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27 Possible Causes for Myoclonic Jerking, Severe Mental Retardation in Some Patients

  • Dravet Syndrome

    As the affected children grow up, they develop myoclonic jerking, and their seizures may become refractory to treatment. Synonym: severe myoclonic epilepsy of infancy[] In severe cases, patients may present with profound neurological deficit including hypotonia, seizures and mental retardation.[] Generalized ictal myoclonic jerks: The generalized ictal myoclonic jerks are always preceded by clear-cut spike-wave discharges.[]

  • Aicardi's Syndrome

    Asymmetric myoclonic jerks developed at one month of age.[] There is a wide range of severity in this disorder as only some patients have seizures, and, while some have significant mental retardation, others can function at a near[] Each patient exhibits some form of psychomotor impairment and varying degrees of mental retardation.[]

  • Lennox-Gastaut Syndrome

    In such cases, massive myoclonic jerks, myoclonic-atonic attacks, as well as atonic seizures may occur, though much less frequent and severe than the other manifestations.[] […] degree of mental retardation at the first visit, whereas 99% exhibited mental retardation at the final follow‐up visit. 12 Four independent risk factors for severe cognitive[] Lennox and Davis first correlated the slow spike-and-wave EEG pattern with a distinctive group of clinical manifestations including specific seizure types (myoclonic jerks[]

  • Rasmussen Syndrome

    However, the clinical history of myoclonic jerks was indicative of SSPE in our patient.[] Untreated, the disorder may lead to severe neurological deficits including mental retardation and paralysis. In some patients the surgery decreases the seizures.[] Juvenile Myoclonic Epilepsy People with juvenile myoclonic epilepsy (JME) have myoclonic seizures, characterized by small, rapid jerks of the arms, shoulders, or occasionally[]

  • Ataxia

    Patient 2 initially presented with an increased startle response and myoclonic jerks, and subsequently developed severe limb and gait ataxia, dysarthria, oculomotor disturbances[] […] have seizures and mild mental retardation.[] jerks.[]

  • Continuous Spike-and-Slow-Waves during Sleep

    Many children will have absence (blank) seizures, some myoclonic (jerk) seizures and others will have focal (partial) motor seizures (seizures involving jerks of one side[] Some patients remain mentally retarded even after the remission of the seizures. 45 – 47 Status epilepticus of BECTS SEBECTS refers to status epilepticus that can be convulsive[] .  Triad of  Polymorphic intractable seizure that are mainly tonic (80%), atonic (50%)and atypical absence seizures (70%), Myoclonic Jerks(11–28%).  Cognitive and behavioural[]

  • Congenital Muscular Dystrophy

    All patients suffered from convulsions, and two presented with myoclonic jerks. The head circumference developed normally with only one exception.[] […] calf hypertrophy, microcephaly, and mental retardation.[] mental retardation and epilepsy are extremely frequent.[]

  • D-Glyceric Aciduria

    jerks ,and progressing to apnea, and often to death.[] Some patients have an encephalopathic presentation, with severe mental retardation, seizures, microcephaly, and sometimes early death, whereas others have a mild phenotype[] Classification Classic neonatal form or classic form Most patients with GCE have the neonatal phenotype, presenting in the first few days of life with lethargy, hypotonia, and myoclonic[]

  • Mitochondrial DNA Depletion Syndrome Type 1

    Seizures may include epilepsia partialis continua, a type of seizure that consists of repeated myoclonic (muscle) jerks.[] MYTH An individual with mitochondrial disease has mental retardation, growth problems, and/or seizures. FACT Only some individuals have these developmental problems.[] Patients’ symptoms can range from extremely mild to severe, involve one or more body systems, and can emerge at any age.[]

  • Pitt-Hopkins Syndrome

    He receives sodium valproate and clobazam for the myoclonic jerks with satisfactory results.[] Mowat-Wilson syndrome resembles PTHS, and common features are severe mental retardation and some similarities in facial features.[] The authors have personal experience with an adult PTHS patient carrying a missense mutation who has myoclonic jerks occuring after falling asleep.[]

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