The Experts below are selected from a list of 228 Experts worldwide ranked by ideXlab platform
Haruki Koike - One of the best experts on this subject based on the ideXlab platform.
-
Clinicopathological features of Neuropathy associated with lymphoma.
Brain : a journal of neurology, 2020Co-Authors: Minoru Tomita, Haruki Koike, Masahiro Iijima, Yuichi Kawagashira, Hiroaki Adachi, Jun Taguchi, Kazuya Sako, Yukiko Tsuji, Masanori NakagawaAbstract:Lymphoma causes various neurological manifestations that might affect any part of the nervous system and occur at any stage of the disease. The peripheral nervous system is one of the major constituents of the neurological involvement of lymphoma. In this study we characterized the clinical, electrophysiological and histopathological features of 32 patients with Neuropathy associated with non-Hodgkin's lymphoma that were unrelated to complications resulting from treatment for lymphoma. Nine patients had pathologically-proven neurolymphomatosis with direct invasion of lymphoma cells into the peripheral nervous system. These patients showed lymphomatous cell invasion that was more prominent in the proximal portions of the nerve trunk and that induced demyelination without macrophage invasion and subsequent axonal degeneration in the portion distal from the demyelination site. Six other patients were also considered to have neurolymphomatosis because these patients showed positive signals along the peripheral nerve on fluorodeoxyglucose positron emission tomography imaging. Spontaneous pain can significantly disrupt daily activities, as frequently reported in patients diagnosed with neurolymphomatosis. In contrast, five patients were considered to have Paraneoplastic Neuropathy because primary peripheral nerve lesions were observed without the invasion of lymphomatous cells, with three patients showing features compatible with chronic inflammatory demyelinating polyNeuropathy, one patient showing sensory ganglionopathy, and one patient showing vasculitic Neuropathy. Of the other 12 patients, 10 presented with multiple mononeuropathies. These patients showed clinical and electrophysiological features similar to those of neurolymphomatosis rather than Paraneoplastic Neuropathy. Electrophysiological findings suggestive of demyelination were frequently observed, even in patients with neurolymphomatosis. Eleven of the 32 patients, including five patients with neurolymphomatosis, fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic criteria of definite chronic inflammatory demyelinating polyNeuropathy. Some of these patients, even those with neurolymphomatosis, responded initially to immunomodulatory treatments, including the administration of intravenous immunoglobulin and steroids. Patients with lymphoma exhibit various neuropathic patterns, but neurolymphomatosis is the major cause of Neuropathy. Misdiagnoses of neurolymphomatosis as chronic inflammatory demyelinating polyNeuropathy are frequent due to a presence of a demyelinating pattern and the initial response to immunomodulatory treatments. The possibility of the concomitance of lymphoma should be considered in various types of Neuropathy, even if the diagnostic criteria of chronic inflammatory demyelinating polyNeuropathy are met, particularly in patients complaining of pain.
-
chapter 41 Paraneoplastic Neuropathy
Handbook of Clinical Neurology, 2013Co-Authors: Haruki Koike, Gen SobueAbstract:Recent progress in serological screening of Paraneoplastic antibodies and in diagnostic imaging techniques to detect malignancies has enabled a broadening of the concept of Paraneoplastic neurological syndromes by integrating nonclassic clinical features. The peripheral nervous system is frequently involved in patients with Paraneoplastic syndrome and may be seen alone or in combination with involvement of other areas of the nervous system. Destruction of dorsal root ganglion cells due to lymphocytic infiltration, especially with CD8-positive cytotoxic T cells, has been postulated to mediate the classic syndrome of subacute sensory neuronopathy. However, the motor and autonomic nervous systems are frequently affected. Indeed, patients can develop clinical features compatible with Guillain–Barre syndrome, chronic inflammatory demyelinating polyNeuropathy, or brachial plexopathy. Other forms of Paraneoplastic Neuropathy are vasculitic Neuropathy, autoimmune autonomic ganglionopathy, and chronic intestinal pseudo-obstruction. Various onconeural antibodies, including anti-Hu, anti-CV2/CRMP-5, and anti-ganglionic acetylcholine receptor antibodies, are associated with Neuropathy. Somatic Neuropathy is the most common manifestation in patients with anti-Hu and anti-CV2/CRMP-5 antibodies, while anti-ganglionic acetylcholine receptor antibody is associated with autonomic neuropathies. A whole-body fluorodeoxyglucose positron emission tomography scan may be useful to detect malignancy in patients with unremarkable conventional radiological findings. Recognition and diagnosis of Paraneoplastic Neuropathy is important, as neuropathic symptoms usually precede the identification of the primary tumor, and treatment at an earlier stage provides better chances of good outcomes.
