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F57. EEG findings in pediatric parainfectious acute bilateral striatal necrosis
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Abstracts / Clinical Neurophysiology 129 (2018) e66–e141
Conclusion: Neuropathies in patients with chronic Lyme disease commonly present as non-vasculitic multiple mononeuritis, the motor deficit follows the multiple pattern. This case is based on an unusual neurological manifestation together with the exclusive predilection for affection of the central nervous system, having as main geographical and occupational risk factors. doi:10.1016/j.clinph.2018.04.219
F57. EEG findings in pediatric parainfectious acute bilateral striatal necrosis—Ignacio Rubio-Agusti *, Ana Felipe-Rucian, Alfons Macaya- Ruiz, Monica Vicente-Rasoamalala (Spain) ⇑
CSF analysis was normal, but HHV6 replication was shown in plasma and CSF. EEG was normal. Metabolic screening showed raised urinary 3-OH-glutaric acid, not confirmed on repeated tests. She was treated with foscarnet, IVIG and glucocorticoids and was discharged after 43 days, with mild symptoms. She was asymptomatic at 8 months follow-up. Control MR showed atrophy and cavitation of posterior putamina. Conclusion: EEG allows objective assessment and monitoring of brain function in parainfectious ABSN, when it presents with seizures and/or altered level of awareness. Focal abnormalities may correlate with damage beyond the striatum and into the cortex. doi:10.1016/j.clinph.2018.04.220
Presenting author.
Introduction: Acute bilateral striatal necrosis (ABSN) is a clinicoradiological syndrome with different causes: genetic (mostly, inborn errors of metabolism) and acquired. Among these, parainfectious forms are especially relevant, since they may be confused with genetic forms, but carry a better prognosis and may benefit from immunomodulation. ABSN presents with any combination of movement disorders, altered state of awareness and seizures. The latter warrant an EEG, likely to be performed before imaging can confirm the syndromic diagnosis. Aim: To describe the electroclinical findings of pediatric patients with confirmed parainfectious ABSN attending our hospital between 01/2015 and 12/2015. Methods: Retrospective review of clinical records of patients fulfilling the following: Inclusion criteria: – Age at onset 616 years. – Onset of movement disorders, altered level of awareness or seizures within 3 weeks of a febrile episode. – Acute bilateral neostriatal lesions (enlargement and T2/Flair hyperintensity on MR or hypodensity on CT of caudate and/or putamen bilaterally). – Evidence of recent/ongoing infection in biological samples (Direct: Positive culture/PCR; Indirect: Seroconversion/Raising antibody titres). Exclusion criteria: – Progressive course after 6 weeks – Evidence of a metabolic disorder (Positive genetic testing for pathogenic mutations or confirmed altered enzyme activity/ altered biochemical markers in biological samples). – Alternative cause. Results: We found 2 cases: 1. 11 year old boy presenting with focal seizures and parkinsonism, 48 h after a febrile episode. MR showed symmetric striatal inflammation and patchy cortical edema, predominantly frontal. CSF analysis showed mononuclear pleocytosis and increased protein. A tracheal aspirate disclosed M. pneumoniae infection. Metabolic screening was negative. Control of seizures was difficult, requiring combined treatment with LEV, LCM, VPA, Antibiotics, IVIG and glucocorticoids. Serial EEGs showed diffuse background slowing and focal asymmetric fronto-temporal slowing (right worse). He was discharged asymptomatic after 13 days, on LEV and LCM, which were stopped over the following 5 months. MR and EEG were normal 3 months after onset. 2: 2 year old girl presenting with parkinsonism, dystonia and bulbar symptoms 7 days after a febrile episode. MR showed symmetric striatal inflammation.
