No abstract available.
Anesthesia* ; Humans ; Opsoclonus-Myoclonus Syndrome* ; Young Adult*

Opsoclonus-myoclonus syndrome (OMS) is characterized by opsoclonus and arrhythmic myoclonic jerks predominantly involving the trunk, limbs, and head. We present two patients with OMS after respiratory tract infection who exhibited diffuse cerebral hypometabolism, particularly in the parieto-occipital cortex on 18F-fluorodeoxyglucose positron-emission tomography (F-FDG PET). This metabolic change might be a consequence rather than a direct cause of motor symptoms, which may be attributable to brainstem or cerebellar pathology.
Brain Stem ; Extremities ; Head ; Humans ; Myoclonus ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome* ; Pathology ; Positron-Emission Tomography ; Respiratory Tract Infections

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.
Brain ; Dizziness ; Humans ; Magnetic Resonance Imaging ; Myoclonus ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome ; Paraneoplastic Syndromes, Nervous System ; Prevalence

OBJECTIVEClinical manifestations of opsoclonus-myoclonus syndrome (OMS) in children were summarized and analyzed and the clinical features and therapeutic approaches to OMS were investigated in order to improve its diagnosis and management.

METHODSClinical information on features and management of 6 cases with OMS inpatients being followed up from 2006 to 2007 were collected and analyzed.

RESULTSAmong the 6 cases, one was male and the other five were female. The age at the onset ranged from 12 to 26 months (average 21.0 months). Four of them had history of prior infection. The symptoms were opsoclonus, myoclonus, ataxia, sleep disturbances and behavioural problems in the 6 cases. Urinary DL-3-methoxy-4-hydroxy-acid amygdalin (VMA) was positive in 1 case. Abdominal B-mode ultrasound showed a mild hepatomegaly in 4 cases. The EEG showed abnormal findings such as slow background activity in 3 cases. Epileptiform discharges were found in none of the patients. MRI showed a high signal in medial longitudinal fasciculus and tectospinal tract on T2-weighted image in 1 case. Computerized tomography found L3-4 arachnoid cysts in 1 case and was normal in the others. Adrenocorticotropic hormone (ACTH) was given to all these patients and was effective in all during acute stage. In 2 cases the disease relapsed during follow-up stage.

CONCLUSIONOMS is a rare neurological condition with opsoclonus, myoclonus, ataxia, sleep disturbances and behavioral problems, which might relapse easily and is associated with adverse neurological outcome. ACTH therapy is effective in management of OMS.

Adrenocorticotropic Hormone ; therapeutic use ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; therapy ; Prognosis ; Recurrence ; Treatment Outcome

Opsoclonus-myoclonus syndrome (OMS) is a rare neurologic disorder characterized by progressive opsoclonus (irregular, rapid, horizontal and vertical eye movements), myoclonus, cerebellar dysfunction and severe hypotonia. Here we present the case of a 19-year-old man with OMS induced by mumps virus infection. Emergency physicians should know about the typical presentation of OMS and make a proper response.
Cerebellar Diseases ; Emergencies ; Eye ; Humans ; Mumps ; Mumps virus ; Muscle Hypotonia ; Myoclonus ; Nervous System Diseases ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome ; Young Adult

BACKGROUND: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder that is characterized by involuntary eye movements and myoclonus. OMS exhibits various etiologies, including paraneoplastic, parainfectious, toxic-metabolic, and idiopathic causes. The exact immunopathogenesis and pathophysiology of OMS are uncertain. CASE REPORT: We report the case of a 19-year-old male who developed opsoclonus and myoclonus several days after a flu-like illness. Serological tests revealed acute mumps infection. The findings of cerebrospinal fluid examinations and brain magnetic resonance imaging were normal. During the early phase of the illness, he suffered from opsoclonus and myoclonus that was so severe as to cause acute renal failure due to rhabdomyolysis. After therapies including intravenous immunoglobulin, the patient gradually improved and had fully recovered 2 months later. CONCLUSIONS: This is the first report of OMS associated with mumps infection in Korea. Mumps infection should be considered in patients with OMS.
Acute Kidney Injury ; Brain ; Cerebrospinal Fluid ; Eye Movements ; Humans ; Immunoglobulins ; Korea ; Magnetic Resonance Imaging ; Male ; Mumps ; Mumps virus* ; Myoclonus ; Nervous System Diseases ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome* ; Rhabdomyolysis ; Serologic Tests ; Young Adult

