Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mental status changes are second only to headaches as a cause of neurologic consultation in children with systemic cancer, but the literature on these patients is sparse. This study consisted of a review of the consultations because of changes in mental status in patients with pediatric cancer, with analysis of clinical presentation, etiology, underlying cancer, and neuroradiologic findings. Hematologic cancers were the underlying disorder in slightly more than one half of the patients. The majority of children suffered from iatrogenically induced encephalopathy, predominantly opioid-related. Several drugs were associated with depressed sensorium, but a pure metabolic deficit was rare. One third of the patients had multiple causative factors. Neuroimaging studies were particularly helpful in children with unexplained somnolence but were less useful if the patient had hallucinations. The absence of localizing signs in children with unexplained somnolence or stupor did not exclude the presence of structural disease. Altered mental status is frequently observed in children being actively treated for systemic cancer. The majority of children suffer from iatrogenically induced encephalopathy, predominantly opioid-related. The presence of hallucinations favors a toxic-metabolic dysfunction, especially if associated with myoclonus. Under those circumstances a neuroimaging study is usually unrewarding. If the main complaint is unexplained somnolence without hallucinations or delusions, the performance of a brain imaging study is mandatory, even in the absence of localizing signs.
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PMID:Mental status changes in children with systemic cancer. 1216 Sep 72

Atonic seizures are part of some childhood epilepsy syndromes and can also result from acquired central nervous system insult. Of 93 survivors of childhood cancer with uncontrolled seizures, 10 (11%) with atonic seizures form the basis for this report. Seven survived acute leukemia, and three survived brain tumors. The median age at cancer diagnosis was 9 months (range 2-52). Nine received systemic and six intrathecal chemotherapy; cranial irradiation was given to seven. All have learning difficulties, abnormal brain magnetic resonance images, and an abnormal electroencephalogram; six are developmentally delayed. All have difficult-to-control seizures, but six improved on myoclonus-specific drugs accelerated development in two younger patients. Atonic seizures in cancer survivors are associated with difficult-to-control seizures and pervasive cognitive impairments. Young age at cancer diagnosis and cranial irradiation may predispose the patient to development of this syndrome. Recognition and appropriate treatment of atonic seizures in cancer survivors could improve seizure control, neurologic development, and quality of life.
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PMID:Atonic seizures in survivors of childhood cancer. 1288 74

Paraneoplastic neurologic syndromes are rare disorders that have potentially devastating effects on the developing brain. Recently, there has been increased interest in possible immunotherapy for these disorders. Recognition of paraneoplastic syndromes in children may lead to early detection and treatment of the pediatric cancer and may diminish the neurologic damage that is the major source of morbidity in children with successfully treated tumors. This article reviews the presenting symptoms, immunology, long-term sequelae, and management options for paraneoplastic neurologic syndromes, focusing on those most commonly reported in children: opsoclonus-myoclonus ataxia, limbic encephalitis, and anti-NMDAR encephalitis. The child neurologist plays an important role in recognizing these disorders, initiating a tumor search, and directing ongoing treatment and management of neurologic symptoms after oncologic treatment is complete. Given the rarity of these conditions, multisite collaborative efforts are needed to develop standardized approaches to characterization and treatment.
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PMID:Paraneoplastic neurologic disorders in children. 2116 3

Although cancer in children is rare, it is the second most common cause of childhood mortality in developed countries. It often presents with nonspecific symptoms similar to those of benign conditions, leading to delays in the diagnosis and initiation of appropriate treatment. Primary care physicians should have a raised index of suspicion and explore the possibility of cancer in children who have worrisome or persisting signs and symptoms. Red flag signs for leukemia or lymphoma include unexplained and protracted pallor, malaise, fever, anorexia, weight loss, lymphadenopathy, hemorrhagic diathesis, and hepatosplenomegaly. New onset or persistent morning headaches associated with vomiting, neurologic symptoms, or back pain should raise concern for tumors of the central nervous system. Palpable masses in the abdomen or soft tissues, and persistent bone pain that awakens the child are red flags for abdominal, soft tissue, and bone tumors. Leukokoria is a red flag for retinoblastoma. Endocrine symptoms such as growth arrest, diabetes insipidus, and precocious or delayed puberty may be signs of endocranial or germ cell tumors. Paraneoplastic manifestations such as opsoclonus-myoclonus syndrome, rheumatic symptoms, or hypertension are rare and may be related to neuroblastoma, leukemia, or Wilms tumor, respectively. Increased suspicion is also warranted for conditions associated with a higher risk of childhood cancer, including immunodeficiency syndromes and previous malignancies, as well as with certain genetic conditions and familial cancer syndromes such as Down syndrome, Li-Fraumeni syndrome, hemihypertrophy, neurofibromatosis, and retinoblastoma.
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PMID:Signs and symptoms of childhood cancer: a guide for early recognition. 2393 97

Neuroblastoma is a childhood cancer derived from cells of neural crest origin. The hallmarks of its enigmatic character include its propensity for spontaneous regression under some circumstances and its association with paraneoplastic opsoclonus, myoclonus, and ataxia. The neurodevelopmental underpinnings of its origins may provide important clues for development of novel therapeutic and preventive agents for this frequently fatal malignancy and for the associated paraneoplastic syndromes. Ann Neurol 2016;80:13-23.
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PMID:The "neuro" of neuroblastoma: Neuroblastoma as a neurodevelopmental disorder. 2704 43