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Quantitative evaluation of the effects of acetazolamide in Friedreich's ataxia: A pilot study

Published online by Cambridge University Press:  18 September 2015

C.L. Richards
Affiliation:
Centre de Recherche en Neurobiologie, Département des Sciences Neurologiques, Hôpital de l'Enfant-Jésus and Ecole de Réadaptation, Université Laval
J.P. Bouchard
Affiliation:
Centre de Recherche en Neurobiologie, Département des Sciences Neurologiques, Hôpital de l'Enfant-Jésus and Ecole de Réadaptation, Université Laval
F. Dumas
Affiliation:
Centre de Recherche en Neurobiologie, Département des Sciences Neurologiques, Hôpital de l'Enfant-Jésus and Ecole de Réadaptation, Université Laval
D. Tardif
Affiliation:
Centre de Recherche en Neurobiologie, Département des Sciences Neurologiques, Hôpital de l'Enfant-Jésus and Ecole de Réadaptation, Université Laval
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Abstract

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We evaluated the effects of acetazolamide in 4 young patients with Friedreich's ataxia by clinical and quantitative laboratory methods. Dynamic muscle function of the lower extremity was measured during isokinetic knee movements and gait. The acetazolamide trial was terminated at 7 to 11 weeks because of reported side effects or increased ataxia in 3 of the patients. The quantitative evaluations revealed lower dynamic strength values and alterations in the gait movement pattern in all patients. These changes, which were interpreted as deterioration, were partially reversible with cessation of acetazolamide. The advantages of such quantitative evaluations of dynamic muscle function in the evaluation of therapy in Friedreich's ataxia are discussed.

Type
A—Clinical Studies
Copyright
Copyright © Canadian Neurological Sciences Federation 1984

