Cerebellar ataxia and opsoclonus as the initial manifestations of myoclonic encephalopathy associated with neuroblastoma. 1987

S Harel, and U Yurgenson, and G Rechavi, and Y Burstein, and Z Spirer
Pediatric Neurology Unit, Tel Aviv Medical Center, Sackler Medical School, University of Tel Aviv, Israel.

Cerebellar ataxia and opsoclonus were the initial manifestations of an associated neuroblastoma in a 20-month-old girl. Two months after the initial symptomatology, a physical examination revealed an abnormal mass palpable left to the midline. Urinary catecholamines were within normal limits. The child's neurological findings improved immediately after surgery, and steroid treatment and the follow-up on her after 2 years revealed normal general and neurological development. The syndrome of myoclonic encephalopathy including cerebellar ataxia, myoclonus and opsoclonus, and its relationship to neuroblastoma is reviewed. Failure to recognize this association can result in delays in both diagnosis and treatment and could be fatal.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D009207 Myoclonus Involuntary shock-like contractions, irregular in rhythm and amplitude, followed by relaxation, of a muscle or a group of muscles. This condition may be a feature of some CENTRAL NERVOUS SYSTEM DISEASES; (e.g., EPILEPSY, MYOCLONIC). Nocturnal myoclonus is the principal feature of the NOCTURNAL MYOCLONUS SYNDROME. (From Adams et al., Principles of Neurology, 6th ed, pp102-3). Myoclonus, Action,Myoclonus, Nocturnal,Myoclonus, Palatal,Polymyoclonus,Myoclonic Jerk,Myoclonic Jerking,Myoclonus Simplex,Myoclonus, Eyelid,Myoclonus, Intention,Myoclonus, Lower Extremity,Myoclonus, Oculopalatal,Myoclonus, Segmental,Myoclonus, Sleep,Myoclonus, Upper Extremity,Action Myoclonus,Extremity Myoclonus, Lower,Extremity Myoclonus, Upper,Eyelid Myoclonus,Intention Myoclonus,Jerk, Myoclonic,Jerking, Myoclonic,Jerks, Myoclonic,Lower Extremity Myoclonus,Myoclonic Jerks,Nocturnal Myoclonus,Oculopalatal Myoclonus,Palatal Myoclonus,Segmental Myoclonus,Simplex, Myoclonus,Sleep Myoclonus,Upper Extremity Myoclonus
D009447 Neuroblastoma A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51) Neuroblastomas
D009759 Nystagmus, Pathologic Involuntary movements of the eye that are divided into two types, jerk and pendular. Jerk nystagmus has a slow phase in one direction followed by a corrective fast phase in the opposite direction, and is usually caused by central or peripheral vestibular dysfunction. Pendular nystagmus features oscillations that are of equal velocity in both directions and this condition is often associated with visual loss early in life. (Adams et al., Principles of Neurology, 6th ed, p272) Convergence Nystagmus,Horizontal Nystagmus,Jerk Nystagmus,Pendular Nystagmus,Periodic Alternating Nystagmus,Rotary Nystagmus,See-Saw Nystagmus,Vertical Nystagmus,Conjugate Nystagmus,Dissociated Nystagmus,Fatigable Positional Nystagmus,Multidirectional Nystagmus,Non-Fatigable Positional Nystagmus,Permanent Nystagmus,Rebound Nystagmus,Retraction Nystagmus,Rotational Nystagmus,Spontaneous Ocular Nystagmus,Symptomatic Nystagmus,Temporary Nystagmus,Unidirectional Nystagmus,Non Fatigable Positional Nystagmus,Nystagmus, Conjugate,Nystagmus, Convergence,Nystagmus, Dissociated,Nystagmus, Fatigable Positional,Nystagmus, Horizontal,Nystagmus, Jerk,Nystagmus, Multidirectional,Nystagmus, Non-Fatigable Positional,Nystagmus, Pendular,Nystagmus, Periodic Alternating,Nystagmus, Permanent,Nystagmus, Rebound,Nystagmus, Retraction,Nystagmus, Rotary,Nystagmus, Rotational,Nystagmus, See-Saw,Nystagmus, Spontaneous Ocular,Nystagmus, Symptomatic,Nystagmus, Temporary,Nystagmus, Unidirectional,Nystagmus, Vertical,Ocular Nystagmus, Spontaneous,Pathologic Nystagmus,Positional Nystagmus, Non-Fatigable,See Saw Nystagmus
D002524 Cerebellar Ataxia Incoordination of voluntary movements that occur as a manifestation of CEREBELLAR DISEASES. Characteristic features include a tendency for limb movements to overshoot or undershoot a target (dysmetria), a tremor that occurs during attempted movements (intention TREMOR), impaired force and rhythm of diadochokinesis (rapidly alternating movements), and GAIT ATAXIA. (From Adams et al., Principles of Neurology, 6th ed, p90) Adiadochokinesis,Ataxia, Cerebellar,Cerebellar Dysmetria,Dysmetria,Cerebellar Hemiataxia,Cerebellar Incoordination,Hypermetria,Adiadochokineses,Ataxias, Cerebellar,Cerebellar Ataxias,Cerebellar Dysmetrias,Cerebellar Hemiataxias,Cerebellar Incoordinations,Dysmetria, Cerebellar,Dysmetrias,Dysmetrias, Cerebellar,Hemiataxia, Cerebellar,Hemiataxias, Cerebellar,Hypermetrias,Incoordination, Cerebellar,Incoordinations, Cerebellar
D005133 Eye Movements Voluntary or reflex-controlled movements of the eye. Eye Movement,Movement, Eye,Movements, Eye
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000008 Abdominal Neoplasms New abnormal growth of tissue in the ABDOMEN. Abdominal Neoplasm,Neoplasm, Abdominal,Neoplasms, Abdominal

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