Anterocollis and startle myoclonus due to Lyme meningomyeloradiculitis


A 26-year-old previously healthy man was admitted to our hospital reporting a 5-month history of fatigue and malaise, headaches, pain in the neck with occasional radiation in both arms, and, new-onset episodic abnormal head posture and involuntary brief jerks of both arms. External magnetic resonance imaging (MRI) of the cervical spine, without contrast application, had been assessed as normal. Prior treatment with physiotherapy, lidocaine injections into neck muscles, and oral medication including diclofenac, prednisolon, tetrazepam and tizanidin had been inefficacious. On admission, both Kernig’s sign and Lhermitte’s sign were positive. He had mild left proximal upper limb weakness and brisk deep tendon reflexes, but there was no spasticity and plantar responses were flexor. There was mild gait ataxia. The most impressive features of his presentation were two kinds of involuntary movements. He displayed patterned, repetitive muscle contractions that resulted in painful neck flexion with marked anterocollis. In addition, there was frequent startle myoclonus involving both arms and the upper trunk following tactile and acoustic stimuli. The rest of the neurological examination was unremarkable. The patient denied any history of neuroleptic medication or illicit drugs. He recalled multiple tick bites in the past 8 months but no skin lesions resembling erythema migrans. Magnetic resonance imaging of the head and spine revealed widespread contrast enhancement of the leptomeninges and cervical nerve roots, and signal alterations in the upper cervical myelon consistent with meningomyeloradiculitis (Fig. 1a–d). Cerebrospinal fluid (CSF) examination confirmed the diagnosis of active neuroborreliosis with lymphocytic pleocytosis (244 cells/lL), raised protein (101 mg/dL), normal lactate (1.6 mmol/L), positive oligoclonal bands, and a positive CSF/serum Borrelia burgdorferi-specific IgG index ([2). Intravenous ceftriaxone 2 g/day over 3 weeks resulted in full recovery, with complete resolution of dystonia and myoclonus already a few days into antibiotic treatment. One year later, the patient was symptom-free and contrast-enhanced MRI of the cervical myelon was normal. Lyme neuroborreliosis (LNB) usually presents with meningitis, cranial neuritis, and polyradiculoneuritis, while central nervous system involvement is rare [1, 2]. Literature on LNB presenting with movement disorders is very scarce [3, 4]. To the best of our knowledge this is the first report on a patient with two kinds of involuntary movements caused by LNB meningomyeloradiculitis. In our patient, MRI showed meningeal enhancement at the level of the medulla oblongata. Some form of inflammatory brainstem involvement, not depicted by MRI, may cause startle myoclonus [4]. Brainstem myoclonus particularly affects the upper body in response to unexpected stimuli, corresponding to pathological exaggeration of the startle reflex generated in the brainstem [5]. Antibiotic treatment led to rapid resolution of involuntary movements, while symptomatic treatment had had little effect. The diagnosis of LNB is not always & Raffi Topakian

DOI: 10.1007/s13760-016-0636-z

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@article{Schneider2016AnterocollisAS, title={Anterocollis and startle myoclonus due to Lyme meningomyeloradiculitis}, author={Andrea L C Schneider and Barbara Pischinger and Sibylle Wimmer and Raffi Topakian}, journal={Acta Neurologica Belgica}, year={2016}, volume={117}, pages={317-318} }