-
Clinicopathological features of Neuropathy associated with lymphoma.
Brain, 2013Co-Authors: Minoru Tomita, Haruki Koike, Masahiro Iijima, Yuichi Kawagashira, Hiroaki Adachi, Jun Taguchi, Kazuya Sako, Yukiko Tsuji, Masanori NakagawaAbstract:Lymphoma causes various neurological manifestations that might affect any part of the nervous system and occur at any stage of the disease. The peripheral nervous system is one of the major constituents of the neurological involvement of lymphoma. In this study we characterized the clinical, electrophysiological and histopathological features of 32 patients with Neuropathy associated with non-Hodgkin’s lymphoma that were unrelated to complications resulting from treatment for lymphoma. Nine patients had pathologically-proven neurolymphomatosis with direct invasion of lymphoma cells into the peripheral nervous system. These patients showed lymphomatous cell invasion that was more prominent in the proximal portions of the nerve trunk and that induced demyelination without macrophage invasion and subsequent axonal degeneration in the portion distal from the demyelination site. Six other patients were also considered to have neurolymphomatosis because these patients showed positive signals along the peripheral nerve on fluorodeoxyglucose positron emission tomography imaging. Spontaneous pain can significantly disrupt daily activities, as frequently reported in patients diagnosed with neurolymphomatosis. In contrast, five patients were considered to have Paraneoplastic Neuropathy because primary peripheral nerve lesions were observed without the invasion of lymphomatous cells, with three patients showing features compatible with chronic inflammatory demyelinating polyNeuropathy, one patient showing sensory ganglionopathy, and one patient showing vasculitic Neuropathy. Of the other 12 patients, 10 presented with multiple mononeuropathies. These patients showed clinical and electrophysiological features similar to those of neurolymphomatosis rather than Paraneoplastic Neuropathy. Electrophysiological findings suggestive of demyelination were frequently observed, even in patients with neurolymphomatosis. Eleven of the 32 patients, including five patients with neurolymphomatosis, fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic criteria of definite chronic inflammatory demyelinating polyNeuropathy. Some of these patients, even those with neurolymphomatosis, responded initially to immunomodulatory treatments, including the administration of intravenous immunoglobulin and steroids. Patients with lymphoma exhibit various neuropathic patterns, but neurolymphomatosis is the major cause of Neuropathy. Misdiagnoses of neurolymphomatosis as chronic inflammatory demyelinating polyNeuropathy are frequent due to a presence of a demyelinating pattern and the initial response to immunomodulatory treatments. The possibility of the concomitance of lymphoma should be considered in various types of Neuropathy, even if the diagnostic criteria of chronic inflammatory demyelinating polyNeuropathy are met, particularly in patients complaining of pain. * Abbreviations : CIDP : chronic inflammatory demyelinating polyNeuropathy EFNS/PNS : European Federation of Neurological Societies/Peripheral Nerve Society FDG : fluorodeoxyglucose IVIg : intravenous immunoglobulin
-
Paraneoplastic Neuropathy wide ranging clinicopathological manifestations
Current Opinion in Neurology, 2011Co-Authors: Haruki Koike, Fumiaki Tanaka, Gen SobueAbstract:PURPOSE OF REVIEW: Recent progress in serological screening for Paraneoplastic autoantibodies and diagnostic imaging techniques to detect malignancies has resulted in a broadening of the concept of Paraneoplastic neurologic syndromes through the characterization of nonclassical clinical features. The goal of this article was to review the recent literature describing the wide-ranging clinicopathological manifestations of Paraneoplastic Neuropathy. RECENT FINDINGS: The classical feature of Paraneoplastic Neuropathy is subacute sensory neuronopathy; in addition, sensorimotor neuropathies, such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyNeuropathy, brachial plexopathy, and vasculitic Neuropathy, are sometimes observed. Some studies also describe the occurrence of autonomic neuropathies, including autoimmune autonomic ganglionopathy and chronic gastrointestinal pseudo-obstruction. Whole-body fluorodeoxyglucose positron emission tomography (FDG-PET) or FDG-PET/computed tomography may be helpful to detect malignancies that cannot be detected by conventional screening tests. The presence of Paraneoplastic Neuropathy should be considered in all patients with malignancy and can occur at any point in the disease, even during or after chemotherapy, radiation, or stem cell transplantation. The presence of Paraneoplastic autoantibodies, especially anti-Hu and anti-CV2/CRMP-5 antibodies, may support the diagnosis of Paraneoplastic Neuropathy. Immunomodulatory treatment before, during, or after antineoplastic therapy may be of benefit for patients with Paraneoplastic Neuropathy and has been used even when the underlying malignancy cannot be identified. SUMMARY: Recognition of the variable manifestations of Paraneoplastic Neuropathy is important, as diagnosis at an earlier stage facilitates prompt treatment and provides better chances of good outcomes.
