Muscle magnetic resonance imaging abnormality in neuroleptic malignant syndrome: a case report.

BACKGROUND: Neuroleptic malignant syndrome (NMS) is a rare and occasionally fatal undesirable reaction to dopamine antagonists, and its phenotype is diverse owing to causative drugs. Classically, elevation of serum creatine kinase is described in NMS. Some reports have described muscular pathological findings; however, muscle magnetic resonance imaging (MRI) has not been reported previously. CASE PRESENTATION: A 63-year-old woman with a history of schizophrenia presented to our hospital with a high fever, excessive sweating, muscle weakness, and elevated serum creatine kinase levels. Muscle MRI revealed T2 high-intensity lesions in several muscles with gadolinium enhancement, and the pathology of the muscle biopsy showed a very mild presence of muscle fiber necrosis and regeneration with type 2c fibers without inflammation. Her symptoms resolved by treatment with levodopa/carbidopa, dantrolene. Finally, the patient was diagnosed with NMS. CONCLUSIONS: This is the first report of muscle MRI abnormalities in a patient with NMS. Muscle MRI abnormalities in NMS may be associated with non-inflammatory myopathic changes. The cause of creatine kinase elevation cannot be explained by abnormal strong muscle contraction nor inflammation.

Síndrome neuroléptico maligno en la urgencia pediátrica / Neuroleptic malignant syndrome in pediatrics. A case report

El síndrome neuroléptico maligno es una urgencia pediátrica con una elevada morbimortalidad, relacionada con alteración de sistema de neurotransmisión dopaminérgico. Se caracteriza por hipertermia junto con hipertonía muscular, alteración autonómica y de los niveles de conciencia. Un diagnóstico precoz es imprescindible para prevenir complicaciones comunes como la broncoaspiración, desgaste, escaras, procesos infecciosos y cambios neuropsiquiátricos. El tratamiento debe incluir en medidas generales de soporte y terapéutica farmacológica sintomática. Pese a que la mayoría de los casos descritos corresponden a población adulta, también se ha descrito en niños y adolescentes. Presentamos un caso de síndrome neuroléptico maligno en un adolescente de 12 años con encefalopatía y tetraparesia espática secundario al cese de la administración de baclofeno

Neuroleptic malignant syndrome is a pediatric emergency with high morbidity and mortality, related to an alteration of the dopaminergic neurotransmission system. It is characterized by hyperthermia along with muscular hypertonia, dysautonomia, and altered level of consciousness. An early diagnosis is essential to prevent common complications such as bronchoaspiration, wear, bedsores, infectious processes, and neuropsychiatric changes. Treatment should include general support measures and symptomatic pharmacological therapy. Although most of the cases described correspond to the adulthood, it has also been described in children and adolescents. We present a case of neuroleptic malignant syndrome in a 12-year-old adolescent with encephalopathy and spastic tetraparesis secondary to the cessation of baclofen administration

Subcortical Structure Disruption in Diffusion Tensor Tractography of the Patient With the Syndrome of Irreversible Lithium-Effectuated Neurotoxicity Combined With Neuroleptic Malignant Syndrome: A Case Report.

BACKGROUND: Lithium can cause not only acute neurotoxicity but also chronic and persistent neurotoxicity known as syndrome of irreversible lithium-effectuated neurotoxicity (SILENT). The combined use of lithium and antipsychotics increases the possibility of SILENT. Neuroleptic malignant syndrome (NMS) is a reversible, idiosyncratic, and potentially life-threatening reaction, which is usually caused by antipsychotics and other agents, such as mood stabilizers (eg, lithium and metoclopramide). Neuroleptic malignant syndrome is characterized by hyperpyrexia, muscle rigidity, and altered mental status. We describe a case of SILENT combined with NMS in this case report. CASE REPORT: A 46-year-old man who had been treated with lithium for bipolar II disorder since 2008 was prescribed lorazepam, lithium, and aripiprazole at his last outpatient visit. The patient experienced financial difficulties (bankruptcy) and suffered severe emotional stress. Subsequently, he overused lorazepam, lithium, and aripiprazole. Two days after the overdose, he experienced a high fever, confused mental status, and rhabdomyolysis and was diagnosed with NMS. However, even after resolution of NMS-related symptoms, quadriplegia, visual field defects, ataxia, and severe dysarthria persisted. A positron emission tomography-computed tomography brain scan showed decreased 15F-fludeoxyglucose uptake in bilateral primary motor cortices and in the thalamus, midbrain, and cerebellum. Brain magnetic resonance imaging diffusion tensor imaging and diffusion tensor tractography of the subcortical tracts revealed structural disruptions, especially in the corticospinal tract, dentatorubrothalamic tract, and optic radiation, which seemed to be correlated with the clinical symptoms of the patient. CONCLUSION: This case suggests that the clinical use of diffusion tensor tractography could be helpful to explain the clinical features in the case of SILENT combined with NMS.

Neuroleptic malignant syndrome as a presenting feature of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a slowly progressive degenerative disorder caused by measles virus. It is characterised by typical clinical and electrophysiological features in the form of slow myoclonic jerks, with progressive cognitive impairment, visual symptoms, and periodic complexes on EEG, with raised titres of anti-measles antibodies in CSF and serum. Atypical presentations of SSPE have been reported including brainstem involvement, ADEM-like presentation, acute encephalitis, and cerebellar ataxia. Presentation with predominant extrapyramidal features is uncommon. We describe a case of SSPE presenting with extensive rigidity with highly elevated CPK values, mimicking neuroleptic malignant syndrome (NMS) which was most probably due to central dopaminergic blockade induced by the disease process. To our knowledge, this is the first case of SSPE presenting with a NMS-like syndrome.

