One month following carbon monoxide poisoning, a 39 year-old man developed incontinence, memory impairment, disorientation and emotional instability. He was hospitalized 7weeks later, and during hospitalization he exhibited myoclonic movements of the neck and lower limbs. He was given piracetam intravenously for 11 days. The myoclonus was significantly reduced by the third day of treatment and had disappeared by the seventh day. There was no recurrence following cessation of treatment.
Adult ; Carbon Monoxide Poisoning/complications* ; Human ; Male ; Myoclonus/drug therapy ; Myoclonus/etiology* ; Piracetam/therapeutic use* ; Pyrrolidinones/therapeutic use*

Anticonvulsants ; therapeutic use ; Clonazepam ; therapeutic use ; Female ; Humans ; Levetiracetam ; therapeutic use ; MERRF Syndrome ; drug therapy ; Male ; Myoclonus ; drug therapy

There are a few case reports describing prolonged myoclonus following propofol. A previously fit 26 year old woman presented for a left knee lateral impingement under general anesthesia as a day case. She had no personal or family history of epilepsy. Induction was smooth and anesthesia was maintained with propofol TCI, nitrous oxide 65% and oxygen 35%. 30 minutes after stopping of the propofol infusion, the patient developed involuntary movement and shivering that recurred intermittently. After drug therapy and psychotic therapy the patient was progressively stabilized. However 4 hours later the patient developed involuntary movement and myoclonus. She was admitted to the department of neurology under the diagnosis of propriospinal myoclonus. Forty days later she was discharged without long term sequelae.
Adult ; Anesthesia* ; Anesthesia, General ; Diagnosis ; Drug Therapy ; Dyskinesias ; Epilepsy ; Female ; Humans ; Knee ; Myoclonus* ; Neurology ; Nitrous Oxide ; Oxygen ; Propofol* ; Shivering

Adult ; Anticonvulsants ; therapeutic use ; Brain ; drug effects ; pathology ; physiopathology ; Carbamazepine ; analogs & derivatives ; therapeutic use ; Electroencephalography ; Epilepsy, Frontal Lobe ; diagnosis ; drug therapy ; Female ; Humans ; Myoclonic Epilepsy, Juvenile ; diagnosis ; drug therapy ; Myoclonus ; diagnosis ; drug therapy

Two hundred and four patients with Parkinson's disease initially treated wth a combination of levodopa and carbidopa ( Sinement 25-250 ) and / or anticholinergic drugs. All patients responded initially to drug. Sixteen patients(7.8%) had 20 acute central nervous system side effects: 8, dyskinesia: 6, visual hallucination:5, psychosis: and 1, akathisia. The response to treatment usually was stable for the first one and a half to four years of drug therapy. Subsequently, over 50 percent of patients had therapeutic failure among 82 patients with long term drug therapy, fourteen(l7.0%) had 18 side effects: 8, on-off phenomenon: 4. Morning dystonia: 3, dyskinesia:and 3, simultaneous dyskinesia with parkinsonism. None had diphasic dyskinesia or myoclonus. The prognosis of the demented parkinsonian was relatively poor. Two patients died due to pneumonia and ovarian carcinoma.
Carbidopa ; Central Nervous System ; Drug Therapy ; Dyskinesias ; Dystonia ; Humans ; Levodopa ; Myoclonus ; Parkinson Disease* ; Parkinsonian Disorders ; Pneumonia ; Prognosis ; Psychomotor Agitation ; Psychotic Disorders