-
ataxic vs painful form of Paraneoplastic Neuropathy
Neurology, 2007Co-Authors: Haruki Koike, Fumiaki Tanaka, Masahiro Iijima, Keiko Mori, Naoki Hattori, Masahisa Katsuno, Tomohiko Nakamura, Masaaki Hirayama, M Shiraishi, S YazakiAbstract:OBJECTIVE: To characterize the clinicopathologic features of ataxic and painful forms of Paraneoplastic Neuropathy. METHODS: Clinical, electrophysiologic, and histopathologic findings were assessed in 17 patients with Paraneoplastic Neuropathy. RESULTS: Clinical features can be categorized into two groups: one group (13 patients) with predominantly deep sensory disturbance and a second group (4 patients) with predominantly superficial sensory disturbance. The former group showed severe sensory ataxia and predominantly large myelinated fiber loss in the sural nerve. The latter group showed marked pain, in particular, severe mechanical hyperalgesia, and predominantly small myelinated and unmyelinated fiber loss. Nerve conduction assessment indicated an axonal Neuropathy pattern in both groups, while sensory action potentials were more markedly diminished in the sensory ataxic form. Anti-Hu antibodies were detected in half of the patients in both groups. Treatment for cancer was effective to improve or stabilize neuropathic symptoms in some cases from both groups. Immunotherapy was effective only for a short time. CONCLUSIONS: Paraneoplastic Neuropathy can be characterized into two groups by the presence of sensory ataxia or severe spontaneous pain and severe mechanical hyperalgesia. Preferential small myelinated and unmyelinated fiber loss correlated to the cases of severe pain.
Gen Sobue - One of the best experts on this subject based on the ideXlab platform.
-
chapter 41 Paraneoplastic Neuropathy
Handbook of Clinical Neurology, 2013Co-Authors: Haruki Koike, Gen SobueAbstract:Recent progress in serological screening of Paraneoplastic antibodies and in diagnostic imaging techniques to detect malignancies has enabled a broadening of the concept of Paraneoplastic neurological syndromes by integrating nonclassic clinical features. The peripheral nervous system is frequently involved in patients with Paraneoplastic syndrome and may be seen alone or in combination with involvement of other areas of the nervous system. Destruction of dorsal root ganglion cells due to lymphocytic infiltration, especially with CD8-positive cytotoxic T cells, has been postulated to mediate the classic syndrome of subacute sensory neuronopathy. However, the motor and autonomic nervous systems are frequently affected. Indeed, patients can develop clinical features compatible with Guillain–Barre syndrome, chronic inflammatory demyelinating polyNeuropathy, or brachial plexopathy. Other forms of Paraneoplastic Neuropathy are vasculitic Neuropathy, autoimmune autonomic ganglionopathy, and chronic intestinal pseudo-obstruction. Various onconeural antibodies, including anti-Hu, anti-CV2/CRMP-5, and anti-ganglionic acetylcholine receptor antibodies, are associated with Neuropathy. Somatic Neuropathy is the most common manifestation in patients with anti-Hu and anti-CV2/CRMP-5 antibodies, while anti-ganglionic acetylcholine receptor antibody is associated with autonomic neuropathies. A whole-body fluorodeoxyglucose positron emission tomography scan may be useful to detect malignancy in patients with unremarkable conventional radiological findings. Recognition and diagnosis of Paraneoplastic Neuropathy is important, as neuropathic symptoms usually precede the identification of the primary tumor, and treatment at an earlier stage provides better chances of good outcomes.
