Introduction: Gitelman Syndrome (GS), a rare autosomal recessive inherited disorder, is frequently unrecognized in the clinical setting. GS typically manifests with severe hypokalemia with debilitating and potentially fatal consequences if untreated. As of writing, confirmatory genetic assays are currently unavailable in the country, and the diagnosis of GS is primarily based on several biochemical laboratory tests. This results in the difficulty with prompt diagnosis of GS in the locality. Case: We present a 52-year-old male who came in with chronic, intermittent paraparesis associated with persistent hypokalemia. A diagnosis of GS was made biochemically based on renal wasting of potassium and magnesium, hypocalciuria, and metabolic alkalosis. Electrolyte correction with lifelong supplementation, and administration of Spironolactone resulted in the resolution of bilateral leg weakness. Electrolyte levels were maintained within normal limits in the outpatient setting. Conclusion GS is an uncommon potentially debilitating disorder that may lead to problematic, potentially fatal consequences to electrolyte abnormalities if left untreated. The lack of awareness and consequent delay in the diagnosis, and the unavailability of confirmatory genetic testing remains a clinical challenge. Timely recognition and initiation of treatment leads to early control of electrolyte levels, and better prognosis.
Gitelman&rsquo ; s Syndrome ; Paraparesis ; Hypokalemia ; Hypomagnesemia ; Spironolactone ; Case Report

Background: Neurofibromatosis-2 (NF2) is a rare neurocutaneous syndrome that typically presents with hearing loss, tinnitus, or weakness associated with few subcutaneous nodules. In contrast to neurofibromatosis-1 (NF1), NF2 presents clinically with more central lesions rather than peripheral lesions. The presence of bilateral vestibular schwannomas through imaging studies distinguishes NF2 from other neurocutaneous syndromes. Case: This is a case of an 18-year-old male who presented with lower paraparesis with associated hearing loss, cataract, and a few subcutaneous nodules. Centrally located lesions were suspected, thus brain and spine magnetic resonance imaging (MRI) were done revealing bilateral vestibular schwannomas and spine neurofibromas. The patient and family were advised for tumor surveillance, and apprised of surgical intervention once with brainstem compression symptoms. Conclusion NF2 is a rare debilitating disease that may lead to multiple neurologic deficits. The absence of recommended medical treatment and the multifocality of the tumors leave surgical resection a high-risk treatment option. Early recognition by tumor surveillance may give patients with NF2 a better prognosis and survivability.
Neurofibromatoses ; Neurilemmoma ; Neurofibroma ; Paraparesis ; Bevacizumab

Segmental zoster paresis(SZP)is a rare complication in herpes zoster infection,with its symptoms often neglected due to the co-existence of pain.Here we reported a case of SZP.Also,we analyzed 42 Chinese SZP cases in literature,which revealed that the male to female ratio of SZP patients was 13∶8,and the median age of disease onset was 65 years.The most commonly affected region is upper limb.The diagnosis depends mainly on typical medical history and clinical symptoms.Although there is no definite therapy for SZP,the antiviral therapy is the most commonly used treatment,which achieved complete recovery in 78.6% of the patients and partial recovery in 14.3% of the patients.
Aged ; Female ; Herpes Zoster/diagnosis* ; Humans ; Male ; Paresis/etiology* ; Upper Extremity/physiopathology*

A 53-year old man who had a left hemiparesis from head injury of traffic accident 20 years ago visited an emergency room with suddenly developed semi-comatose mental status. Brain CT showed 8.6-cm sized solid and cystic mass on right temporal lobe that was associated with hemorrhage. Solid lesion showed a strong enhancement after an administration of contrast media. Because of severe mass effect, emergency operation was performed. The mass was an intraparenchymal lesion with yellowish cystic fluid and the firm reddish-brown solid lesion was hemorrhagic. The lesion was totally resected. Pathologically, anaplastic solitary fibrous tumor/hemangiopericytoma was diagnosed with 70/10 high power fields. Postoperative radiotherapy of 50 Gy was done. Postoperative 2 months later, the patient was recovered to alert mental state. We report this unusual case of non-dural based intraparenchymal solitary fibrous tumor/hemangiopericytoma with high mitotic index and acute massive hemorrhage. Rapid tumor growth of hypervascular tumor might have a chance of bleeding.
Accidents, Traffic ; Brain ; Brain Neoplasms ; Central Nervous System ; Cerebral Hemorrhage ; Contrast Media ; Craniocerebral Trauma ; Emergencies ; Emergency Service, Hospital ; Hemangiopericytoma ; Hemorrhage ; Humans ; Mitotic Index ; Paresis ; Radiotherapy ; Solitary Fibrous Tumors ; Temporal Lobe

