Status Dystonicus in a Child with Familial Idiopathic Hypoparathyroidism

Angurana, Suresh Kumar; Jayashree, Muralidharan; Dayal, Devi; Ismail, Javed

doi:10.1007/s12098-017-2295-3

Cited by 5 publications

(6 citation statements)

References 6 publications

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“…With the exception of decompensated calcium metabolism in hypoparathyroidism, the triggers recognized after 2012 have confirmed those previously reported, particularly the role of infection . With the expanding use of DBS for dystonia patients, more SD related to hardware failure have been reported: 3 children with DYT‐TOR1A and 1 case with tardive dystonia .…”

Section: Triggerssupporting

confidence: 69%

“…In 2012, the largest SD series was published: 68 patients undergoing 89 SD episodes allowed drawing some conclusion on the clinical expression, management strategies, and outcome of SD . During the past 5 years, isolated case reports and a small case series of SD have been published: a fatal refractory case in ataxia telangiectasia, 2 patients with Sex Determining Region Y‐box 2 (SOX2)‐Anophthalmia syndrome, a child with familial idiopathic hypoparathyroidism, 2 NBIA/DYT‐PANK2 patients, a chromosomopathy (deletion of the long arm of chromosome 22), a DYT/CHOR‐GCDH (glutaric aciduria type I), epileptic encephalopathy, idiopathic bilateral striatal necrosis, 3 idiopathic isolated dystonia, additional dyskinetic JCP patients, a 19‐year‐old boy affected by mental retardation and severe behavior disorder treated with high doses of haloperidol, tardive dystonia acutely worsened by the depletion of deep brain stimulation (DBS) implantable pulse generator, dystonia secondary to kernicterus, a series of 5 DYT‐TOR1A positive (DYT1) patients, and an isolated case report of a DYT‐TOR1A patient with SD as a result of implantable pulse generator depletion …”

Section: Historical Review and Current Definitionsmentioning

confidence: 99%

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Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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Status dystonicus is a movement disorder emergency that has been a source of controversy in terms of terminology, phenomenology, and management since it was first described in 1982. Here we argue that the current use of the term status dystonicus falls well short of the precision needed for either clinical or academic use. We performed a critical review on this topic, describing possible pathophysiological mechanisms and areas of uncertainties. This review also addresses the problems derived by the extreme clinical heterogeneity of this condition, as the lack of an objective criterion useful for the definition, or the fact that status dystonicus may present not only in the context of a known dystonic syndrome. We propose a new possible definition that includes not only dystonia but also other hyperkinetic movements in the wide range of movement disorders that can be seen during an episode. The new definition keeps the term status dystonicus and highlights the fact that this is a medical emergency based on the impairment of bulbar and/or respiratory function requiring hospital admission as the principal feature. Furthermore, the new definition should not consider as necessary unspecific features as patient's condition at baseline, the distribution of dystonia, occurrence of systemic symptoms such as fever or laboratory findings. We hope that this proposal will stimulate the debate on this subject among our peers, further developing a clinical and pathophysiological understanding of status dystonicus. © 2017 International Parkinson and Movement Disorder Society.

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Section: Triggerssupporting

confidence: 69%

Section: Historical Review and Current Definitionsmentioning

confidence: 99%

Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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“…Standard treatment is insufficient to prevent the elevation of calcium phosphorus product which increases the risk of posterior subcapsular cataracts as well as soft tissue and brain calcifications [1]. The consequences of basal ganglia and intracerebral calcifications such as extrapyramidal movement disorders and psychosis have been reported in some studies [1,2]. Additionally, elevations of calcium phosphorus product are associated with increased risks of cardiac arrhythmias and cardiovascular diseases in adults [1,7].…”

Section: Ectopic Calcificationsmentioning

confidence: 99%

“…Unlike adults, HPT in children is often symptomatic because of high skeletal requirements of calcium and is frequently diagnosed due to clinical symptoms of hypocalcemia such as muscle spasms, laryngospasm, seizures, stridor and tetany. Non-specific manifestations include cognitive and or motor delay, slowness, dystonia, and ungual, dental and skin anomalies [1,2]. Children with HPT suffer from several complications related to the disease itself or its treatment that include extracellular calcifications in the basal ganglia, posterior subcapsular cataracts, renal dysfunction, nephrocalcinosis, nephrolithiasis, and low bone turnover and increased bone density [1].…”

Section: Introductionmentioning

confidence: 99%

Recombinant parathyroid hormone for hypoparathyroidism in children: a narrative review

Dayal

Gupta

et al. 2019

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The conventional management of hypoparathyroidism in children involves the use of calcium and vitamin D analogs. This therapy effectively increases the serum calcium levels but worsens hypercalciuria and its consequences such as nephrocalcinosis and renal insufficiency. Although replacement with the missing parathyroid hormone (PTH) is ideal and available for more than 2 decades, the reported concerns of osteosarcoma prohibited its use in children with open epiphyses. Nevertheless, the data accumulated over the past several years suggests that the fears of bone malignancies were probably overstated. With an aim to review the available data on recombinant PTH (rhPTH) use, we performed a literature search using international databases and identified 15 studies involving approximately 70 children with hypoparathyroidism due to various etiologies who received rhPTH1-34 for durations between 1 day and 13.5 years. All the studies appear to indicate that rhPTH1-34 therapy is an effective short and long-term strategy for treatment of hypoparathyroidism with better metabolic control, lesser effects on renal function and improved quality of life as compared to conventional therapy. A more significant conclusion is the safety of long-term use of rhPTH1-34 with no observed adverse skeletal effects so far. However, all studies mention the importance of a continued surveillance for adverse effects in the treated patients. This narrative review discusses the experience of rhPTH1-34 use exclusively in children.

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“…A single sporadic case of SD in a child with familial idiopathic hypothyroidism has been reported from India. 7 There is no consensus definition of SD as to the number of episodes/severity of dystonia required to be categorized into SD. The common differentials include neuroleptic malignant syndrome, serotonin syndrome, malignant hyperthermia, paroxysmal autonomic instability with dystonia, and acute dystonic reactions.…”

Section: Discussionmentioning

confidence: 99%

Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome

Singh¹,

Aulakh²

2022

Indian Journal of Critical Care Medicine

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A bstract Dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contraction causing repetitive twisting movements and abnormal postures. Status dystonicus (SD) is an enigmatic disease of cryptic etiology. We hereby report a child with West syndrome (WS) who went on to develop SD following intramuscular adrenocorticotropic hormone (ACTH) injection. An 11-month-old male child presented with complaints of flexor spasms for 2 months. The diagnosis of WS was confirmed by electroencephalography (EEG), which showed hypsarrhythmia. Intramuscular ACTH was added, and oral trihexyphenidyl was started for dystonia. On day 7 of ACTH, the child developed frequent opisthotonic posturing. Management protocol for grade IV SD was initiated. Administration of N-terminal of ACTH in rat locus coeruleus has been shown to produce human dystonia-like movement and abnormal posturing. How to cite this article Singh J, Aulakh R. Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome. Indian J Crit Care Med 2022;26(8):961–962.

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Status Dystonicus in a Child with Familial Idiopathic Hypoparathyroidism

Cited by 5 publications

References 6 publications

Rethinking status dystonicus

Rethinking status dystonicus

Recombinant parathyroid hormone for hypoparathyroidism in children: a narrative review

Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome

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