F58. Separation of human central pain and temperature pathways—Nakia Wilson (USA)
Introduction: Facial sensations of sharp (pin), warm, cool and touch are transmitted by different peripheral fibers. Animal studies show separate trigeminal nucleus caudalis (NC) neurons responding to pain and temperature modalities but also possibly some polymodal neurons. Traditionally, the animal and human central sensory pathways are described as follows. Pain and temperature fibers travel together to synapse in the inferior trigeminal spinal nucleus, NC. Post-synaptic axons cross the midline in a single pathway to form the pain and temperature trigemino-thalamic pathway. Larger myelinated touch fibers synapse in the brainstem 5th nucleus but smaller touch fibers synapse in spinal nuclei. Disruption of crossing fibers at the midline causes simultaneous pain and temperature deficits in syringomyelia. This report describes 11 syringomyelia patients whose deficits provide evidence for separate, central afferent, post-synaptic pathways for facial pain, warm and cool sensations. Methods: Evaluations: MRIs and exams including stimuli at 21–22 °C and 40–41 °C. Results: Exams show discrepancies between warm and cool sensations and between pin and temperature sensations. Preserved touch is present in 3 patients with pin deficit. Three cases lack MRI abnormalities appropriate to their deficit, but 2 of those probably had syringes that were previously larger. Conclusion: Translation of experimental animal studies to human function benefits from verification. Unlike human case reports and animal studies, the spinal cord lesions in our patients did not cause symmetrical pain and temperature deficits. Most human reports of temperature sensation don’t indicate what thermal stimulus was used. Use of careful warm and cool stimuli would likely find additional discrepant cases that also suggest distinct but probably adjacent pain, warm and cool central sensory pathways. Differences between touch and pin sensation may be due to the bilateral touch pathways. doi:10.1016/j.clinph.2018.04.221
F59. Spasmodic reflex myoclonus-an characteristic electrophysiological manifestation for the diagnosis of Stiff-person syndrome—Chon-Haw Tsai (Taiwan)
Introduction: Stiff person syndrome (SPS) is a rare disease entity and the pivotal clinical features contain progressive course of fluctuating stiffness of the low back and lower limbs. Progressive encephalomyelitis with rigidity and myoclonus (PERM) is one of
Abstracts / Clinical Neurophysiology 129 (2018) e66–e141
Conclusion: Neuropathies in patients with chronic Lyme disease commonly present as non-vasculitic multiple mononeuritis, the motor deficit follows the multiple pattern. This case is based on an unusual neurological manifestation together with the exclusive predilection for affection of the central nervous system, having as main geographical and occupational risk factors. doi:10.1016/j.clinph.2018.04.219
F57. EEG findings in pediatric parainfectious acute bilateral striatal necrosis—Ignacio Rubio-Agusti *, Ana Felipe-Rucian, Alfons Macaya- Ruiz, Monica Vicente-Rasoamalala (Spain) ⇑
CSF analysis was normal, but HHV6 replication was shown in plasma and CSF. EEG was normal. Metabolic screening showed raised urinary 3-OH-glutaric acid, not confirmed on repeated tests. She was treated with foscarnet, IVIG and glucocorticoids and was discharged after 43 days, with mild symptoms. She was asymptomatic at 8 months follow-up. Control MR showed atrophy and cavitation of posterior putamina. Conclusion: EEG allows objective assessment and monitoring of brain function in parainfectious ABSN, when it presents with seizures and/or altered level of awareness. Focal abnormalities may correlate with damage beyond the striatum and into the cortex. doi:10.1016/j.clinph.2018.04.220
Presenting author.
