We performed a revisionary open reduction and internal fixation for treating nonunion of the mid-shaft of the left clavicle with an autogenous cancellous bone graft. On postoperative day 4, the patient presented with neurologic deficits in the left upper extremity. We removed the implant and made a superior angulation to decompress the brachial plexus. At 6 months postoperatively, callus bridging and consolidation were visible and all hand and elbow functions were fully recovered. Our case suggests that brachial plexus neuropathy may be caused by stretching and compression after reduction and straightening of the nonunion site around adhesions or scar tissue. Therefore, care should be taken whether there are the risk factors that can cause brachial plexus neuropathy when revision surgery is performed for treating nonunion of a clavicle shaft fracture.
Bony Callus ; Brachial Plexus Neuropathies ; Brachial Plexus ; Cicatrix ; Clavicle ; Elbow ; Hand ; Humans ; Neurologic Manifestations ; Risk Factors ; Transplants ; Upper Extremity

PURPOSE: Previous studies have shown that neurologic symptoms are dominant in patients with mitochondrial diseases, and most of these patients have seizure-related disorders. The epileptic classification of these patients as Lennox-Gastaut syndrome (LGS) is as high as 25%. This study aimed to investigate the clinical manifestations, diagnoses, treatments, and epilepsy in LGS, which is associated with mitochondrial disease. MATERIALS AND METHODS: A retrospective study was conducted on 372 patients who were diagnosed with mitochondrial disease between 2006 and 2016. Of these 372 patients, 40 patients diagnosed with LGS were selected, and they were classified into two groups based on the history of West syndrome. Patient characteristics were reviewed, and associations between clinical factors and outcomes after the treatment were analyzed. RESULTS: The proportion of individuals with mitochondrial disease with LGS with a history of West syndrome was 32.5%. Among the patients with mitochondrial disease with LGS, neonatal seizure (p=0.029), seizure as the first symptom (p=0.018), and generalized paroxysmal fast activity frequency on electroencephalogram (p=0.018) in the group with a history of West syndrome were statistically significantly high. The first symptom onset (0.6±0.4 yrs vs. 1.6±0.9 yrs, p=0.003) and first seizure onset (0.9±0.7 yrs vs. 3.9±3.1 yrs, p < 0.001) were significantly faster in patients with a history of West syndrome. CONCLUSION: Close monitoring of the medical condition and early intervention might improve the prognosis of individuals with mitochondrial disease with LGS and a history of West syndrome.
Child ; Classification ; Diagnosis ; Early Intervention (Education) ; Electroencephalography ; Epilepsy ; Humans ; Infant ; Infant, Newborn ; Mitochondrial Diseases* ; Neurologic Manifestations ; Prognosis ; Retrospective Studies ; Seizures ; Spasms, Infantile

No abstract available.
Humans ; Neurologic Manifestations* ; Nitrous Oxide*

Adipsic hypernatremia is a rare disease where patients do not feel thirst even in the increased serum osmotic pressure and results in electrolyte imbalance, severely increased osmotic pressure and neurologic symptoms like nausea, vomiting, and seizures. We report a 12-year-old male patient who had underwent a trans-sphenoidal surgery for craniopharyngioma newly diagnosed with adipsic hypernatremia after having growth hormone replacement for growth hormone deficiency. The patient visited emergency room complaining of generalized weakness, tremor in both legs, and poor oral intake including water after starting growth hormone replacement therapy. Laboratory test revealed serum sodium 168 mmol/L and serum osmolality 329 mOsm/kg, despite the patient didn't feel any thirst at all. We treated him with scheduled water intake of 2.5 L a day with intranasal vasopressin. He admitted to Soonchunhyang University Gumi Hospital and Soonchunhyang University Seoul Hospital for 4 times during the following 8 months and serum sodium level and osmolality was controlled by scheduled water intake combined with intranasal vasopressin treatment. It is still unclear whether growth hormone replacement worked as a trigger of hypernatremia.
Child ; Craniopharyngioma ; Drinking ; Emergency Service, Hospital ; Growth Hormone ; Gyeongsangbuk-do ; Humans ; Hypernatremia ; Leg ; Male ; Nausea ; Neurologic Manifestations ; Osmolar Concentration ; Osmotic Pressure ; Rare Diseases ; Seizures ; Seoul ; Sodium ; Thirst ; Tremor ; Vasopressins ; Vomiting ; Water

With increasing sensitivity, availability, and use of magnetic resonance imaging (MRI) in the past three decades, there is also an increase in incidental abnormal findings. The most common findings of these incidental abnormalities are white matter lesions that are interpreted as demyelinating based on radiological criteria. MRI findings suggestive of multiple sclerosis (MS) in patients without typical MS symptoms and with normal neurologic findings are now defined as a radiologically isolated syndrome (RIS) which reported relatively low incidence, especially hard to find case report in Korea. The natural history and pathophysiologic processes of RIS remain largely unknown. The nature, prevalence, and long-term prognosis of RIS, as well as the role of disease-modifying therapy should be established in the future.
Demyelinating Diseases ; Humans ; Incidence ; Korea ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Natural History ; Neurologic Manifestations ; Prevalence ; Prognosis ; White Matter

