ArticleJournal of neurology, neurosurgery, and psychiatry2004
Hyperekplexia and stiff-man syndrome: abnormal brainstem reflexes suggest a physiological relationship.
Article in Journal of neurology, neurosurgery, and psychiatry, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 56 citations in OpenAlex.
- Neurophysiological Insights into the Pathophysiology of Stiff-Person Spectrum Disorders.Movement disorders clinical practice · 2025Review
- Myoclonus and other jerky movement disorders.Clinical neurophysiology practice · 2022Review
- The Head Retraction Reflex in Niemann-Pick Type C: A Novel Diagnostic Clue.Movement disorders clinical practice · 2020Article
- GAD antibodies in neurological disorders - insights and challenges.Nature reviews. Neurology · 2020Review
- Principles of Electrophysiological Assessments for Movement Disorders.Journal of movement disorders · 2020Article
- A case report of rigidity and recurrent lower limb myoclonus: progressive encephalomyelitis rigidity and myoclonus syndrome, a chameleon.BMC neurology · 2018Article
- Synaptopathies: synaptic dysfunction in neurological disorders - A review from students to students.Journal of neurochemistry · 2016Review
- Lockjaw in stiff-person syndrome with autoantibodies against glycine receptors.Neurology(R) neuroimmunology & neuroinflammation · 2016Article
- Spinal-generated movement disorders: a clinical review.Journal of clinical movement disorders · 2015Review
- [Stiff man syndrome and variants].Der Nervenarzt · 2013Article
- Autoimmune stiff person syndrome and related myelopathies: understanding of electrophysiological and immunological processes.Muscle & nerve · 2012Review
- Cranial movement disorders: clinical features, pathophysiology, differential diagnosis and treatment.Nature clinical practice. Neurology · 2009Review
- Axial Dystonia Mimicking Stiff Person Syndrome.Movement disorders clinical practiceArticle
Corrections and comments
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Authors and funding
3 authors at 1 institution in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesHyperekplexia and the stiff-man syndrome (SMS) are both conditions with exaggerated startle suggesting abnormal brainstem function. Investigation of brainstem reflexes may provide insight into disturbed reflex excitation and inhibition underlying these movement disorders. PATIENTS AND
methodsUsing four-channel EMG, we examined four trigeminal brainstem reflexes (monosynaptic masseter, masseter inhibitory, glabella, and orbicularis oculi blink reflexes) and their spread into pericranial muscles in five patients with familial hyperekplexia (FH), two with acquired hyperekplexia (AH), 10 with SMS, and 15 healthy control subjects.
resultsBoth FH/AH and SMS patients had abnormal propagation of brainstem reflexes into pericranial muscles. All patients with hyperekplexia showed an abnormal short-latency (15-20 ms) reflex in the trapezius muscle with a characteristic clinical appearance ("head retraction jerk") evoked by tactile or electrical stimulation of the trigeminal nerve, but normal monosynaptic masseter reflexes. Inhibitory brainstem reflexes were attenuated in some FH/AH patients. Four of 10 patients with SMS had similar short-latency reflexes in the neck muscles and frequently showed widespread enhancement of other excitatory reflexes, reflex spasms, and attenuation of inhibitory brainstem reflexes.
conclusionReflex excitation is exaggerated and inhibition is attenuated in both stiff-man syndrome and familial or acquired hyperekplexia, indicating a physiological relationship. Reflex transmission in the brainstem appears biased towards excitation which may imply dysfunction of inhibitory glycinergic or GABAergic interneurons, or both.
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