References

Barbeau, A (1978) Emerging treatments. Replacement therapy with choline or lecithin in neurological disease. Can J Neurol Sci 5: 157160.CrossRefGoogle ScholarPubMed
Barbeau, A (1979) Lecithin in movement disorders. In: Nutrition and the Brain. Vol 5, Barbeau, A, Growdon, HH, Wurtman, RJ (eds) New York, Raven Press, pp 263271.Google Scholar
Barbeau, A (1982) Friedreich’s disease 1982: Etiologic hypotheses. A personal analysis. Can J Neurol Sci 9: 243263.CrossRefGoogle ScholarPubMed
Barbeau, A, Butterworth, RF, Ngo, T, Breton, G, Melancon, S, Shapcott, D, Geoffrey, G, Lemieux, B (1976) Pyruvate metabolism in Friedreich’s ataxia. Can J Neurol Sci 3: 379388.CrossRefGoogle ScholarPubMed
Barbeau, H, Richards, CL, Bédard, PJ (1982) Action of cyproheptadine in spastic paraparetic patients. J Neurol Neurosurg Psychiatry, 45: 923926.CrossRefGoogle ScholarPubMed
Blass, JP, Kark, RAP, Menon, NK, Harris, SE (1976) Low activities of pyruvate and oxoglutarate dehydrogenase complex in five patients with Friedreich’s ataxia. N Engl J Med 295: 6267.CrossRefGoogle ScholarPubMed
Bogardh, E, Richards, CL (1974) Gait analysis and re-learning of gait control in hemiplegic patients. In: Seventh International Congress, World Confederation for Physical Therapy, Montreal, pp 443453.Google Scholar
Bouchard, JP, Roberge, C, Van Gelder, NM, Barbeau, A (1984) Familial periodic ataxia responsive to acetazolamide. Can J Neurol Sci (this issue).CrossRefGoogle ScholarPubMed
Brooks, VB, Thach, WT (1981) Cerebellar control of posture and movement. In: Handbook of Physiology — The Nervous System II, vol 2, Part 2, Brooks, VB (ed). American Physiological Society, Bethesda, Maryland, pp 877946.Google Scholar
Chamberlain, S, Robinson, N, Walker, J, Smith, C, Benton, S, Kennard, C, Swash, M, Kilkenny, B, Brandbury, S (1980) Effect of lecithin on disability and plasma free-choline levels in Friedreichs ataxia. J Neurol Neurosurg Psychiatry 43: 843845.CrossRefGoogle Scholar
De Smet, Y, Mear, JY, Tell, G, Schechter, PJ, Lhermitte, F, Agid, Y (1982) Effect of gamma-vinyl GABA in Friedreich’s ataxia. Can J Neurol Sci 9: 171174.CrossRefGoogle ScholarPubMed
Engel, WK, Siddique, T,Nicoloff, JT(1983)TRH acutely in amyotrophic lateral sclerosis (ALS) patients causes increased mobility and strength, lessened spasticity, shivering, and tachypnea. Neurology, 33 (suppl 2): 120121, abstract.Google Scholar
Evans, OB, Kilroy, AW, Fenichel, GM (1978) Acetazolamide in the treatment of pyruvate dysmetabolism syndromes. Arch Neurol 35: 302305.CrossRefGoogle ScholarPubMed
Faden, Al, Jacobs, TB, Holaday, JW (1981) Thyrotropin-releasing hormone improves neurologic recovery after spinal trauma in cats. N Engl J Med 305: 10631067.CrossRefGoogle ScholarPubMed
Faden, Al, Hallenbeck, JM, Brown, CQ (1982) Nalaxone vs thyrotropinreleasing hormone in the treatment of experimental stroke. Neurology 32: 10831087.CrossRefGoogle Scholar
Geoffroy, G, Barbeau, A, Breton, G, Lemieux, B, Aubé M, Léger, C, Bouchard, JP (1976) Clinical description and roentgenologic evaluation of patients with Friedreich’s ataxia. Can J Neurol Sci 3:279286.CrossRefGoogle ScholarPubMed
Gransberg, L, Knutsson, E, Litton, JE (1980) A computer programmed system for the analysis of active and passive isokinetic movements. In: IEEE Frontiers of Engineering in Health Care, Washington, pp 292295.Google Scholar
Griggs, RC, Moxley, RT, Lafrance, RA, Mcquillen, J (1978) Hereditary paroxysmal ataxia: response to acetazolamide. Neurology 28: 12591264.CrossRefGoogle ScholarPubMed
Grillner, S (1975) Locomotion in vertebrates: central mechanisms and reflex interaction. Physiol Rev 55: 247304.CrossRefGoogle ScholarPubMed
Grimm, RJ (1983) Program disorders of movement. In: Advances in Neurology, Vol 39, Motor Control Mechanisms in Health and Disease, Desmedt, JE (ed). New York, Raven Press, pp 112.Google Scholar
Holmes, G (1939) The cerebellum of man. (The Hughlings Jackson memorial lecture). Brain 62: 130.CrossRefGoogle Scholar
Isaksson, Al, Knutsson, E (1980) Microcomputer implementation of gait examination in clinical routine. In: IEEE Frontier’s of Engineering and Health Care, Washington, pp 4245.Google Scholar
Kark, RAP, Blass, JP, Spence, MA (1977) Physostigmine in familial ataxias. Neurology 27: 7072.CrossRefGoogle ScholarPubMed
Knutsson, E (1981) Gait control in hemiparesis. Scand J Rehab Med 13: 101108.Google Scholar
Knutsson, E (1983) Analysis of gait and isokinetic movements for evaluation of antispastic drugs or physical therapies. In: Advances in Neurology, Vol, 39, Motor Control Mechanisms in Health and Disease, Desmedt, JE (ed) New York, Raven Press, pp 10131034.Google Scholar
Knutsson, E, Martensson, A (1976) Action of dantrolene sodium in spasticity with a low dependence of fusimotor drive. J Neurol Sci 29: 195212.CrossRefGoogle ScholarPubMed
Knutsson, E, Martensson, A (1980) Dynamic motor capacity in spastic paresis and its relation to prime mover dysfunction, spastic reflexes and antagonist co-activation. Scand J Rehab Med 12: 93106.Google Scholar
Knutsson, E, Richards, CL (1979) Different types of disturbed motor control in gait of hemiparetic patients. Brain 102: 405430.CrossRefGoogle ScholarPubMed
Legg, NJ (1978) Oral choline in cerebellar ataxia. Brit Med J 2: 1403.CrossRefGoogle ScholarPubMed
Legg, NJ (1979) Oral choline in cerebellar ataxia. Brit Med J 2: 133.CrossRefGoogle ScholarPubMed
Livingstone, IR, Mastaglia, FL, Pennington, RJT, Skilbeck, C (1981) Choline chloride in the treatment of cerebellar and spinocerebellar ataxia. J Neurol Sci 50: 161174.CrossRefGoogle ScholarPubMed
Melancon, JB, Vanasse, M, Geoffroy, G, Barabé, L, Proulx, A, Fontaine, G, Dallaire, L, Potier, M (1982) Oral lecithin and linoleic acid in Friedreich’s ataxia. II. Clinical results. Can J Neurol Sci 9: 155164.CrossRefGoogle ScholarPubMed
Murray, MP, Drought, AB, Kory, RC (1964) Walking patterns of normal men. J Bone Joint Surg 46-A: 335360.CrossRefGoogle ScholarPubMed
Murray, MP, Kory, RC, Sepic, S (1970) Walking patterns of normal women. Arch Phys Med Rehabil 51: 637650.Google ScholarPubMed
Nashner, LM, Grimm, RJ (1978) Analysis of multiloup dyscontrols in standing cerebellar patients. In: Progress in Clinical Neurophysiology, Vol. 4, Cerebral Control in Man: Long Loop Mechanisms, Desmedt JE (ed) Karger, Basel, pp 300319.Google Scholar
Normand, MC, Richards, CL, Filion, M, Dumas, F, Tardif, D (1984) A simplified method for tridemensional analysis of gait movements. In: Biomechanics, IX-A, Winter, D, Wells, R, Norman, R, Hayes, K and Patla, A (eds). Human Kinetics Publishers, Champaign, Illinois (in press).Google Scholar
Pentland, B, Martyn, CN, Steer, CR, Christie, JE (1981) Lecithin in Friedreich’s ataxia. Br Med J, 282: 11971198.CrossRefGoogle ScholarPubMed
Perry, J (1974) Kinesiology of lower extremity bracing. Clin Orthop Rel Res 102: 1831.CrossRefGoogle Scholar
Pourcher, E, Barbeau, A (1980) Field testing of an ataxia scoring and staging system. Can J Neurol Sci 7: 339344.CrossRefGoogle ScholarPubMed
Resnick, JS, Engel, WK, Griggs, RC, Stam, AC (1968) Acetazolamide prophylaxis in hypokalemic periodic paralysis. N Engl J Med 278: 582586.CrossRefGoogle ScholarPubMed
Richards, CL (1980) Dynamic Muscle Function in Human Normal, Pathological and Prosthetic Knee Joints. PhD Thesis. McGill University, Montreal, 1980.Google Scholar
Richards, CL (1981) Dynamic strength characteristics during isokinetic knee movements in healthy women. Physiother Can 33: 211.Google Scholar
Richards, CL, Bédard, PJ, Fortin, G, Malouin, F (1983) Quantitative evaluation of the effects of L-dopa in torsion dystonia: A case report. Neurology, 33: 10831087.CrossRefGoogle ScholarPubMed
Richards, CL, Bouchard, JP, Bouchard, R, Barbeau, H (1980) A preliminary study of dynamic muscle function in hereditary ataxia. Can J Neurol Sci 7: 367377.CrossRefGoogle ScholarPubMed
Richards, CL, Knutsson, E (1974) Evaluation of abnormal gait patterns by intermittent-light photography and electromyography. Scand J Rehab Med Suppl 3: 6168.Google ScholarPubMed
Simon, SR, Deutsch, SD, Nuzzo, RM, Mansour, MJ, Jackson, JL, Koskinen, M, Rosenthal, RK (1978) Genu recurvatum in spastic cerebral palsy J Bone Joint Surg 60-A: 822894.Google ScholarPubMed
Sobue, I, Muroga, T, Konagaya, M, Yamamoto, H, Mitsuma, T, Takayanagi, T (1980) Effects of thyrotropin-releasing hormone on ataxia inspinocerebellar degenerations. In: Spinocerebellar Degenerations. I Sobue (ed). University Park Press, Baltimore, pp 8392.Google Scholar
Sutherland, DH, Olshen, R, Cooper, L, Woo, Sly (1980) The development of mature gait. J Bone Joint Surg 62A: 336353.CrossRefGoogle ScholarPubMed
Van Gelder, NM (1983) Metabolic interactions between neurons and astroglia: glutamine synthetase, carbonic anhydrase and water balance. In: Basic Mechanisms of Neuronal Excitability. Jasper, HH and Van Gelder, NM, (eds) Alan R Liss, New York, pp 529.Google Scholar
Yoshida, M, Shimizu, N, Niijima, K, Mizuno, M (1980) Pathophysiology of ataxia and ocular disturbance in SCD with quantitative evaluation of TRH treatment. In: Spinocerebellar Degenerations. Sobue, I (ed). University Park Press, Baltimore, pp 251256.Google Scholar