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P48. Demyelinating disease affecting the central and peripheral nervous system
Society Proceedings / Clinical Neurophysiology 126 (2015) e63–e170
subjects and patients suffering from global cerebellocortical degeneration. Subjects were asked to make a long series of rapid alternating up and down movements of their index finger about the MCP joint in order to move a cursor, representing the finger tip’s vertical position, into narrow target zones on a monitor in front of the subject. During the experiment the hand was kept in a comfortable horizontal resting position and movements of the distal finger joints were prevented by a splint. Finger position was measured with the search coil technique. To keep the participants motivated during the task, we provided continuous feedback on task performance. Healthy subjects were able to ensure precise pointing movements despite trial to trial as well as long-term variations in finger velocity by adjusting duration accordingly. This velocity-duration tradeoff was deteriorated in patients, explaining the much larger endpoint variability. These findings suggest that dysmetria of movement, the hallmark of cerebellar disease, is in general a consequence of the inability to fine tune movement duration. In other words, these observations on finger movements support the notion, originally suggested by work on saccades, that the cerebellum contributes to movement control by deploying a fast and precise velocity-duration tradeoff mechanism. doi:10.1016/j.clinph.2015.04.184
P48. Demyelinating disease affecting the central and peripheral nervous system—F.P. Bernhard a, J. Marquetand b, F. Bischof c (a Universitätsklinikum Tübingen, Klinik für Neurologie, Abteilung für Neurodegenerative Erkrankungen, Tübingen, b Germany, Universitätsklinikum Tübingen, Klinik für Neurologie, Abteilung für Epilepsie, Tübingen, Germany, c Universitätsklinikum Tübingen, Klinik für Neurologie, Abteilung mit Schwerpunkt für Neurovaskuläre Erkrankungen, Tübingen, Germany) Inflammatory demyelinating diseases are common in the field of neurology and lead to a destruction of the myelin sheath. Either Oligodendrocytes of the central nervous system (e.g. encephalomyelitis disseminata) or Schwann cells- of the peripheral nervous system (e.g. chronic inflammatory demyelinating polyneuropathy) are affected, but never are both of them at the same time. Within this article we report one of the first documentated two cases of simultaneous demyelination of the central and the peripheral nervous system (Mao and Hu, 2014). First, a 33-year-old male after allogenic stem cell transplantation due to a hodgkins-lymphoma. Our second case is a healthy 28-year-old female patient. Both developed at first a demyelination of the peripheral nervous system and secondly, after four and eight weeks a subacute
Fig. 1. T1-weighted sequence with gadolinium.
e119
Fig. 2. T1-weighted sequence with gadolinium.
demyelination of the central nervous system. Both cases showed electrophysiological findings of demyelination, gadolinum-enhanced lesions in MRI and positive oligoclonal bands with high IgG-indices in the cerebrospinal fluid. Up to our knowledge these are the first two cases of a central and a peripheral demyelination. As a consequence of the affection of both: peripheral and central myelin sheath we postulate that there has to be a similar antigen between these two different cell-identities: oligodendrocytes and Schwann cells. Further we address the question of a not yet known pathomechanism, affecting both nervous systems simultaneously and leading to the postulation of a finding of a new demyelinisating disease identity, affecting the two different- and dissimiliarnervous-systems. doi:10.1016/j.clinph.2015.04.185
P50. An extraordinary hour glass forming enhancing mass in the neuroforamina L2/3 with spontaneously complete remission—P. Kern, J. Faiss (ASKLEPIOS Fachklinikum Teupitz, Neurologie, Teupitz, Germany) A 46 year old patient presented with a since 4 weeks progressive lumbar pain, which radiate to the left ventral thigh down to the knee, since 2 weeks additionally sensory changes in this location. Apart from Crohn’s disease there are no further illnesses known in the patient’s history. The clinical-neurological results show numbness and paresthesia in the left ventral thigh, no paresis and normal deep tendon reflexes. No signs of herpetiform skin disorders. The MRI scan of the lumbar spine showed an hour glass forming gadolinium enhancing mass with intra- and extradural parts between the 2nd and 3rd lumbar vertebrae on the left side (Figs. 1 and 2), interpretated most likely as a schwannoma. Laboratory chemistry results were without adverse findings. CRP, blood sedimentation rate, ACE, HIV serology, VZV serology and serology of borreliosis were all negative. The liquor findings were also normal, no intrathecal immunglobuline synthesis, no oligoclonal bands. In the following 2 months the symptoms came into complete remission spontaneously. The follow-up- MRI scan also showed surprisingly also a complete remission of the enhancing mass. Discussion: The case report describes a typical radicular syndrome, the correlation in the MRI was an hour glass forming enhancing mass, primarily interpretated as a schwannoma. Through the complete clinical remission and remission in MRI, a neoplasma could be ruled out. Possible differential diagnoses are a radiculitis or an atypical intervertrebral disc prolapse. Gadolinum uptake in the