A Reversible Isolated Lesion in the Splenium of Corpus Callosum in a Patient with Probable Neuroleptic Malignant Syndrome -- Case Report.

PURPOSE: A reversible isolated lesion in the splenium of the corpus callosum (SCC) is rare. We present such a case in a young female patient with neuroleptic malignant syndrome (NMS) and elaborate on its proposed pathophysiology and the possible differential diagnoses. CASE REPORT: A 28-year-old female was on neuropsychiatric treatment (clozapine) for schizoaffective disorder. NMS was diagnosed based on the clinical presentation of fever, mental status change (with disorientation and visual hallucination), generalized muscular rigidity (catatonic signs), tremor, and markedly increased creatine phosphokinase (1824 U/l) after 10-day administration of clozapine. The SCC lesion had a "boomerang" appearance and high signal intensity on the initial T2-weighted, T2 fluid attenuated inversion recovery, and diffusion-weighted magnetic resonance images, and decreased apparent diffusion coefficient values. The follow-up magnetic resonance imaging 12 weeks later showed complete resolution of the SCC lesion. CONCLUSION: A reversible isolated SCC lesion is a distinct clinicoradiological syndrome of varied etiology. The changes may occur in certain psychiatric patients with NMS and most patients with epilepsy and encephalitis. The etiological mechanism remains uncertain and enigmatic, but the neurological course and outcome are good.

Neuroleptic malignant syndrome and methylphenidate.

A 1-year-old female presented with neuroleptic malignant syndrome probably caused by methylphenidate. She had defects in the supratentorial brain including the basal ganglia and the striatum (multicystic encephalomalacia) due to severe perinatal hypoxic-ischemic encephalopathy, which was considered to be a possible predisposing factor causing neuroleptic malignant syndrome. A dopaminergic blockade mechanism generally is accepted as the pathogenesis of this syndrome. However, methylphenidate is a dopamine agonist via the inhibition of uptake of dopamine, and therefore dopaminergic systems in the brainstem (mainly the midbrain) and the spinal cord were unlikely to participate in the onset of this syndrome. A relative gamma-aminobutyric acid-ergic deficiency might occur because diazepam, a gamma-aminobutyric acid-mimetic agent, was strikingly effective. This is the first reported patient with neuroleptic malignant syndrome probably caused by methylphenidate.

Imaging of dopamine receptors with [123I]iodobenzamide single-photon emission-computed tomography in neuroleptic malignant syndrome.

With the tracer [123I]iodobenzamide ([123I]-IBZM), it is possible to image dopamine receptor occupancy with single-photon emission-computed tomography (SPECT). We report follow-up examinations with IBZM-SPECT in neuroleptic malignant syndrome (NMS) to display D2-receptor availability in the acute phase and during the course of remission. A 27-year-old man was admitted with severe akinesia, rigor, tachycardia, fever, and elevated creatine phosphokinase level (CK) after neuroleptic medication. NMS was diagnosed, and treatment was started with dantrolene, amantadine, and dopamine agonists. IBZM-SPECT examination was performed on days 6, 34, 90, 107, 131, and 201. In the acute state of NMS, there was no binding of IBZM to D2-receptors. SPECT reached almost normal values on day 131, but clinical examination still showed a mild parkinsonian syndrome. With SPECT, the D2-receptor occupancy in NMS could be successfully shown in correlation with extrapyramidal signs. IBZM-SPECT may therefore serve to monitor D2-receptor occupancy in patients at risk for NMS.

Single photon emission computed tomography with 123I-IMP in three cases of the neuroleptic malignant syndrome.

Single photon emission computed tomography (SPECT) perfusion brain scans using 123I-N-isopropyl-p-iodoamphetamine (123I-IMP) were performed in three patients with the neuroleptic malignant syndrome (NMS). In two accumulation was increased in the left basal ganglia and decreased in the right on the early images during the active phase of NMS; this asymmetry was not seen after recovery. In the third patient two examinations were performed during the active phase; on the first, increased accumulation of 123I-IMP in the left basal ganglia was found on the early images, but on the second, increased accumulation of tracer was found in the right basal ganglia on the delayed images. These abnormalities disappeared after improvement of the NMS. These results suggest that a disturbance in the basal ganglia is related to the development of NMS.

Positron emission tomographic studies of changes in cerebral blood flow and oxygen metabolism in neuroleptic malignant syndrome.

Three patients with neuroleptic malignant syndrome were studied by positron emission tomography (PET), using the steady-state technique with C15O2 and 15O2. In 2 patients the PET examination was repeated after resolution of the syndrome. In 2 patients, under treatment with bromocriptine, high blood flow and oxygen metabolism were demonstrated in the striatum, cerebellum and occipital cerebral cortex during the neuroleptic malignant phase, showing that not only the dopaminergic system is disturbed but also other neurotransmitter systems are involved. In the 3rd case the PET scan findings were not as conclusive.

Cerebral infarction in neuroleptic malignant syndrome.

Two young women with no risk factors for cerebrovascular disease developed hyperpyrexia, rigidity, and autonomic features while taking neuroleptic agents. The first presented with increasing rigidity, profuse diaphoresis and dehydration, and a right hemiparesis, and computed tomography (CT) showed a left striato-capsular infarction. The second became unresponsive following severe hypoxia and was found to have left-sided pyramidal signs three days later. Hemoconcentration and hypoxemia predispose to cerebral ischemic injury in the neuroleptic malignant syndrome and should be avoided as much as possible.