Supratentorial extraventricular anaplastic ependymoma (SEAE) in adults is a relatively rare intracranial tumor. Because of the very low prevalence, only a few cases have been reported. According to a recent study, SEAE is associated with a poor prognosis and there is no definite consensus on optimal treatment. We report a case of an adult SEAE patient who had no recurrence until seven years after a gross total resection (GTR) followed by conventional radiotherapy. A 42-year-old male had a persistent mild headache, left facial palsy, dysarthria, and left hemiparesis. Preoperative neuroimaging revealed an anaplastic astrocytoma or supratentorial ependymoma in the right frontal lobe. A GTR was performed, followed by adjuvant radiotherapy. Histologic and immunohistochemical results revealed anaplastic ependymoma. After seven years of initial therapy, a regular follow-up MRI showed a 3-cm-sized partially cystic mass in the same area as the initial tumor. The patient underwent a craniotomy, and a GTR was performed. Histopathologic examination revealed recurrence of the SEAE. External radiotherapy was performed. The patient has been stable without any disease progression or complications for 12 months since the surgery for recurrent SEAE.
Adult ; Astrocytoma ; Consensus ; Craniotomy ; Disease Progression ; Dysarthria ; Ependymoma ; Facial Paralysis ; Follow-Up Studies ; Frontal Lobe ; Headache ; Humans ; Magnetic Resonance Imaging ; Male ; Neuroimaging ; Paresis ; Prevalence ; Prognosis ; Radiotherapy ; Radiotherapy, Adjuvant ; Recurrence ; Supratentorial Neoplasms

OBJECTIVE: To investigate the effects of combination functional electrical stimulation (FES) and standing frame training on standing balance in stroke patients. METHODS: Patients who had hemiparesis and postural instability after stroke were randomly assigned to one of the two groups; study group underwent FES on the quadriceps and tibialis anterior muscle simultaneously with standing balance training. The control group received standing frame training and FES separately. Both the groups received their respective therapies for 3 weeks. Stability index in Biodex Balance master system, Berg Balance Scale (BBS), manual muscle test, the Korean version of Modified Barthel Index, and Korean version of Mini-Mental State Examination were used to evaluate the effects of the treatment. RESULTS: In total, 30 patients were recruited to the study group and 30 to the control group. Three weeks after treatment, both the groups showed improvement in postural stability scores and physical and cognitive functions. When changes in postural stability were compared between the groups, the study group showed more significant improvement than the control group with regards to the scores of BBS and the stability indices. CONCLUSION: In this study, we found the therapeutic effectiveness of combined therapy of FES and standing frame in subacute stroke patients. The presented protocol is proposed as time-saving and can be applied easily in the clinical setting. Thus, the proposed combined therapy could be a useful method for improving standing balance in subacute stroke patients.
Cognition ; Electric Stimulation ; Humans ; Methods ; Paresis ; Rehabilitation ; Stroke

Thiamine (vitamin B₁) is a water-soluble vitamin that is not endogenously synthesized in humans. It is absorbed by the small intestine, where it is activated. Its active form acts as a coenzyme in many energy pathways. We report a rare case of thiamine deficiency in a 3.5-year old boy with short bowel syndrome secondary to extensive bowel resection due to necrotizing enterocolitis during his neonatal age. The patient was parenteral nutrition-dependent since birth and had suffered from recurrent central catheter-related bloodstream infections. He developed confusion with disorientation and unsteady gait as well as profound strabismus due to bilateral paresis of the abductor muscle. Based on these and a very low thiamine level he was diagnosed and treated for Wernicke encephalopathy due to incomplete thiamine acquisition despite adequate administration. He fully recovered after thiamine administration. After 1999 eight more cases have been reported in the PubMed mostly of iatrogenic origin.
Child ; Enterocolitis, Necrotizing ; Gait Disorders, Neurologic ; Humans ; Intestine, Small ; Male ; Parenteral Nutrition, Total ; Paresis ; Parturition ; Short Bowel Syndrome ; Strabismus ; Thiamine Deficiency ; Thiamine ; Vitamins ; Wernicke Encephalopathy