The syndrome of the trephined is a neurologic phenomenon that manifests as sudden decline in cognition, behavior, and sensorimotor function due to loss of intracranial domain. This scenario typically occurs in the setting of large craniectomy defects, resulting from trauma, infection, and/or oncologic extirpation. Cranioplasty has been shown to reverse these symptoms by normalizing cerebral hemodynamics and metabolism. However, successful reconstruction may be difficult in patients with complex and/or hostile calvarial defects. We present the case of a 48-year-old male with a large cranial bone defect, who failed autologous cranioplasty secondary to infection, and developed rapid neurologic deterioration leading to a near-vegetative state. Following debridement and antibiotic therapy, delayed cranioplasty was accomplished using a polyetheretherketone (PEEK) implant with free chimeric latissimus dorsi/serratus anterior myocutaneous flap transfer for vascularized resurfacing. Significant improvements in cognition and motor skill were noted in the early postoperative period. At 6-month follow-up, the patient had regained the ability to speak, ambulate and self-feed—correlating with evidence of cerebral/ventricular re-expansion on computed tomography. Based on our findings, we advocate delayed alloplastic implantation with total vascularized soft tissue coverage as a viable alternative for reconstructing extensive, hostile calvarial defects in patients with the syndrome of the trephined.
Cognition ; Craniocerebral Trauma ; Debridement ; Follow-Up Studies ; Free Tissue Flaps ; Hemodynamics ; Humans ; Male ; Metabolism ; Middle Aged ; Motor Skills ; Myocutaneous Flap ; Neurologic Manifestations ; Postoperative Period ; Reconstructive Surgical Procedures

Type 2 diabetes (T2D) increases the risk for cerebrovascular disease (CVD) and dementia. The underlying molecular mechanisms remain elusive, which hampers the development of treatment or/and effective prevention strategies. Recent studies suggest that dyshomeostasis of amylin, a satiety hormone that forms pancreatic amyloid in patients with T2D, promotes accumulation of amylin in cerebral small blood vessels and interaction with Alzheimer's disease (AD) pathology. Overexpression of human amylin in rodents (rodent amylin does not form amyloid) leads to late-life onset T2D and neurologic deficits. In this Review, we discuss clinical evidence of amylin pathology in CVD and AD and identify critical characteristics of animal models that could help to better understand molecular mechanisms underlying the increased risk of CVD and AD in patients with prediabetes or T2D.
Alzheimer Disease ; Amyloid ; Blood Vessels ; Cerebrovascular Disorders ; Dementia ; Diabetes Complications ; Diabetes Mellitus, Type 2 ; Humans ; Islet Amyloid Polypeptide ; Models, Animal ; Neurologic Manifestations ; Pathology ; Prediabetic State ; Rodentia

OBJECTIVE: To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism. METHODS: The radiologic information systems at an academic center and affiliated veterans' hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient's neurologic symptoms. RESULTS: Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11). CONCLUSION: MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).
Adult ; Autopsy ; Cerebellar Ataxia ; Dementia ; Hexachlorobenzene ; Humans ; Lewy Bodies ; Magnetic Resonance Imaging ; Multiple System Atrophy ; Neurologic Manifestations ; Olivopontocerebellar Atrophies ; Parkinsonian Disorders ; Radiology Information Systems ; Sensitivity and Specificity

BACKGROUND: The neurological manifestations of Sjögren's syndrome (SjS) are nonspecific and may precede the onset of sicca symptoms. Hence, the diagnosis of SjS is often delayed. Recurrent aseptic meningitis is an uncommon neurological manifestation of primary SjS; only few cases have been reported in the medical literature. CASE REPORT: A 54-year-old woman was admitted for recurrent aseptic meningitis. The patient had a history of two episodes of aseptic meningitis, which had occurred 12 and 7 years before this presentation. The patient had overt sicca symptoms for 5 years. SjS was diagnosed based on the results of serum autoantibody tests, Schirmer's test, and salivary scintigraphy. We concluded that recurrent aseptic meningitis occurred as an initial presentation of primary SjS. CONCLUSION: This case suggest that SjS should be included in the differential diagnosis of recurrent aseptic meningitis.
Autoimmune Diseases ; Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Meningitis, Aseptic ; Middle Aged ; Neurologic Manifestations ; Radionuclide Imaging ; Sjogren's Syndrome

Digital therapeutics is an evidence-based intervention using high-quality software, with the sole purpose of treatment. As many healthcare systems are encountering high demands of quality outcomes, the need for digital therapeutics is gradually increasing in the clinical field. We conducted review of the implications of digital therapeutics in the treatment of neurological deficits for stroke patients. The implications of digital therapeutics have been discussed in four domains: cognition, speech and aphasia, motor, and vision. It was evident that different forms of digital therapeutics such as online platforms, virtual reality trainings, and iPad applications have been investigated in many trials to test its feasibility in clinical use. Although digital therapeutics may deliver high-quality solutions to healthcare services, the medicalization of digital therapeutics is accompanied with many limitations. Clinically validated digital therapeutics should be developed to prove its efficacy in stroke rehabilitation.
Aphasia ; Aphasia, Broca ; Cognition ; Delivery of Health Care ; Hemianopsia ; Hemiplegia ; Humans ; Medicalization ; Neurologic Manifestations ; Rehabilitation ; Stroke