-
Paraneoplastic Neuropathy wide ranging clinicopathological manifestations
Current Opinion in Neurology, 2011Co-Authors: Haruki Koike, Fumiaki Tanaka, Gen SobueAbstract:PURPOSE OF REVIEW: Recent progress in serological screening for Paraneoplastic autoantibodies and diagnostic imaging techniques to detect malignancies has resulted in a broadening of the concept of Paraneoplastic neurologic syndromes through the characterization of nonclassical clinical features. The goal of this article was to review the recent literature describing the wide-ranging clinicopathological manifestations of Paraneoplastic Neuropathy. RECENT FINDINGS: The classical feature of Paraneoplastic Neuropathy is subacute sensory neuronopathy; in addition, sensorimotor neuropathies, such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyNeuropathy, brachial plexopathy, and vasculitic Neuropathy, are sometimes observed. Some studies also describe the occurrence of autonomic neuropathies, including autoimmune autonomic ganglionopathy and chronic gastrointestinal pseudo-obstruction. Whole-body fluorodeoxyglucose positron emission tomography (FDG-PET) or FDG-PET/computed tomography may be helpful to detect malignancies that cannot be detected by conventional screening tests. The presence of Paraneoplastic Neuropathy should be considered in all patients with malignancy and can occur at any point in the disease, even during or after chemotherapy, radiation, or stem cell transplantation. The presence of Paraneoplastic autoantibodies, especially anti-Hu and anti-CV2/CRMP-5 antibodies, may support the diagnosis of Paraneoplastic Neuropathy. Immunomodulatory treatment before, during, or after antineoplastic therapy may be of benefit for patients with Paraneoplastic Neuropathy and has been used even when the underlying malignancy cannot be identified. SUMMARY: Recognition of the variable manifestations of Paraneoplastic Neuropathy is important, as diagnosis at an earlier stage facilitates prompt treatment and provides better chances of good outcomes.
Christopher Doig - One of the best experts on this subject based on the ideXlab platform.
-
Paraneoplastic intestinal pseudo obstruction mononeuritis multiplex and sensory Neuropathy neuronopathy
The Journal of the American Osteopathic Association, 1996Co-Authors: David A Simpson, Anne M Pawlak, Louis Tegmeyer, Christopher DoigAbstract:: A patient with diffuse intestinal pseudo-obstruction consisting of gastric paresis and impaired small and large bowel motility, monoNeuropathy multiplex, and sensory Neuropathy/neuronopathy was found to have small-cell carcinoma of the lung. The clinical symptoms were thought to be consistent with Paraneoplastic Neuropathy, and high antineuronal and anti-calcium channel antibodies led to the diagnosis of small-cell carcinoma of the lung. Identification of Paraneoplastic Neuropathy is important, because early treatment may halt the progression of the underlying carcinoma.
-
Paraneoplastic intestinal pseudo-obstruction, mononeuritis multiplex, and sensory Neuropathy/neuronopathy.
The Journal of the American Osteopathic Association, 1996Co-Authors: David A Simpson, Anne M Pawlak, Louis Tegmeyer, Christopher DoigAbstract:: A patient with diffuse intestinal pseudo-obstruction consisting of gastric paresis and impaired small and large bowel motility, monoNeuropathy multiplex, and sensory Neuropathy/neuronopathy was found to have small-cell carcinoma of the lung. The clinical symptoms were thought to be consistent with Paraneoplastic Neuropathy, and high antineuronal and anti-calcium channel antibodies led to the diagnosis of small-cell carcinoma of the lung. Identification of Paraneoplastic Neuropathy is important, because early treatment may halt the progression of the underlying carcinoma.
Melcior Martinez - One of the best experts on this subject based on the ideXlab platform.
-
repeated neostigmine dosage as palliative treatment for chronic colonic pseudo obstruction in a patient with autonomic Paraneoplastic Neuropathy
The American Journal of Gastroenterology, 2003Co-Authors: Xavier Calvet, Josep Miquel Martinez, Melcior MartinezAbstract:Repeated Neostigmine Dosage as Palliative Treatment for Chronic Colonic Pseudo-Obstruction in a Patient With Autonomic Paraneoplastic Neuropathy
Jérome Honnorat - One of the best experts on this subject based on the ideXlab platform.
-
Paraneoplastic peripheral Neuropathy associated with anti-Hu antibodies. A clinical and electrophysiological study of 20 patients.