BACKGROUND AND OBJECTIVES: Video head impulse tests (vHITs) and caloric tests are widely used to assess the loss of vestibular function in acute vestibular neuritis. Although previous studies have reported on the results of each test, longitudinal comparison of these tests is rare. In the present study, vHITs and caloric tests were performed in patients with unilateral vestibular neuritis during the acute phase and after a long follow-up period (>6 months). The goal of this study was to evaluate the changes in vHIT and caloric test results and to analyze the relationships between them. SUBJECTS AND METHOD: Between September 2013 and December 2015, charts from 13 patients with unilateral vestibular neuritis were retrospectively reviewed. Among the 13 patients, caloric tests and vHITs were performed in 9 and 10 patients, respectively. Results of the vHITs and caloric tests were analyzed and the changes were compared. RESULTS: During the acute phase of vestibular neuritis, the results of the caloric test showed an increase in canal paresis (CP), and the results of the vHIT showed a decrease in horizontal gain. Although subjective symptoms improved in all patients after a long follow-up period (mean: 13.9 months), the occurrence of CP determined from the caloric test was not significantly changed (p=0.889). On the other hand, the mean horizontal gain of the vHIT had improved significantly (p < 0.05). CONCLUSION: While CP determined from the caloric test did not change after a long follow-up period, the decreased horizontal gain in the vHIT was significantly recovered in patients with unilateral vestibular neuritis.
Caloric Tests* ; Follow-Up Studies ; Hand ; Head Impulse Test ; Head* ; Humans ; Methods ; Paresis ; Retrospective Studies ; Vestibular Neuronitis*

We encountered a very rare case of spontaneous spinal cerebrospinal fluid (CSF) leakage and a spinal intradural arachnoid cyst (AC) that were diagnosed at different sites in the same patient. These two lesions were thought to have interfered with the disease onset and deterioration. A 30-year-old man presented with sudden neck pain and orthostatic headache. Diplopia, ophthalmic pain, and headache deteriorated. CSF leakage was confirmed in C2 by radioisotope cisternography, and an epidural blood patch was performed. While his symptoms improved gradually, paraparesis suddenly progressed. Thoracolumbar magnetic resonance imaging (MRI) revealed an upper thoracic spinal intradural AC, which was compressing the spinal cord. We removed the outer membrane of the AC and performed fenestration of the inner membrane after T3-4 laminectomy. Postoperative MRI showed complete removal of the AC and normalized lumbar subarachnoid space. All neurological deficits including motor weakness, sensory impairment, and voiding function improved to normal. We present a case of spontaneous spinal CSF leakage and consecutive intracranial hypotension in a patient with a spinal AC. Our report suggests that if spinal CSF leakage and a spinal AC are diagnosed in one patient, even if they are located at different sites, they may affect disease progression and aggravation.
Adult ; Arachnoid ; Blood Patch, Epidural ; Cerebrospinal Fluid Leak ; Cerebrospinal Fluid ; Diplopia ; Disease Progression ; Headache ; Humans ; Intracranial Hypotension ; Laminectomy ; Magnetic Resonance Imaging ; Membranes ; Neck Pain ; Paraparesis ; Spinal Cord ; Subarachnoid Space

Traumatic cervical epidural hematoma (EDH) with no osseous fracture or underlying hematological abnormalities is a rare disorder that sometimes requires emergent surgical decompressive therapy. A 47-year-old woman was admitted to our emergency room due to severe neck pain and rapid onset hemiparesis after a car accident. Plain cervical radiographs and computed tomography scan did not reveal any abnormality. However, magnetic resonance imaging (MRI) revealed a large posterior EDH compressing the spinal cord extensively from C3 to C5. Emergent hematoma removal was performed following laminectomy, and subsequently the patient showed substantial clinical improvement. Complete removal of the hematoma was confirmed by MRI at 10 days after surgery. Here, the authors present a discussion of the etiology, pathogenesis, and prognosis of this rare pathologic entity.
Emergency Service, Hospital ; Female ; Hematoma ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Middle Aged ; Neck Pain ; Paresis ; Prognosis ; Spinal Cord