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Sensory ataxic neuropathy
486
Rev Neurol (Paris) 2007 ; 163 : 4, 483-492
social/vocational disability may be a greater problem than the seizures and may be related to the perceived social stigma associated with the diagnosis. Insufficient information about the disorder and inadequate professional support is common. Supportive cognitive therapy, specialist epilepsy nursing, and structured educational programmes are essential parts of an operative comprehensive epilepsy service. Intervention of this kind has demonstrated a beneficial effect on the quality of life in patients with active epilepsy. During recent years, it has been increasingly recognized that AEDs have différent pharmacodynamic properties and multiple applications. Some may have CNS side effects which may negatively influence affective disorders, whereas others have mood stabilizing or even thymoleptic or anxiolytic effects. It is important to be familiar with the various potential effects of the different treatment modalities, including vagal nerve stimulation. The therapy should be carefully tailored to the clinical profile of each patient. Both benefits and risks should be weighed when treating epileptic patients with antidepressants. Mutual pharmacokinetic and pharmacodynamic interactions should be considered. Compounds without known association with seizures should be preferred. SSRIs are usually safe and effective, but further research is needed to define optimal strategies. In mood disorders, attempts to analyze intrinsic neurobiological factors and the pharmacological profiles of prescribed AEDs, as well as individual psychosocial aspects should be performed. All these considerations have to be taken into account for the individual patient.
● Instructive cases from the neurology service S.I. Harik Professor and Chairman of Neurology, University of Arkansas for Medical Sciences, USA.
Several relatively difficult, yet instructive actual cases in clinical neurology were be presented. There are lessons to be learned from each case. Audience interaction is encouraged. The give and take that I hope will materialize will help focus on many important issues in clinical neurology that are illustrated by these cases. The subject matter of the cases include: stroke, central nervous system infections, brain tumors, IVIG treatment for Guillian-Barré Syndrome, and seizure disorders.
● Diagnosis of sensory neuropathy J.-M. Leger Reference Center for Neuromuscular Diseases, Bâtiment Babinski, Salpêtrière hospital, Paris.
A number of disorders of the Peripheral Nervous System (PNS) can present with purely or predominant sensory symptoms and signs, mainly a subacute or chronic sensory ataxia. They mainly encompass the so-called sensory neuronopathy (ganglionopathy), which is associated with the involvement of dorsal root ganglion (DRG) sensory neurons, but also distal axonal polyneuropathy in its predomi-
nantly sensory presentation (small fiber sensory polyneuropathy), and less often demyelinating polyneuropathy, which may present with purely or predominantly sensory features. The 2 first types of sensory neuropathy have a relatively clear-cut clinical and electrophysiological profile. In the last one, the diagnosis may be easy when electrodiagnostic studies show typical abnormalities of motor nerve conduction velocities (MNCV), but be very difficult when these abnormaliies are lacking. Sensory neuronopathies are frequently associated with dysimmune or neoplastic diseases, and with the use of toxic agents. Small fiber sensory polyneuropathies are classically found in association with diabetes mellitus, alcohol abuse and nutritional deficiency, HIV infection and amyloidosis. In a number of cases, however, no etiology is found despite repeated investigations, which leads to define the specific subgroup of chronic idiopathic axonal polyneuropathy (CIAP). Lastly, sensory demyelinating polyneuropathies are mainly those associated with an anti-MAG IgM monoclonal gammopathy, but may be chronic inflammatory demyelinating polyneuropathies (CIDP). This review will focuse on recent advances in defining the clinical features, pathophysiological basis and management of these different sensory neuropathies.
● Sensory ataxic neuropathy J. Pouget, J.P. Azulay, S. Attarian, A. Verschueren, D. Uzenot Department of Neuromuscular Diseases, La Timone Hospital, 13005 Marseille.