Introduction: Acute bilateral striatal necrosis (ABSN) is a clinicoradiological syndrome with different causes: genetic (mostly, inborn errors of metabolism) and acquired. Among these, parainfectious forms are especially relevant, since they may be confused with genetic forms, but carry a better prognosis and may benefit from immunomodulation. ABSN presents with any combination of movement disorders, altered state of awareness and seizures. The latter warrant an EEG, likely to be performed before imaging can confirm the syndromic diagnosis. Aim: To describe the electroclinical findings of pediatric patients with confirmed parainfectious ABSN attending our hospital between 01/2015 and 12/2015. Methods: Retrospective review of clinical records of patients fulfilling the following: Inclusion criteria: – Age at onset 616 years. – Onset of movement disorders, altered level of awareness or seizures within 3 weeks of a febrile episode. – Acute bilateral neostriatal lesions (enlargement and T2/Flair hyperintensity on MR or hypodensity on CT of caudate and/or putamen bilaterally). – Evidence of recent/ongoing infection in biological samples (Direct: Positive culture/PCR; Indirect: Seroconversion/Raising antibody titres). Exclusion criteria: – Progressive course after 6 weeks – Evidence of a metabolic disorder (Positive genetic testing for pathogenic mutations or confirmed altered enzyme activity/ altered biochemical markers in biological samples). – Alternative cause. Results: We found 2 cases: 1. 11 year old boy presenting with focal seizures and parkinsonism, 48 h after a febrile episode. MR showed symmetric striatal inflammation and patchy cortical edema, predominantly frontal. CSF analysis showed mononuclear pleocytosis and increased protein. A tracheal aspirate disclosed M. pneumoniae infection. Metabolic screening was negative. Control of seizures was difficult, requiring combined treatment with LEV, LCM, VPA, Antibiotics, IVIG and glucocorticoids. Serial EEGs showed diffuse background slowing and focal asymmetric fronto-temporal slowing (right worse). He was discharged asymptomatic after 13 days, on LEV and LCM, which were stopped over the following 5 months. MR and EEG were normal 3 months after onset. 2: 2 year old girl presenting with parkinsonism, dystonia and bulbar symptoms 7 days after a febrile episode. MR showed symmetric striatal inflammation.
F58. Separation of human central pain and temperature pathways—Nakia Wilson (USA)
Introduction: Facial sensations of sharp (pin), warm, cool and touch are transmitted by different peripheral fibers. Animal studies show separate trigeminal nucleus caudalis (NC) neurons responding to pain and temperature modalities but also possibly some polymodal neurons. Traditionally, the animal and human central sensory pathways are described as follows. Pain and temperature fibers travel together to synapse in the inferior trigeminal spinal nucleus, NC. Post-synaptic axons cross the midline in a single pathway to form the pain and temperature trigemino-thalamic pathway. Larger myelinated touch fibers synapse in the brainstem 5th nucleus but smaller touch fibers synapse in spinal nuclei. Disruption of crossing fibers at the midline causes simultaneous pain and temperature deficits in syringomyelia. This report describes 11 syringomyelia patients whose deficits provide evidence for separate, central afferent, post-synaptic pathways for facial pain, warm and cool sensations. Methods: Evaluations: MRIs and exams including stimuli at 21–22 °C and 40–41 °C. Results: Exams show discrepancies between warm and cool sensations and between pin and temperature sensations. Preserved touch is present in 3 patients with pin deficit. Three cases lack MRI abnormalities appropriate to their deficit, but 2 of those probably had syringes that were previously larger. Conclusion: Translation of experimental animal studies to human function benefits from verification. Unlike human case reports and animal studies, the spinal cord lesions in our patients did not cause symmetrical pain and temperature deficits. Most human reports of temperature sensation don’t indicate what thermal stimulus was used. Use of careful warm and cool stimuli would likely find additional discrepant cases that also suggest distinct but probably adjacent pain, warm and cool central sensory pathways. Differences between touch and pin sensation may be due to the bilateral touch pathways. doi:10.1016/j.clinph.2018.04.221
F59. Spasmodic reflex myoclonus-an characteristic electrophysiological manifestation for the diagnosis of Stiff-person syndrome—Chon-Haw Tsai (Taiwan)
Introduction: Stiff person syndrome (SPS) is a rare disease entity and the pivotal clinical features contain progressive course of fluctuating stiffness of the low back and lower limbs. Progressive encephalomyelitis with rigidity and myoclonus (PERM) is one of