Brain : a journal of neurology, 2020Co-Authors: Jeanphilippe Camdessanche, Jérome Honnorat, Jean-christophe Antoine, Christophe Vial, P Petiot, P Convers, D MichelAbstract:Although Paraneoplastic subacute sensory neuronopathy is the most frequent presentation of peripheral Neuropathy in patients with anti-Hu antibodies, other neuropathies have been reported. In order to investigate the clinical and electrophysiological manifestations of neuropathies associated with anti-Hu antibodies, we conducted a retrospective study of 20 patients. For the electrophysiological study, each nerve was classified as normal, demyelinating, axonal/neuronal or axonal/demyelinating. Peripheral Neuropathy was the presenting symptom in 95% of patients. CNS and autonomic Neuropathy were present in 40% and 30% of patients, respectively. The course of the Neuropathy was acute, mimicking Guillain-Barré syndrome in one patient (5%), and subacute (55%) or progressive (40%) in the others. Clinically, the Neuropathy was sensory (70%), sensorimotor (25%) or motor (5%). At onset, symptoms were symmetrical (65%), asymmetrical (25%) or multifocal (10%). Pain was a predominant manifestation (80%). Amyotrophia and fasciculations were rare. The median Rankin's score was 2, three patients having an indolent form. Electrophysiology showed the axonal/neuronal pattern to be the most frequent (46.9% of studied nerves); an axonal/demyelinating or demyelinating pattern being seen in 18.3% and 4.9% of nerves, respectively. The axonal/neuronal pattern was more frequent in sensory nerves and the mixed axonal/demyelinating pattern more frequent in motor nerves (P < 0.01). A higher proportion of abnormal nerves correlated with a progressive course (P < 0.05) or a Rankin's score between 3 and 5 (P < 0.01). In patients with sensory Neuropathy, 88.5% of sensory nerves were abnormal, mostly with an axonal/neuronal pattern. In addition, 47% of motor nerves were abnormal so that only four out of 14 patients with a clinically pure sensory Neuropathy (28.6%) had an electrophysiological pattern typical of sensory neuronopathy. In patients with a sensorimotor Neuropathy, 96.6% of sensory and 71% of motor nerves were abnormal. The only statistical difference between sensory and sensorimotor neuropathies was that patients with sensorimotor Neuropathy had more frequent motor nerve involvement (P < 0.05) without differences concerning the distribution of the abnormal patterns. Needle neuromyography showed only limited evidence of motor neurone degeneration in both sensory and sensorimotor Neuropathy. The present work shows that the typical clinical and electrophysiological pattern of subacute sensory neuronopathy is rarely encountered in patients with anti-Hu antibody and that motor nerve involvement is frequently seen, even in the absence of a motor deficit. In addition to their potential pathophysiological involvement in the mechanism of the Paraneoplastic Neuropathy, these findings have practical consequences for the diagnosis of the disorder.
-
Facial pain as first manifestation of anti-Hu Paraneoplastic syndrome
The Journal of Headache and Pain, 2010Co-Authors: Geneviève Demarquay, Adrien Didelot, Véronique Rogemond, Philippe Ryvlin, Michel Gouttard, Paul Garassus, François Mauguière, Jérome HonnoratAbstract:The diagnosis of anti-Hu-associated encephalomyelitis/sensory Neuropathy may be particularly difficult when cranial nerve involvement represents the first clinical manifestation of the disease. We report a case of a patient who presented with facial pain as the first manifestation of an anti-Hu Paraneoplastic syndrome, which needs a rapid detection and treatment of the underlying tumour. We suggest that Paraneoplastic Neuropathy should be considered during the management of trigeminal neuropathic pain, especially when brain imagery is normal.
-
Paraneoplastic Peripheral Neuropathy Associated With Anti‐hu Antibodies. A Clinical And Electrophysiological Study Of 20 Patients
Journal of The Peripheral Nervous System, 2002Co-Authors: Jeanphilippe Camdessanche, Jérome Honnorat, Jean-christophe Antoine, Christophe Vial, P Petiot, P Convers, D MichelAbstract:Summary Although Paraneoplastic subacute sensory neuronopathy is the most frequent presentation of peripheral neuropa- thy in patients with anti-Hu antibodies, other neuropa- thies have been reported. In order to investigate the clinical and electrophysiological manifestations of neu- ropathies associated with anti-Hu antibodies, we con- ducted a retrospective study of 20 patients. For the electrophysiological study, each nerve was classified as normal, demyelinating, axonal/neuronal or axonal/ demyelinating. Peripheral Neuropathy was the present- ing symptom in 95% of patients. CNS and autonomic Neuropathy were present in 40% and 30% of patients, respectively. The course of the Neuropathy was acute, mimicking Guillain-Barresyndrome in one patient (5%), and subacute (55%) or progressive (40%) in the others. Clinically, the Neuropathy was sensory (70%), sensorimotor (25%) or motor (5%). At onset, symptoms were symmetrical (65%), asymmetrical (25%) or multi- focal (10%). Pain was a predominant manifestation (80%). Amyotrophia and fasciculations were rare. The median Rankin's score was 2, three patients having an indolent form. Electrophysiology showed the axonal/ neuronal pattern to be the most frequent (46.9% of studied nerves); an axonal/demyelinating or demyelinat- ing pattern being seen in 18.3% and 4.9% of nerves, respectively. The axonal/neuronal pattern was more fre- quent in sensory nerves and the mixed axonal/demyeli- nating pattern more frequent in motor nerves (P < 0.01). A higher proportion of abnormal nerves correlated with a progressive course (P < 0.05) or a Rankin's score between 3 and 5 (P < 0.01). In patients with sensory Neuropathy, 88.5% of sensory nerves were abnormal, mostly with an axonal/neuronal pattern. In addition, 47% of motor nerves were abnormal so that only four out of 14 patients with a clinically pure sensory neuro- pathy (28.6%) had an electrophysiological pattern typ- ical of sensory neuronopathy. In patients with a sensorimotor Neuropathy, 96.6% of sensory and 71% of motor nerves were abnormal. The only statistical differ- ence between sensory and sensorimotor neuropathies was that patients with sensorimotor Neuropathy had more frequent motor nerve involvement (P < 0.05) with- out differences concerning the distribution of the abnor- mal patterns. Needle neuromyography showed only limited evidence of motor neurone degeneration in both sensory and sensorimotor Neuropathy. The present work shows that the typical clinical and electrophysiolo- gical pattern of subacute sensory neuronopathy is rarely encountered in patients with anti-Hu antibody and that motor nerve involvement is frequently seen, even in the absence of a motor deficit. In addition to their potential pathophysiological involvement in the mechanism of the Paraneoplastic Neuropathy, these findings have practical consequences for the diagnosis of the disorder.
-
Paraneoplastic peripheral Neuropathy associated with anti hu antibodies a clinical and electrophysiological study of 20 patients
Journal of The Peripheral Nervous System, 2002Co-Authors: Jeanphilippe Camdessanche, Jérome Honnorat, Jean-christophe Antoine, Christophe Vial, P Petiot, P Convers, D MichelAbstract:Summary Although Paraneoplastic subacute sensory neuronopathy is the most frequent presentation of peripheral neuropa- thy in patients with anti-Hu antibodies, other neuropa- thies have been reported. In order to investigate the clinical and electrophysiological manifestations of neu- ropathies associated with anti-Hu antibodies, we con- ducted a retrospective study of 20 patients. For the electrophysiological study, each nerve was classified as normal, demyelinating, axonal/neuronal or axonal/ demyelinating. Peripheral Neuropathy was the present- ing symptom in 95% of patients. CNS and autonomic Neuropathy were present in 40% and 30% of patients, respectively. The course of the Neuropathy was acute, mimicking Guillain-Barresyndrome in one patient (5%), and subacute (55%) or progressive (40%) in the others. Clinically, the Neuropathy was sensory (70%), sensorimotor (25%) or motor (5%). At onset, symptoms were symmetrical (65%), asymmetrical (25%) or multi- focal (10%). Pain was a predominant manifestation (80%). Amyotrophia and fasciculations were rare. The median Rankin's score was 2, three patients having an indolent form. Electrophysiology showed the axonal/ neuronal pattern to be the most frequent (46.9% of studied nerves); an axonal/demyelinating or demyelinat- ing pattern being seen in 18.3% and 4.9% of nerves, respectively. The axonal/neuronal pattern was more fre- quent in sensory nerves and the mixed axonal/demyeli- nating pattern more frequent in motor nerves (P < 0.01). A higher proportion of abnormal nerves correlated with a progressive course (P < 0.05) or a Rankin's score between 3 and 5 (P < 0.01). In patients with sensory Neuropathy, 88.5% of sensory nerves were abnormal, mostly with an axonal/neuronal pattern. In addition, 47% of motor nerves were abnormal so that only four out of 14 patients with a clinically pure sensory neuro- pathy (28.6%) had an electrophysiological pattern typ- ical of sensory neuronopathy. In patients with a sensorimotor Neuropathy, 96.6% of sensory and 71% of motor nerves were abnormal. The only statistical differ- ence between sensory and sensorimotor neuropathies was that patients with sensorimotor Neuropathy had more frequent motor nerve involvement (P < 0.05) with- out differences concerning the distribution of the abnor- mal patterns. Needle neuromyography showed only limited evidence of motor neurone degeneration in both sensory and sensorimotor Neuropathy. The present work shows that the typical clinical and electrophysiolo- gical pattern of subacute sensory neuronopathy is rarely encountered in patients with anti-Hu antibody and that motor nerve involvement is frequently seen, even in the absence of a motor deficit. In addition to their potential pathophysiological involvement in the mechanism of the Paraneoplastic Neuropathy, these findings have practical consequences for the diagnosis of the disorder.