subjects and patients suffering from global cerebellocortical degeneration. Subjects were asked to make a long series of rapid alternating up and down movements of their index finger about the MCP joint in order to move a cursor, representing the finger tip’s vertical position, into narrow target zones on a monitor in front of the subject. During the experiment the hand was kept in a comfortable horizontal resting position and movements of the distal finger joints were prevented by a splint. Finger position was measured with the search coil technique. To keep the participants motivated during the task, we provided continuous feedback on task performance. Healthy subjects were able to ensure precise pointing movements despite trial to trial as well as long-term variations in finger velocity by adjusting duration accordingly. This velocity-duration tradeoff was deteriorated in patients, explaining the much larger endpoint variability. These findings suggest that dysmetria of movement, the hallmark of cerebellar disease, is in general a consequence of the inability to fine tune movement duration. In other words, these observations on finger movements support the notion, originally suggested by work on saccades, that the cerebellum contributes to movement control by deploying a fast and precise velocity-duration tradeoff mechanism. doi:10.1016/j.clinph.2015.04.184
P48. Demyelinating disease affecting the central and peripheral nervous system—F.P. Bernhard a, J. Marquetand b, F. Bischof c (a Universitätsklinikum Tübingen, Klinik für Neurologie, Abteilung für Neurodegenerative Erkrankungen, Tübingen, b Germany, Universitätsklinikum Tübingen, Klinik für Neurologie, Abteilung für Epilepsie, Tübingen, Germany, c Universitätsklinikum Tübingen, Klinik für Neurologie, Abteilung mit Schwerpunkt für Neurovaskuläre Erkrankungen, Tübingen, Germany) Inflammatory demyelinating diseases are common in the field of neurology and lead to a destruction of the myelin sheath. Either Oligodendrocytes of the central nervous system (e.g. encephalomyelitis disseminata) or Schwann cells- of the peripheral nervous system (e.g. chronic inflammatory demyelinating polyneuropathy) are affected, but never are both of them at the same time. Within this article we report one of the first documentated two cases of simultaneous demyelination of the central and the peripheral nervous system (Mao and Hu, 2014). First, a 33-year-old male after allogenic stem cell transplantation due to a hodgkins-lymphoma. Our second case is a healthy 28-year-old female patient. Both developed at first a demyelination of the peripheral nervous system and secondly, after four and eight weeks a subacute
Fig. 1. T1-weighted sequence with gadolinium.
e119
Fig. 2. T1-weighted sequence with gadolinium.
demyelination of the central nervous system. Both cases showed electrophysiological findings of demyelination, gadolinum-enhanced lesions in MRI and positive oligoclonal bands with high IgG-indices in the cerebrospinal fluid. Up to our knowledge these are the first two cases of a central and a peripheral demyelination. As a consequence of the affection of both: peripheral and central myelin sheath we postulate that there has to be a similar antigen between these two different cell-identities: oligodendrocytes and Schwann cells. Further we address the question of a not yet known pathomechanism, affecting both nervous systems simultaneously and leading to the postulation of a finding of a new demyelinisating disease identity, affecting the two different- and dissimiliarnervous-systems. doi:10.1016/j.clinph.2015.04.185
P50. An extraordinary hour glass forming enhancing mass in the neuroforamina L2/3 with spontaneously complete remission—P. Kern, J. Faiss (ASKLEPIOS Fachklinikum Teupitz, Neurologie, Teupitz, Germany) A 46 year old patient presented with a since 4 weeks progressive lumbar pain, which radiate to the left ventral thigh down to the knee, since 2 weeks additionally sensory changes in this location. Apart from Crohn’s disease there are no further illnesses known in the patient’s history. The clinical-neurological results show numbness and paresthesia in the left ventral thigh, no paresis and normal deep tendon reflexes. No signs of herpetiform skin disorders. The MRI scan of the lumbar spine showed an hour glass forming gadolinium enhancing mass with intra- and extradural parts between the 2nd and 3rd lumbar vertebrae on the left side (Figs. 1 and 2), interpretated most likely as a schwannoma. Laboratory chemistry results were without adverse findings. CRP, blood sedimentation rate, ACE, HIV serology, VZV serology and serology of borreliosis were all negative. The liquor findings were also normal, no intrathecal immunglobuline synthesis, no oligoclonal bands. In the following 2 months the symptoms came into complete remission spontaneously. The follow-up- MRI scan also showed surprisingly also a complete remission of the enhancing mass. Discussion: The case report describes a typical radicular syndrome, the correlation in the MRI was an hour glass forming enhancing mass, primarily interpretated as a schwannoma. Through the complete clinical remission and remission in MRI, a neoplasma could be ruled out. Possible differential diagnoses are a radiculitis or an atypical intervertrebral disc prolapse. Gadolinum uptake in the