Glucose transport 1 (GLUT-1) deficiency is a rare syndrome caused by mutations in the glucose transporter 1 gene (SLC2A1) and is characterized by early-onset intractable epilepsy, delayed development, and movement disorder. De novo mutations and several hot spots in N34, G91, R126, R153, and R333 of exons 2, 3, 4, and 8 of SLC2A1 are associated with this condition. Seizures, one of the main clinical features of GLUT-1 deficiency, usually develop during infancy. Most patients experience brief and subtle myoclonic jerk and focal seizures that evolve into a mixture of different types of seizures, such as generalized tonic-clonic, absence, myoclonic, and complex partial seizures. Here, we describe the case of a patient with GLUT-1 deficiency who developed infantile spasms and showed delayed development at 6 months of age. She had intractable epilepsy despite receiving aggressive antiepileptic drug therapy, and underwent a metabolic workup. Cerebrospinal fluid (CSF) examination showed CSF-glucose-to-blood-glucose ratio of 0.38, with a normal lactate level. Bidirectional sequencing of SLC2A1 identified a missense mutation (c.1198C>T) at codon 400 (p.Arg400Cys) of exon 9.
Cerebrospinal Fluid ; Codon ; Drug Resistant Epilepsy ; Drug Therapy ; Exons* ; Glucose Transport Proteins, Facilitative ; Glucose Transporter Type 1 ; Glucose* ; Humans ; Infant ; Infant, Newborn ; Lactic Acid ; Movement Disorders ; Mutation, Missense ; Myoclonus ; Seizures ; Spasms, Infantile*

OBJECTIVEThe study was designed to examine the clinical and electroencephalographic characteristics of children with Jeavons syndrome.

METHODVideo-electroencephalography (VEEG) monitoring was carried out in 9 patients with Jeavons syndrome. The clinical and electroencephalographic characteristics, treatment and prognoses were analyzed.

RESULTOf the 9 patients, 8 were female, and 1 was male. The onset age of children with eyelid myoclonia (EM) was from 3 to 9 years old. It was obtained through the chief complaint, prosecution or VEEG monitoring. Three cases were misdiagnosed and 2 cases were overlooked initially. Seven out of 9 patients had generalized tonic clonic seizures (GTCS) during the course of disease, of whom 5 experienced only one episode. GTCS was the cause for the first visits to hospital in 5 patients. Since the clinical manifestations of EM with or without absence were often slight, VEEG monitoring with eye closure and intermittent photic stimulation tests helped to induce discharges and seizures. Eye closure was more potent than intermittent photic stimulation as a triggering factor. Ictal EEG showed 3 - 6 Hz generalized spike and waves and polyspikes burst. The main treatment option was valproate monotherapy (6 cases) or combined with other antiepileptic drugs (1 case). Levetiracetam, lamotrigine and topiramate were also used in patients and effective to some degree. Two patients lost follow up. The age of 7 patients at follow-up ranged from 9 y to 15 y. Seizures were controlled in 1 case, suspiciously controlled in 1 case, decreased in frequency in 4 cases and were still frequent in 1 case. During follow-up, normal intelligence was found in the former 2 cases, difficult learning in 2 cases, and slightly intellectual impairment in 2 cases.

CONCLUSIONJeavons syndrome is one of the idiopathic generalized epilepsies characterized by EM with or without absence. The age of seizure onset might be difficult to be exactly established, as EM was often misinterpreted and overlooked initially. Clinical history combined with VEEG monitoring with eye closure and intermittent photic stimulation tests could diagnose this disease. Valproate and other new antiepileptic drugs were effective for this disease. Jeavons syndrome is a lifelong disorder. Seizures sometimes could be well controlled. When seizures were resistant to treatment, cognitive and intellectual impairment might occur.

Adolescent ; Age of Onset ; Anticonvulsants ; administration & dosage ; therapeutic use ; Child ; Child, Preschool ; Electroencephalography ; Electromyography ; Epilepsies, Myoclonic ; diagnosis ; drug therapy ; physiopathology ; Epilepsy, Tonic-Clonic ; diagnosis ; drug therapy ; physiopathology ; Eyelids ; Female ; Follow-Up Studies ; Humans ; Male ; Myoclonus ; diagnosis ; drug therapy ; physiopathology ; Photic Stimulation ; methods ; Retrospective Studies ; Seizures ; physiopathology ; Syndrome ; Valproic Acid ; administration & dosage ; therapeutic use

Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis