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Lower Motor Neuron Syndrome 982 Journal ofNeurology, Neurosurgery, and Psychiatry 1993;56:982-987 Motor conduction block and high titres of anti- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.9.982 on 1 September 1993. Downloaded from GM1 ganglioside antibodies: pathological evidence of a motor neuropathy in a patient with lower motor neuron syndrome David Adams, Thierry Kuntzer, Andreas J Steck, Alexander Lobrinus, Robert C Janzer, Franco Regli Abstract and temporal dispersion included a more A patient with a progressive lower motor than 20% reduction in the negative CMAP neuron syndrome had neurophysiologi- amplitude and an increase in the negative cal evidence of motor axon loss, multi- duration of no more than 15% of the maxi- focal proximal motor nerve conduction mum CMAP elicited by stimulation at the block, and high titres of anti-ganglioside proximal site compared with that evoked by GM1 antibodies. Neuropathological find- distal stimulation.12 In cases where stimula- ings included a predominantly proximal tion could not be applied proximally, a con- motor radiculoneuropathy with multi- duction block was admitted when the number focal IgG and IgM deposits on nerve of motor unit potentials evoked by graded fibres associated with a loss of spinal stimulation was much greater than could be motor neurons. These findings support obtained during maximal voluntary contrac- an autoimmune origin of this lower tion or when the total area of the CMAP was motor neuron syndrome with retrograde greater than the sum of the areas of the indi- degeneration of spinal motor neurons vidual motor unit potentials."3 Motor nerve and severe neurogenic muscular atrophy. conduction studies were measured over long segments at conventional sites (Erb's point, (J Neurol Neurosurg Psychiatry 1993;56:982-987) axilla, elbow, wrist) and also over short seg- ments for the ulnar nerve. F wave latencies from at least 10 responses were recorded fol- Interest in the pathological role of anti-gan- lowing stimulation at the wrist. Using surface glioside antibodies has been stimulated by the electrodes, sensory nerve action potentials identification of monoclonal IgM antibodies were recorded orthodromically. Needle reacting with specific gangliosides in patients examination was performed using standard with motor neuron diseases and monoclonal Dantec concentric electrodes. gammopathy.l1 Polyclonal anti-GMl anti- bodies have also been found in the absence of ENZYME LINKED IMMUNOSORBENT ASSAY a gammopathy in patients with motor neuron The presence of anti-ganglioside GMl anti- syndromes, particularly in a subgroup of bodies in serum was measured by the enzyme patients with multiple motor neuropathy and linked immunosorbent assay (ELISA) using a lower motor neuron syndrome.5-9 The pres- technique previously described.8 The activity ence of these antibodies raises the possibility against other gangliosides GM2, GM3, http://jnnp.bmj.com/ of an ongoing autoimmune process in GD1a, GDlb, asialo-GMl was also evaluat- patients with motor neuron disease. In a few ed. The purified gangliosides were a kind gift of these patients improvement in strength has of Dr A Martelli (Fidia Research Lab- occurred after reduction of antibody titres by oratories, Abano Terme, Italy). treatment with immunosuppressive drugs.5910 Anti-GM1 antibodies may bind to the nodes PATHOLOGICAL STUDY of Ranvier," but the of this anti- Brain, spinal cord and roots, several peripher- significance on October 2, 2021 by guest. Protected copyright. body binding pattern and its role in nerve al nerves, and muscles were fixed for three weeks in a Centre Hospitalier conduction block remain to be determined.9 10% buffered paraformaldehyde Universitaire Vaudois, We report the clinical, immunological, physi- solution. Forty samples from relevant regions Lausanne, ological and pathological findings in a patient were routinely processed. Paraffin embedded Switzerland with lower motor neuron syndrome and a and frozen sections were stained with haema- Service de Neurologie toxylin and eosin, van Gieson and Luxol and D Adams high titre of anti-ganglioside GM1 antibodies. T Kuntzer Nissl and Luxol stains. Additionally, muscles A J Steck were stained with periodic acid Schiff, retic- F Regli Methods ulin, Congo red, muscle enzyme, and lipid Institut Universitaire NEUROPHYSIOLOGY stains. Immunochemical stains were per- de Pathologie studies were formed with the A Lobrinus Electrophysiological performed following commercial avail- R C Janzer using a Mystro system (Vickers Medical able antibodies against phosphorylated Correspondence to: Corp). Multisegmental motor nerve conduc- neurofilaments, ubiquitin, glial fibrillary acidic Professor A Steck, Service tion studies of both arms were performed protein (GFAP), lysozyme, myelin basic pro- de Neurologie, CHUV, 1011 Lausanne, with percutaneous supramaximal stimulation tein (MBP) and myelin associated glycopro- Switzerland. while recording the compound muscle action tein (MAG), and anti-IgG, IgM, and IgA Received 12 February 1992 