-
Paraneoplastic peripheral Neuropathy associated with anti hu antibodies a clinical and electrophysiological study of 20 patients
Brain, 2002Co-Authors: Jeanphilippe Camdessanche, Jérome Honnorat, Jean-christophe Antoine, Christophe Vial, P Petiot, P Convers, D MichelAbstract:Although Paraneoplastic subacute sensory neuronopathy is the most frequent presentation of peripheral Neuropathy in patients with anti‐Hu antibodies, other neuropathies have been reported. In order to investigate the clinical and electrophysiological manifestations of neuropathies associated with anti‐Hu antibodies, we conducted a retrospective study of 20 patients. For the electrophysiological study, each nerve was classified as normal, demyelinating, axonal/neuronal or axonal/demyelinating. Peripheral Neuropathy was the presenting symptom in 95% of patients. CNS and autonomic Neuropathy were present in 40% and 30% of patients, respectively. The course of the Neuropathy was acute, mimicking Guillain–Barre syndrome in one patient (5%), and subacute (55%) or progressive (40%) in the others. Clinically, the Neuropathy was sensory (70%), sensorimotor (25%) or motor (5%). At onset, symptoms were symmetrical (65%), asymmetrical (25%) or multifocal (10%). Pain was a predominant manifestation (80%). Amyotrophia and fasciculations were rare. The median Rankin’s score was 2, three patients having an indolent form. Electrophysiology showed the axonal/neuronal pattern to be the most frequent (46.9% of studied nerves); an axonal/demyelinating or demyelinating pattern being seen in 18.3% and 4.9% of nerves, respectively. The axonal/neuronal pattern was more frequent in sensory nerves and the mixed axonal/demyelinating pattern more frequent in motor nerves ( P < 0.01). A higher proportion of abnormal nerves correlated with a progressive course ( P < 0.05) or a Rankin’s score between 3 and 5 ( P < 0.01). In patients with sensory Neuropathy, 88.5% of sensory nerves were abnormal, mostly with an axonal/neuronal pattern. In addition, 47% of motor nerves were abnormal so that only four out of 14 patients with a clinically pure sensory Neuropathy (28.6%) had an electrophysiological pattern typical of sensory neuronopathy. In patients with a sensorimotor Neuropathy, 96.6% of sensory and 71% of motor nerves were abnormal. The only statistical difference between sensory and sensorimotor neuropathies was that patients with sensorimotor Neuropathy had more frequent motor nerve involvement ( P < 0.05) without differences concerning the distribution of the abnormal patterns. Needle neuromyography showed only limited evidence of motor neurone degeneration in both sensory and sensorimotor Neuropathy. The present work shows that the typical clinical and electrophysiological pattern of subacute sensory neuronopathy is rarely encountered in patients with anti‐Hu antibody and that motor nerve involvement is frequently seen, even in the absence of a motor deficit. In addition to their potential pathophysiological involvement in the mechanism of the Paraneoplastic Neuropathy, these findings have practical consequences for the diagnosis of the disorder. Received May 21, 2001. Revised July 31, 2001. Accepted August 13, 2001.