and invasive techniques appropriate to the nature of the etiological cause and the severity of the disease. In this cross-sectional study, we aimed to investigate the clinical and imaging features and treatment options of intracranial hypotension patients and to compare them in terms of different etiologies. Methods The data from intracranial hypotension patients were analyzed retrospectively. Symptomatology, neurological findings, and radiological features were compared between patients with spontaneous intracranial hypotension (SIH) and those with secondary causes. Radiological outcomes of conservative treatment and epidural blood patch (EBP) were also evaluated for both groups. Results Of the 30 patients, 23 were female. In 14 of the patients (46.6%), a possible cause of CSF leakage was detected. Compared to intracranial hypotension patients with a secondary cause, SIH patients complained of posterior neck and shoulder pain more frequently (p=0.014, p=0.006). MRI features did not differ significantly when the two groups were compared (p>0.05). The first and sixth-month follow-up MRIs of patients treated with EBP or a conservative approach showed similar improvement rates (p=0.788). Conclusions There was no significant difference in radiological recovery time between conservative treatment and EBP in patients with intracranial hypotension. Radiological recovery times are similar in patients with secondary intracranial hypotension and SIH.
RESUMEN
Polyneuropathy is a frequently encountered clinical presentation where peripheral nerves are affected due to the same cause and physiopathological processes. We report a case of acute sensorimotor polyneuropathy in a patient with Tangier disease (TD) who was treated with miglustat which is a glycosphingolipid synthesis inhibitor. TD is a very rare genetic disorder caused by mutations in the ATP-binding cassette transporter A1 (ABCA1) gene which encodes the cholesterol efflux regulatory protein. It leads to accumulation of cholesterol esters within various tissues and affects lipid metabolism by deficiency of high-density lipoprotein (HDL) in the blood. Due to the accumulation of cholesterol esters in Schwann cells, it could provoke polyneuropathy in TD. Our case presented to our clinic with quadriparesis and after treated with miglustat therapy his weakness regressed.
