Delayed-onset Dystonia Research Articles

A rare presentation of systemic complication of hypoxic ischemic encephalopathy in post cochlear implant child

<p class="abstract">Cochlear implant is a small, complex electronic device to restore some hearing in profoundly deaf or severely hard-of hearing people when organ of corti is not developed or destroyed by injury to such an extent that no hearing can be obtained by hearing aids. Many classifications have been proposed for cochlear implant related problems .We report a case of post cochlear implant child who reported to OPD with complaint of progressive weakness of both upper and lower limb which developed 1 year after surgery ,which parents attributed it as a complication of surgery. On retrospective analysis all patient preop investigations were normal. After detailed examination of the child, neurophysician concluded that child is suffering from global developmental delay with dystonic cerebral palsy. MRI brain and cochlea which was done before the surgery which showed T1 hypointense,T2 and FLAIR hyperintense ,bilateral symmetrical foci with high ADC values are seen in bilateral lentiform nuclei .It has been reported that the bithalamic hyperdensities on CT and/or MRI in severely asphyxiated neonates, were suggested of a distinctive pattern of brain injury which might be useful as an early predictor of status marmoratus,afrequent pathological correlate of dyskinetic cerebral palsy. Approximately 1% of infants who survived perinatal asphyxial HIE would develop delayed-onset dystonia in the course of 7 to 13 years (median 10 years) after birth.</p>

Delayed‐onset dystonia due to perinatal asphyxia: A prospective study

The objective of this work was to establish the existence and incidence of possible delayed-onset dystonia in a cohort of infants with diagnosed perinatal asphyxial hypoxic-ischemic encephalopathy (HIE). This prospective study comprised 103 survivors of perinatal asphyxial HIE, who were regularly followed and neurologically examined in the course of 7 to 13 years after birth (median 10 years). Neurological outcome at the end of the follow-up period was normal in 87 (84.5%) patients, while in 7 (6.8%) only mild neurological signs were detected (behavioral disturbances in 3, clumsiness in 2, and hypotonia in 1 patient). Severe cerebral palsy was diagnosed in nine patients (8.7%). Only one patient was diagnosed with possible delayed-onset segmental dystonia. At the age of 4 years he developed cervical dystonia with spread to one arm in the course of 1.5 years (segmental dystonia) and then stabilized. Other known causes of dystonia, including a DYT1 mutation, were excluded. Our preliminary data suggest that over the course of at least 7 years after birth, approximately 1% of infants who survived perinatal asphyxial HIE would develop delayed-onset dystonia.

Delayed-onset dystonia in patients with antecedents of perinatal asphyxia

We report 27 cases of delayed-onset dystonia in patients with antecendents of perinatal asphyxia after excluding other possible causes of dystonia. The patients were 16 males and 11 females (mean +/- SD age at onset of dystonia = 13.0 +/- 9.4 yr). Adverse obstetric and/or perinatal events were presented by 20 patients, and other neurological symptoms or signs were present prior to dystonia in 15 patients. The pattern of dystonia was: generalized (eight cases), segmental (six), multifocal (three), hemidystonia (two), and focal (eight). Cranial CT or MRI were normal in 21 cases, and showed brain hemiatrophy in three cases, and periventricular demyelination, subcortical atrophy and increased ventricular size in one case each. The possible cause-effect relationship is discussed.

Delayed‐onset dystonia

Delayed‐onset dystonia

Delayed‐onset dystonia

Delayed‐onset dystonia

Dystonia Due to Perinatal Asphyxia

Delayed onset dystonia associated with perinatal asphyxia in two patients and asphyxia in early childhood in two patients is reported from the Neurological Institute of New York.

Delayed‐onset dystonia due to perinatal or early childhood asphyxia

We report 10 patients with delayed-onset dystonia associated with perinatal asphyxia and 2 associated with asphyxia in childhood. In the perinatal group, the mean age of onset was 12.9 years. Among these patients, dystonia continued to progress for a mean of 7 years, and as long as 28 years. These patients had moderate motor disability; none was wheelchair-bound, and thus their prognosis was better than that of the childhood-onset idiopathic torsion dystonias. The most frequently beneficial drugs were anticholinergics. Since some of these patients closely resembled cases of idiopathic torsion dystonia, the prior occurrence of asphyxia should be used as a criterion of exclusion for that diagnosis.

Multiple Sclerosis and Rheumatoid Arthritis

Eight cases of persistent dystonia appearing one to 14 years after non-progressive cerebral insults are described. Five were due to perinatal anoxia, one to trauma, and two to cerebral infarction. This phenomemon of delayed-onset dystonia has not been described previously, although review of earlier literature reveals several probable examples. Delayed-onset dystonia due to perinatal anoxia is an important diagnostic alternative to dystonia musculorum deformans for dystonia occurring in childhood.