Enterovirus 71 (EV71) has been recognized as a frequent cause of epidemics of hand-foot-and-mouth disease (HFMD) associated with severe neurological symptoms. In the spring of 2009, HFMD was epidemic in Korea. Severe cases with complication, including death, have been reported and it has become a public health issue. Most symptomatic EV71 infections commonly result in HFMD or herpangina. These clinical manifestations can be associated with neurologic syndromes frequently. Neurologic syndromes observed in EV71 include meningitis, meningoencephalomyelitis, poliomyelitis-like paralytic disease, Guillain-Barre syndrome, transverse myelitis, cerebellar ataxia, opsoclonus-myoclonus syndrome, benign intracranial hypertension, and brainstem encephalitis. Examinations for EV 71 were performed from the stools, respiratory secretion or CSF of the children by realtime PCR. Gene analysis showed that most of them were caused by EV71 subgenotype C4a which was prevalent in China, 2008. Public health measures including personal and environmental hygiene, must to target daycare centers, kindergartens, and schools where highly susceptible children congregate. To prevent the spread of infection, preschools where transmission persists for more than 2 incubation periods, have been recommended for closure, and trigger criteria for voluntary closure was instituted. During closure, operators are to thoroughly clean the centers before they are allowed to reopen. In addition, parents are advised to ensure that their children adopt a high standard of personal hygiene and to keep the infected child at home until full recovery. Because the outbreaks occur in a cyclical pattern, surveillance system to predict next outbreaks and adequate public health measures to control need to be planned for future. Control of EV71 epidemics through surveillance and public health intervention needs to be maintained in Korea. Future research should focus on understanding of EV71 virulence, identification of the receptor(s) for EV71, development of antiviral agents and development of vaccine.
Antiviral Agents ; Brain Stem ; Cerebellar Ataxia ; Child ; China ; Dietary Sucrose ; Disease Outbreaks ; Encephalitis ; Enterovirus ; Guillain-Barre Syndrome ; Herpangina ; Humans ; Hygiene ; Korea ; Meningitis ; Myelitis, Transverse ; Opsoclonus-Myoclonus Syndrome ; Parents ; Polymerase Chain Reaction ; Pseudotumor Cerebri ; Public Health

Scrub typhus is an infective acute febrile disorder caused by the intracellular parasite Orientia tsutsugamushi. Neurological manifestations of scrub typhus are meningoencephalitis, cerebellitis, transverse myelitis, papilledema, and cranial nerve palsy. However, opsoclonus-myoclonus syndrome associated with scrub typhus has been rarely reported. A 59-year-old man developed vertigo, nausea, vomiting, and imbalance following scrub typhus infection for eight days. Examination showed eschar at the axilla, decreased mentality, and opsoclonus-myoclonus syndrome. Video-oculography disclosed opsoclonus with an amplitude of 15°–20° and a frequency of 6–8 Hz. The serum antibody titers to Orientia tsutsugamushi were 1:5,120, and cerebrospinal fluid analysis revealed pleocytosis. Brain magnetic resonance imaging was normal. Neurological symptoms and signs completely improved by systemic steroid and antibiotics treatment. Various mechanisms including direct disseminating inflammation or indirect immune modulation may give rise to neurological complications following scrub typhus.
Anti-Bacterial Agents ; Axilla ; Brain ; Cerebrospinal Fluid ; Cranial Nerve Diseases ; Encephalitis ; Humans ; Inflammation ; Leukocytosis ; Magnetic Resonance Imaging ; Meningoencephalitis ; Middle Aged ; Myelitis, Transverse ; Nausea ; Neurologic Manifestations ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome ; Orientia tsutsugamushi ; Papilledema ; Parasites ; Scrub Typhus ; Vertigo ; Vomiting

OBJECTIVETo investigate the basic clinical characteristics of paraneoplastic neurological syndrome (PNS) in children.

