Caspr2 antibodies in limbic encephalitis with cerebellar ataxia, dyskinesias and myoclonus

Balint, Bettina; Regula, Jens Ulrich; Jarius, Sven; Wildemann, Brigitte

doi:10.1016/j.jns.2013.01.040

Cited by 34 publications

(20 citation statements)

References 9 publications

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“…Encephalitides HSV (Park et al, 2011) EBV West Nile virus (Birlutiu and Birlutiu, 2014;Cooper and Said, 2014) SSPE (Sharma and Biswas, 2013;Shibasaki and Hallett, 2005) Mumps infections (Kang and Kim, 2014) Autoimmune (Balint et al, 2013;DeFelipe-Mimbrera et al, 2014;Ghoreishi et al, 2013;Leigh et al, 1980;Smith et al, 2011;Stern et al, 2014;Uehara et al, 2011) Paraneoplastic (Berger and Mehari, 1999;Kim et al, 2009;Kumar et al, 2005; Spinal cord Traumatic spinal cord injury Calancie (2006) individual has fallen, and it lasts for 1-16 s. The recognition of myoclonus associated with transient cerebral hypoxia is important in the context of critically ill patients, as during intensive care, patients can suddenly develop episodes of severe arterial hypotension that (if detected early) can be treated with vasopressors to avoid hypoxic brain injury. Distinguishing psychogenic from somatic myoclonus is challenging calling for the exclusion of somatic diseases.…”

Section: Metabolic Encephalopathiesmentioning

confidence: 99%

Myoclonus in the critically ill: Diagnosis, management, and clinical impact

Sutter

Ristic

Rüegg

et al. 2016

Clinical Neurophysiology

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Myoclonus is the second most common involuntary non-epileptic movement in intensive care units following tremor-like gestures. Although there are several types of myoclonus, they remain underappreciated, and their diagnostic and prognostic associations are largely ignored. This review discusses clinical, electrophysiological, neuroanatomical, and neuroimaging characteristics of different types of myoclonus in critically ill adults along with their prognostic impact and treatment options. Myoclonus is characterized by a sudden, brief, and sometimes repetitive muscle contraction of body parts, or a brief and sudden cessation of tonic muscle innervation followed by a rapid recovery of tonus. Myoclonus can resemble physiologic and other pathologic involuntary movements. Neurologic injuries, anesthetics, and muscle relaxants interfere with the typical appearance of myoclonus. Identifying "real myoclonus" and determining the neuroanatomical origin are important, as treatment responses depend on the involved neuroanatomical structures. The identification of the type of myoclonus, the involved neuroanatomical structures, and the associated illnesses is essential to direct treatment. In conclusion, the combined clinical, electrophysiological, and neuroradiological examination reliably uncovers the neuroanatomical sources and the pathophysiology of myoclonus. Recognizing cortical myoclonus is critical, as it is treatable and may progress to generalized convulsive seizures or status epilepticus.

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Section: Metabolic Encephalopathiesmentioning

confidence: 99%

Myoclonus in the critically ill: Diagnosis, management, and clinical impact

Sutter

Ristic

Rüegg

et al. 2016

Clinical Neurophysiology

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“…9 Although permanent ataxia without remissions is a well-described feature of anti–CASPR2 antibody-associated encephalitis, to our knowledge, EA has never been reported before in patients with autoimmune encephalitis, including for instance patients with anti-Lgi1 encephalitis. 4,3,10,11 …”

Section: Discussionmentioning

confidence: 99%

Autoimmune episodic ataxia in patients with anti-CASPR2 antibody-associated encephalitis

Joubert

Gobert

Thomas

et al. 2017

Neurol Neuroimmunol Neuroinflamm

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Objective:To report paroxysmal episodes of cerebellar ataxia in a patient with anti–contactin-associated protein-like 2 (CASPR2) antibody-related autoimmune encephalitis and to search for similar paroxysmal ataxia in a cohort of patients with anti–CASPR2 antibody-associated autoimmune encephalitis.Methods:We report a patient with paroxysmal episodes of cerebellar ataxia observed during autoimmune encephalitis with anti-CASPR2 antibodies. In addition, clinical analysis was performed in a retrospective cohort of 37 patients with anti-CASPR2 antibodies to search for transient episodes of ataxia. Paroxysmal symptoms were further specified from the referral physicians, the patients, or their relatives.Results:A 61-year-old man with limbic encephalitis and anti-CASPR2 antibodies developed stereotyped paroxysmal episodes of cerebellar ataxia, including gait imbalance, dysarthria, and dysmetria, 1 month after the onset of the encephalitis. The ataxic episodes were specifically triggered by orthostatism and emotions. Both limbic symptoms and transient ataxic episodes resolved after treatment with steroids and IV cyclophosphamide. Among 37 other patients with anti-CASPR2 antibodies, we identified 5 additional cases with similar paroxysmal ataxic episodes that included gait imbalance (5 cases), slurred speech (3 cases), limb dysmetria (3 cases), and nystagmus (1 case). All had concomitant limbic encephalitis. Paroxysmal ataxia was not observed in patients with neuromyotonia or Morvan syndrome. Triggering factors (orthostatism or anger) were reported in 4 patients. Episodes resolved with immunomodulatory treatments in 4 patients and spontaneously in 1 case.Conclusions:Paroxysmal cerebellar ataxia must be added to the spectrum of the anti-CASPR2 antibody syndrome.

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“…Recently, the clinical spectrum of anti-Caspr2 antibody-related encephalitis has been broadened considerably from limbic encephalitis; 10% of patients with idiopathic cerebellar ataxia were positive for this antibody [6]. Moreover, a case report described a patient with ataxia, myoclonus, and dyskinesia in addition to symptoms of limbic encephalitis [7]. Despite such a broadened clinical spectrum, a recent large clinicoserologic study demonstrated that extrapyramidal signs, including tremor, parkinsonism, and chorea, were not common in this disease as they were observed in only 1 patient (about 3%) [8].…”

Section: Discussionmentioning

confidence: 99%

Eyelid Tremor in a Patient with Anti-Caspr2 Antibody-Related Encephalitis

Ueno¹,

Hirano²,

Sakamoto³

et al. 2014

Case Rep Neurol

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We describe the first case of a patient with eyelid tremor probably associated with anti-contactin-associated protein-like 2 (Caspr2) antibody. Encephalitis associated with anti-voltage-gated potassium channel antibody is now attributed to autoantibodies against leucine-rich glioma inactivated 1 (Lgi1) and less frequently against Caspr2. Eyelid tremor or blepharoclonus is a rare or underdiagnosed involuntary movement that has been found in patients with infarction in the thalamus or drug-induced or idiopathic parkinsonism. Since patients with anti-Caspr2 antibody-related encephalitis occasionally have extrapyramidal signs, we speculate that the eyelid tremor was also caused by anti-Caspr2 antibody in our patient. Partial resolution of his symptoms by plasmapheresis also supported the involvement in immunological processes.

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Caspr2 antibodies in limbic encephalitis with cerebellar ataxia, dyskinesias and myoclonus

Cited by 34 publications

References 9 publications

Myoclonus in the critically ill: Diagnosis, management, and clinical impact

Myoclonus in the critically ill: Diagnosis, management, and clinical impact

Autoimmune episodic ataxia in patients with anti-CASPR2 antibody-associated encephalitis

Eyelid Tremor in a Patient with Anti-Caspr2 Antibody-Related Encephalitis

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