Sensory ataxic neuropathies are uncommon disorders characterised by a predominant loss of large myelinated fibers which impairs kinaesthesia and leads to sensory ataxia and loss of tendon reflexes. Lesions concern peripheral nerves or dorsal root ganglions or dorsal roots. Clinical presentation of proprioceptive deafferentation concerns mainly motor than sensory symptoms. Sensory ataxia of gait must be distinguished from cerebellar gait ataxia and from the unsteadiness caused by weakness. Sensory ganglionopathy usually includes sensory ataxia of upper and lower limbs, a painful dysesthetic syndrome, widespread impairment of cutaneous sensation and some autonomic involvement. Causes of sensory ataxic neuropathy are multiple. Toxic, metabolic, infectious, dysimmune and hereditary etiologies have been reported. Toxics concern vitamin B6, cis-platinum and taxol and are easily recognised. Metabolic causes are vitamin B12 and vitamin E deficiency. Sensory ataxia has been described in association with celiac disease. Interest has recently developed concerning dysimmune origin with several newly described clinico-biological syndromes. Among IgM paraproteonaemic neuropathies, anti-MAG neuropathy is the best defined and most frequent. It has distinctive clinical, electrophysiological and pathological features that may distinguish it from CIDP and other IgM-related neuropathy. Several therapies have been tried with most often no effect
NEUROMÉDITERRANÉE VIII
Rev Neurol (Paris) 2007 ; 163 : 4, 483-492
social/vocational disability may be a greater problem than the seizures and may be related to the perceived social stigma associated with the diagnosis. Insufficient information about the disorder and inadequate professional support is common. Supportive cognitive therapy, specialist epilepsy nursing, and structured educational programmes are essential parts of an operative comprehensive epilepsy service. Intervention of this kind has demonstrated a beneficial effect on the quality of life in patients with active epilepsy. During recent years, it has been increasingly recognized that AEDs have différent pharmacodynamic properties and multiple applications. Some may have CNS side effects which may negatively influence affective disorders, whereas others have mood stabilizing or even thymoleptic or anxiolytic effects. It is important to be familiar with the various potential effects of the different treatment modalities, including vagal nerve stimulation. The therapy should be carefully tailored to the clinical profile of each patient. Both benefits and risks should be weighed when treating epileptic patients with antidepressants. Mutual pharmacokinetic and pharmacodynamic interactions should be considered. Compounds without known association with seizures should be preferred. SSRIs are usually safe and effective, but further research is needed to define optimal strategies. In mood disorders, attempts to analyze intrinsic neurobiological factors and the pharmacological profiles of prescribed AEDs, as well as individual psychosocial aspects should be performed. All these considerations have to be taken into account for the individual patient.
● Instructive cases from the neurology service S.I. Harik Professor and Chairman of Neurology, University of Arkansas for Medical Sciences, USA.
Several relatively difficult, yet instructive actual cases in clinical neurology were be presented. There are lessons to be learned from each case. Audience interaction is encouraged. The give and take that I hope will materialize will help focus on many important issues in clinical neurology that are illustrated by these cases. The subject matter of the cases include: stroke, central nervous system infections, brain tumors, IVIG treatment for Guillian-Barré Syndrome, and seizure disorders.
● Diagnosis of sensory neuropathy J.-M. Leger Reference Center for Neuromuscular Diseases, Bâtiment Babinski, Salpêtrière hospital, Paris.
A number of disorders of the Peripheral Nervous System (PNS) can present with purely or predominant sensory symptoms and signs, mainly a subacute or chronic sensory ataxia. They mainly encompass the so-called sensory neuronopathy (ganglionopathy), which is associated with the involvement of dorsal root ganglion (DRG) sensory neurons, but also distal axonal polyneuropathy in its predomi-
nantly sensory presentation (small fiber sensory polyneuropathy), and less often demyelinating polyneuropathy, which may present with purely or predominantly sensory features. The 2 first types of sensory neuropathy have a relatively clear-cut clinical and electrophysiological profile. In the last one, the diagnosis may be easy when electrodiagnostic studies show typical abnormalities of motor nerve conduction velocities (MNCV), but be very difficult when these abnormaliies are lacking. Sensory neuronopathies are frequently associated with dysimmune or neoplastic diseases, and with the use of toxic agents. Small fiber sensory polyneuropathies are classically found in association with diabetes mellitus, alcohol abuse and nutritional deficiency, HIV infection and amyloidosis. In a number of cases, however, no etiology is found despite repeated investigations, which leads to define the specific subgroup of chronic idiopathic axonal polyneuropathy (CIAP). Lastly, sensory demyelinating polyneuropathies are mainly those associated with an anti-MAG IgM monoclonal gammopathy, but may be chronic inflammatory demyelinating polyneuropathies (CIDP). This review will focuse on recent advances in defining the clinical features, pathophysiological basis and management of these different sensory neuropathies.
● Sensory ataxic neuropathy J. Pouget, J.P. Azulay, S. Attarian, A. Verschueren, D. Uzenot Department of Neuromuscular Diseases, La Timone Hospital, 13005 Marseille.
Sensory ataxic neuropathies are uncommon disorders characterised by a predominant loss of large myelinated fibers which impairs kinaesthesia and leads to sensory ataxia and loss of tendon reflexes. Lesions concern peripheral nerves or dorsal root ganglions or dorsal roots. Clinical presentation of proprioceptive deafferentation concerns mainly motor than sensory symptoms. Sensory ataxia of gait must be distinguished from cerebellar gait ataxia and from the unsteadiness caused by weakness. Sensory ganglionopathy usually includes sensory ataxia of upper and lower limbs, a painful dysesthetic syndrome, widespread impairment of cutaneous sensation and some autonomic involvement. Causes of sensory ataxic neuropathy are multiple. Toxic, metabolic, infectious, dysimmune and hereditary etiologies have been reported. Toxics concern vitamin B6, cis-platinum and taxol and are easily recognised. Metabolic causes are vitamin B12 and vitamin E deficiency. Sensory ataxia has been described in association with celiac disease. Interest has recently developed concerning dysimmune origin with several newly described clinico-biological syndromes. Among IgM paraproteonaemic neuropathies, anti-MAG neuropathy is the best defined and most frequent. It has distinctive clinical, electrophysiological and pathological features that may distinguish it from CIDP and other IgM-related neuropathy. Several therapies have been tried with most often no effect
NEUROMÉDITERRANÉE VIII