The spectrum of movement disorders in children with anti‐<scp>NMDA</scp> receptor encephalitis

Baizabal‐Carvallo, José Fidel; Stocco, Amber; Muscal, Eyal; Jankovic, Joseph

doi:10.1002/mds.25354

Cited by 141 publications

(91 citation statements)

References 11 publications

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“…4 These are in contrast with the complex and severe orofacial or limb dyskinesias of anti-NMDAR encephalitis that are not mood-related and usually occur in the acute stage of the disease when most patients have other deficits or decreased level of consciousness (table). 5 Patients with anti-NMDAR encephalitis usually present with insomnia, not hypersomnia. If hypersomnia occurs, it is during the recovery phase, several months after symptom onset.…”

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confidence: 99%

When a serum test overrides the clinical assessment

2015

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The clinical features of narcolepsy-cataplexy are substantially different from anti-NMDA receptor (NMDAR) encephalitis. We report a patient whose diagnosis was established only after traditional clinical assessments prevailed over a presumably abnormal serum test result.Clinical case. A 10-year-old girl without a medical history of interest came for a second opinion regarding treatment of anti-NMDAR encephalitis. Her symptoms started 16 months earlier when she developed tiredness, irritability, episodes of falling asleep during the day, and lingual movements when doing enjoyable activities, such as playing. On 2 occasions, she had sudden falls without loss of consciousness. Blood chemistry, brain MRI, and EEG were normal. Six months after symptom onset, she was seen at a second institution, where a blood test using a live cell-based assay was reported as low positive for NMDAR antibodies. Routine CSF studies (antibodies not examined) were unremarkable. Based on this serum test, she was diagnosed with anti-NMDAR encephalitis, screened for an underlying tumor (abdominal ultrasound negative), and treated with steroids, IV immunoglobulin, and psychotherapy. Her symptoms did not improve, and she continued falling asleep at school and while playing. Nine months after symptom onset, she was seen at a third institution; repeat NMDAR antibody testing in the same laboratory was reported positive, and additional immunotherapy was recommended but not given. She continued with excessive daytime sleepiness, irritability, and mood-dependent lingual movements (video on the Neurology ® Web site at Neurology.org); however, she was maintaining her grades in school, although she required a 30-minute nap during the day and a 2-hour nap after school.At arrival to our center, 16 months after symptom onset, her family described, in addition to the above mentioned symptoms, episodes of floppy head without losing consciousness; nocturnal shouting, talking, and moving; and compulsive eating and a weight gain of 14 kg (over 97th percentile) that started before receiving steroids. At examination, the patient was attentive and collaborative but showed childish behavior inappropriate for her age, and rapid mood swings when asked about her symptoms. She did not exhibit memory, cognitive, or language dysfunction, and there was no evidence of myoclonus or dyskinesias during the visits.The polysomnogram revealed a total sleep time of 442 minutes, sleep efficiency 73%. Episodes of smile-like movements and bursts of EMG activity in the muscles of the face were noted during REM sleep. She had periodic leg movements during wakefulness and sleep, associated with arousals and vocalizations as if she was complaining (index of movements during sleep: 30). No sleep-disordered breathing was noted. A 5-nap multiple sleep latency test 1 showed 5 sleep-onset REM episodes, immediately after wakefulness (2 naps) or after a short stage N1 (3 naps), and a very short mean sleep latency (1 minute 24 seconds; normal .8 minutes). Human leukocyte antigen typing was DQ...

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confidence: 99%

When a serum test overrides the clinical assessment

2015

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“…7 More than one movement disorder may be present, which can make early diagnosis difficult and delay commencement of appropriate treatment. 8 Multiple movement disorders may be Downloaded by [University of California Santa Barbara] at 23:21 24 June 2016 seen in the same patient, and the movement disorder often evolves and changes with the disease course; thus, none of these movement disorders alone can be a guide to a specific auto-immune disease. 6 Diagnosis relies on excluding other causes of encephalitis and undertaking the antibody screen for the NMDA receptor in serum as well as CSF, which is usually positive.…”

Section: Discussionmentioning

confidence: 98%

Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports

Bashiri¹,

Al-Rasheed

Hassan³

et al. 2016

Paediatrics and International Child Health

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Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified auto-immune disorder characterised by severe memory deficit, a decreased level of consciousness, seizures, autonomic dysfunction and movement disorders. Three girls with the disorder are reported; they were aged 4 years, 5 years and 10 months. The 10-month-old infant who is one of the youngest patients reported with anti-NMDAR encephalitis worldwide, had MRI features suggestive of herpes simplex encephalitis (known to trigger anti-NMDAR encephalitis), but CSF PCR for herpes simplex was negative. All the patients presented with seizures, behavioural change, regression of speech, dystonia and choreo-athetosis. Anti-NMDAR antibodies were detected in all patients' sera and cerebrospinal fluid (CSF). Intravenous immunoglobulin, corticosteroids and rituximab were administered at different intervals. Cases 1 and 2 made a full recovery, but case 3 has mild motor and speech delay. Patients who present with encephalopathy, seizures and movement disorders should be tested for anti-NMDAR antibodies in serum and CSF in addition to being screened for herpes simplex encephalitis.

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“…Patients are often oblivious to these movements, which are absent during sleep [24]. Although TS have been well characterized in adults, they have not been extensively studied in children so clinicians evaluating children exposed to DRBAs must be vigilant in recognizing the early symptoms and signs of TS in this population in order to provide appropriate and timely interventions [27,28].…”

Section: Tardive Ticsmentioning

confidence: 99%

Identification and management of tardive dyskinesia: A case series and literature review

Khouzam

2015

Postgraduate Medicine

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Tardive dyskinesia (TD) is a serious, disabling and potentially permanent, neurological hyperkinetic movement disorder that occurs after months or years of taking dopamine receptor-blocking agents. The pathophysiology of TD is complex, multifactorial and still not fully understood. Although there is no identified effective and standard treatment for TD, several agents have been tried for the management of this motor disturbance. The aim of this case series is to review the literature in regard to the identification, diagnosis and the treatment of TD with anticholinergics, anticholinergic medication withdrawal, cholinergic agents, botulinum toxin intramuscular injections, tetrabenazine, levetiracetam, propranolol and zolpidem, and to describe one case of TD that responded favorably to clonazepam and two cases of TD that responded favorably to Ginkgo biloba.

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The spectrum of movement disorders in children with anti‐NMDA receptor encephalitis

Abstract: A wide variety of movement disorders, often in combination, can be observed in children with anti-NMDAR encephalitis. Patients commonly present with more than a single movement disorder.

Cited by 141 publications

References 11 publications

When a serum test overrides the clinical assessment

When a serum test overrides the clinical assessment

Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports

Identification and management of tardive dyskinesia: A case series and literature review

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