METHODTo retrospectively analyze the clinical data of 12 PNS children who were hospitalized in neurology department in Beijing Children's Hospital from 2010 to 2011. Some patients were followed up after surgery.

RESULTIn 12 patients with PNS, 11 were male and 1 was female. The mean onset age were (30.5 ± 15.3) months. The mean duration from neurological symptom onset to finding out of tumor was (112.7 ± 154.4) days. The onset of the disease in 2 patients was acute, in 3 was subacute and in the other 7 was chronic (2 of 7 had 2 to 3 relapses). Of 12 patients, 11 had symptoms of ataxia (3 patients also had opsoclonus and myoclonus, OMS), 1 had weakness of limbs at onset and then had ataxia. Nine of 12 patients had surgery, and pathologic diagnosis was neuroblastoma and ganglioneuroma. Six patients were followed-up for 8 to 21 months. One patient had a little improvement and 5 almost recovered.

CONCLUSIONThe PNS children can have neurological symptoms only at the onset and there were no particular evidence of tumor. It is prone to misdiagnosis. The prognosis of PNS in children was poor.

Adrenocorticotropic Hormone ; therapeutic use ; Biomarkers, Tumor ; analysis ; Brain ; diagnostic imaging ; pathology ; Child, Preschool ; Female ; Ganglioneuroma ; diagnosis ; pathology ; therapy ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Infant ; Magnetic Resonance Imaging ; Male ; Neuroblastoma ; diagnosis ; pathology ; therapy ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; pathology ; therapy ; Paraneoplastic Syndromes, Nervous System ; diagnosis ; pathology ; therapy ; Prognosis ; Radiography ; Retrospective Studies

OBJECTIVETo investigate the efficacy of combined modality therapy for neuroblastoma in children associated with opsoclonus-myoclonus syndrome (OMS-NB).

METHODFrom May 2011 to December 2013, 6 consecutive patients (4 boys and 2 girls) diagnosed as OMS-NB underwent surgery and chemotherapy in the First Hospital, Peking University. The median age of onset was 19.5 months (range 13-24 months) and misdiagnosis occurred 7.5 months (range 2-14 months) ago. A retrospective analysis for the location, stage, pathological type, treatment way and outcome of neuroblastoma was done.

RESULT(1) All patients were misdiagnosed as simply opsoclonus-myoclonus syndrome (OMS) at the time of onset. They had been receiving treatment with adrenocorticotropic hormone and intravenous immunoglobulin within 1-13 months.OMS-NB was diagnosed by means of enhanced abdominal CT image which was delayed to be given after the poor efficacy or relapse. (2) The primary tumors were almost all small, stage I-II, located in adrenal, retroperitoneal or pelvis. The pathology of tumors included ganglioneuroblastoma (5/6) and neuroblastoma (1/6). (3) All these cases underwent surgery, 4/6 cases with complete tumor resection, 2/6 cases with tumor around the aorta and induced local residue. Preoperative and postoperative chemotherapy was given to 2 and 5 cases, respectively. (4) The patients were followed up for 3-31 months, except 1 patient lost, the other 5 are currently surviving disease-free (3 having been at the end of chemotherapy, 1 still in chemotherapy, and another had local recurrence and is receiving radiotherapy and chemotherapy after the second operation and now also stopped taking the medicine). The symptoms of nervous system have been significantly improved during postoperative chemotherapy.

CONCLUSIONTo reduce the misdiagnosis, regular CT imaging of the abdomen or pelvic should be ordered for all cases with OMS. The children with OMS-NB need to be actively treated with the combined modality therapy including surgery, chemotherapy or radiotherapy, to reduce recurrence and reduce the symptoms of nervous system.

Adrenal Gland Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Antineoplastic Agents ; administration & dosage ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Biomarkers ; analysis ; Chemotherapy, Adjuvant ; Child, Preschool ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Neoplasm Recurrence, Local ; surgery ; therapy ; Neoplasm Staging ; Neuroblastoma ; complications ; diagnosis ; surgery ; therapy ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; etiology ; therapy ; Retroperitoneal Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome