Spinal Internuncial Neurones in Progressive Encephalomyelitis with Rigidity

Overview

Journal J Neurol Neurosurg Psychiatry

Publisher BMJ Publishing Group

Specialties Neurology
Neurosurgery
Psychiatry

Date 1979 Sep 1

PMID 501376

Citations 28

Authors

D A Howell

A J Lees

P J Toghill

Affiliations

Soon will be listed here.

Abstract

The clinical and pathological features of a fourth patient with progressive encephalomyelitis with rigidity are reported and compared with those previously described. It is suggested that the muscular rigidity, abnormal postures, painful muscular spasms, and myoclonus are a product of excessive and abnormal discharges of alpha motor neurones caused by their release from control by spinal internuncial neurones. A count of neuronal perikarya in the ventral horns confirmed that the disease selectively destroyed small and medium sized neurones, most of which were spinal internuncial neurones. Experimental, clinical, and pathological evidence concerning spinal internuncial neurones is reviewed and found to conform to this theory. The pathogenesis of opsoclonus may be similar.

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References

MOERSCH F, WOLTMAN H . Progressive fluctuating muscular rigidity and spasm ("stiff-man" syndrome); report of a case and some observations in 13 other cases. Proc Staff Meet Mayo Clin. 1956; 31(15):421-7. View

Murayama S, Smith C . RIGIDITY OF HIND LIMBS OF CATS PRODUCED BY OCCLUSION OF SPINAL CORD BLOOD SUPPLY. Neurology. 1965; 15:565-77. DOI: 10.1212/wnl.15.6.577. View

GELFAN S, TARLOV I . ALTERED NEURON POPULATION IN L7 SEGMENT OF DOGS WITH EXPERIMENTAL HIND-LIMB RIGIDITY. Am J Physiol. 1963; 205:606-16. DOI: 10.1152/ajplegacy.1963.205.3.606. View

Swanson P, LUTTRELL C, Magladery J . Myoclonus--a report of 67 cases and review of the literature. Medicine (Baltimore). 1962; 41:339-56. DOI: 10.1097/00005792-196212000-00003. View

RUSHWORTH G, Lishman W, Hughes J, Oppenheimer D . Intense rigidity of the arms due to isolation of motoneurones by a spinal tumour. J Neurol Neurosurg Psychiatry. 1961; 24:132-42. PMC: 495377. DOI: 10.1136/jnnp.24.2.132. View

PENRY J, Hoefnagel D, Van Den Noort S, DENNYBROWN D . Muscle spasm and abnormal postures resulting from damage to interneurones in spinal cord. Arch Neurol. 1960; 3:500-12. DOI: 10.1001/archneur.1960.00450050020004. View

GELFAN S, TARLOV I . Interneurones and rigidity of spinal origin. J Physiol. 1959; 146(3):594-617. PMC: 1357053. DOI: 10.1113/jphysiol.1959.sp006214. View

LUTTRELL C, Bang F, LUXENBERG K . Newcastle disease encephalomyelitis in cats. II. Physiological studies on rhythmic myoclonus. AMA Arch Neurol Psychiatry. 1959; 81(3):285-91. DOI: 10.1001/archneurpsyc.1959.02340150017002. View

BROOKS V, Curtis D, ECCLES J . The action of tetanus toxin on the inhibition of motoneurones. J Physiol. 1957; 135(3):655-72. PMC: 1358940. DOI: 10.1113/jphysiol.1957.sp005737. View

10.

Campbell A, Garland H . Subacute myoclonic spinal neuronitis. J Neurol Neurosurg Psychiatry. 1956; 19(4):268-74. PMC: 497217. DOI: 10.1136/jnnp.19.4.268. View

11.

COGAN D . Ocular dysmetria; flutterlike oscillations of the eyes, and opsoclonus. AMA Arch Ophthalmol. 1954; 51(3):318-35. DOI: 10.1001/archopht.1954.00920040324006. View

12.

Kawamura Y, OBrien P, Okazaki H, Dyck P . Lumbar motoneurons of man II: the number and diameter distribution of large- and intermediate-diameter cytons in "motoneuron columns" of spinal cord of man. J Neuropathol Exp Neurol. 1977; 36(5):861-70. DOI: 10.1097/00005072-197709000-00010. View

13.

Kawamura Y, Okazaki H, OBrien P, Dych P . Lumbar motoneurons of man: I) number and diameter histogram of alpha and gamma axons of ventral root. J Neuropathol Exp Neurol. 1977; 36(5):853-60. DOI: 10.1097/00005072-197709000-00009. View

14.

Whiteley A, Swash M, Urich H . Progressive encephalomyelitis with rigidity. Brain. 1976; 99(1):27-42. DOI: 10.1093/brain/99.1.27. View

15.

Hardin J, Griggs R . Diazepam treatment in a case of strychnine poisoning. Lancet. 1971; 2(7720):372-3. DOI: 10.1016/s0140-6736(71)90085-7. View

16.

Curtis D, de Groat W . Tetanus toxin and spinal inhibition. Brain Res. 1968; 10(2):208-12. DOI: 10.1016/0006-8993(68)90123-6. View

17.

LHERMITTE F, Chain F, ESCOUROLLE R, Chedru F, Guilleminault C, Francoual M . [A further case of tetanus-like contracture distinct from the stiff man syndrome. Pharmacological and neuropathological study of a case of predominantly spinal encephalomyelitis]. Rev Neurol (Paris). 1973; 128(1):3-21. View

18.

Curtis D, Felix D, Game C, McCulloch R . Tetanus toxin and the synaptic release of GABA. Brain Res. 1973; 51:358-62. DOI: 10.1016/0006-8993(73)90389-2. View

19.

GORDON E, Januszko D, Kaufman L . A critical survey of stiff-man syndrome. Am J Med. 1967; 42(4):582-99. DOI: 10.1016/0002-9343(67)90057-5. View

20.

Kasperek S, ZEBROWSKI S . Stiff-man syndrome and encephalomyelitis. Report of a case. Arch Neurol. 1971; 24(1):22-30. DOI: 10.1001/archneur.1971.00480310050004. View