potential (CMAP) with TECA 4 mm surface antibodies. Samples of spinal roots were also and in final revised form 8 December 1992 disc electrodes and needle electrodes (radial postfixed in glutaraldehyde, epon embedded, Accepted 11 December 1992 nerve). Determination of conduction block and lpum sections stained with toluidine blue. Motor neuropathy and anti-GM1 ganglioside antibodies 983 Case report nal and respiratory muscles, but the cranial J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.9.982 on 1 September 1993. Downloaded from A 71 year old man first experienced painful nerves remained normal. There was no senso- cramps in the thighs and calves on exertion at ry deficit or symptoms. Deep tendon reflexes the end of 1983. Past medical history was not were all absent, plantar responses were neu- relevant and there was no family history of tral, and no sphincter dysfunction or fascicu- similar illness. Over the ensuing three years, lations were seen. On general examination the patient-developed progressive paraparesis, diffuse oedema of the four limbs was noted. at first characterised by asymmetrical (left Biochemical studies were within the normal more than right) difficulties in climbing stairs; range, including serum electrophoresis and then a bilateral foot drop appeared. In 1985, immunoelectrophoresis, triiodothyronine, thy- he needed a stick to walk; in summer 1986 he roxine thyroid stimulating hormone, and could only walk with the assistance of two immunoelectrophoresis. Serological tests for canes. Over the next six months, the weakness syphilis, anti-DNA antibodies, CSF, and extended to the hands and he fell frequently. blood Lyme serology were normal or negative. In December 1986 on examination the cranial The patient was treated with chlorambucil, nerves were normal but there was a moderate 9 mg daily but no improvement was observed atrophy of shoulders and intrinsic hand mus- over the next four months. Death occurred six cles and diffuse wasting of the lower limbs. years after onset. The necropsy was per- Fasciculations were observed in proximal formed 48 hours after death. muscles. The muscle strength was weak and asymmetrical, left side more than the right side, MRC Grade 3 + to 4/5 distally, 4 + /5 Results proximally in the upper limbs, and 2 to 4/5 NEUROPHYSIOLOGY distally, 2 to 3/5 proximally in the lower The results of the nerve conduction studies limbs. Sensory responses to light touch, pin- are summarised in the table. There were prick, vibration, and joint position sense were motor conduction blocks in four nerves out of normal. Deep tendon reflexes were absent in the eight tested on both sides, all of them the left leg and present but decreased in the located at the right Erb's point. The degree of other limbs. Plantar responses were neutral on the block was more than 50% for the radial the right side and questionable on the left. A and ulnar nerves. For the axillary and muscu- general examination revealed oedema of the locutaneous nerves the number of motor unit legs up to the knees. There was physiological potentials evoked by graded stimulation was evidence of diffuse denervation reinnervation greater than could be obtained during maxi- by needle EMG. The distal motor and senso- mal voluntary contraction. The blocks were ry nerve conduction velocities and amplitudes associated with a low distal amplitude of the of lower and upper limb sensory nerve action CMAP, expressing axonal loss. Despite the potentials were normal. A diagnosis of proba- low distal amplitude which leads to difficulty ble motor neuron disease was made. in measuring the block, the proximal and dis- The weakness progressed in the arms and tal responses were of the same shape and he was found to be tetraplegic in October could be superimposed: the difference in 1988. In November 1989 he was found to duration of CMAPs did not exceed 10% have high anti-ganglioside GM1 antibodies between distal and proximal responses. The and was reinvestigated. Examination revealed differences in amplitude were thus not simply a severe hypotonic tetraplegia: in his arms and due to increased dispersion of CMAPs. The http://jnnp.bmj.com/ legs, only slight fingers and toes flexion could conduction velocity for the remaining nerve be seen. He also had weakness of the abdomi- fibres was nearly normal or diminished by less Table Results ofconduction studies in motor and sensoryfibres Amplitude (i1W) Distal latency Ratio proximal/distal Conduction velocity on October 2, 2021 by guest. Protected copyright. Nerve Proximal Distal (ms) amplitude (%) (range, mls) Motor: Right ulnarl* 0 310 5 0 44-55 7 Right radial2* 20 110 6-4 18-1 39-45 Right musculocutaneous* 640 ND 6-2 ND ND Right axillary* 300 ND 7-4 ND ND Right median ND 0 Left median3 150 180 5-6 83 35-8-46 Left musculocutaneous 0 ND Left ulnar' 160 180 6-4 88 38-45 Sensory (right side): Median digit I to wrist 8-4 3 45 Median palm to wrist 28-5 1-5 53-3 Ulnar digit V to wrist 6 3 45 Radial digit I to wrist 7-9 3 41-6 *Conduction block at Erb's point (see discussion).
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