MORVAN'S DISEASE

Synonyms.—Syringomyelia; Myelosyringosis; Analgesic paralysis with whitlow;
Fr., Panaris analgésique; Maladie de Morvan; Ger., Morvansche Krankheit.

Definition.—An affection of the spinal cord, with peripheral
symptoms, chiefly of the upper extremities, and characterized by mus­
cular atrophy and trophic disturbances, partial anesthesia, and the oc­
currence of whitlows, cutaneous ulceration, and necrosis.

This disease was first described by Morvan,² in the year 1883, under
the title “De la parésie analgésique à panaris des extrémités supérieures
ou pareso-analgésie des extremités supérieures,” and regarded by him as
being a special disease, independent of syringomyelia. The present
acceptance, on the whole, however, is to regard this condition as a type
of the latter, a view adopted by Charcot, and also previously by Roth,³
and the correctness of which is confirmed by the histologic findings of
Joffroy and Achard⁴ and others.

Symptoms.—The disease usually begins insidiously, with pain in
an arm, accompanied by loss of muscular power, which is succeeded
by analgesia and the occurrence of recurrent whitlows, in some in­
stances occurring in one or several crops. In some cases analgesia is

¹ Treves, “Treatment of Perforating Ulcer of the Foot,” Lancet, 1884, ii, p. 949.

² Morvan, Gazette hebdom., 1883, pp. 580, 590, 624, and 721.

³ Roth, “Contribution a l‘étude symptomatologique de la gliomatose médullaire,”
Arch, de neurologie, 1888, p. 161, records a number of cases.

⁴ Joffroy and Achard, “Un cas de maladie de Morvan avec autopsie,” Arch, de
méd, exper., 1890, p. 540 (with histologic and other illustrations); see also paper by
Joffroy, “Syringomyelie et maladie de Morvan,” Le Progrés méd., 1890, vol. xii, p. 51
(regarding identity and histologic findings).

632                                           ATROPHIES

the first symptom which attracts the patient's attention. One or several
whitlows may present at the one time, and in the course of the disease
as many as nine have been known to occur (Morvan). They may suc­
ceed one another rapidly, or several years may intervene between their
appearance. They usually are seen on the fingers only, but occasionally
the same condition has been noted on the toes. The phalanges become
necrosed and drop off, the hand generally becomes claw-like, great de­
formity resulting. Trophic and vasomotor cutaneous disturbances,
blueness of the skin, fissures, vesicles, bullæ, ulceration involving the
sheath of the tendons, changes in the nail substance, pigmentary changes,
and glossy skin are quite frequently also observed. Other symptoms
are hyperidrosis, more or less loss of tendon-reflexes, impaired vision,
scoliosis, and arthropathies. Atrophy of the muscles of the hand and
flexor muscles of the wrist and paresis result; the sensations of heat, cold,
and pain are lost, although the sense of touch remains.

Ordinarily the upper extremities are alone involved, and it may be
confined to one, but usually first one and then the other becomes affected.
In other rare instances one leg and foot show the changes, or it may be
in both lower extremities, and exceptionally it has involved both arms
and legs. A rare instance is also reported by Jacquet,¹ in which trophic
ulcerations were on the neck, head, and shoulders. The malady is not
only insidious, but often extremely slow in its progress, in some instances,
as in Prouff's² cases, lasting many years, sometimes with more or less
prolonged remissions.

Etiology and Pathology.—This rare affection occurs more
frequently in males, and with occasional exception between the ages
of twenty and fifty. Some of the cases are seemingly attributable to
traumatism, malaria, syphilis, rheumatism, and other constitutional
diseases, but it is to be said that these are simply possible factors, and
that the underlying cause, in the great majority of instances, is wholly
obscure, and certainly cannot be demonstrated. Its resemblance to
anesthetic leprosy has been remarked upon, and Zambaco³ believes it
to be a slight or modified form of that disease. Dyer⁴ states that, except
the absence of bacillus, it has no distinguishing features from some of the
mild types of lepra, and reports a few cases in point.

Pathologically, the malady is apparently of central spinal origin.
According to Gombault, Joffroy, Church, Marinesco, Dercum and
Spiller, and Starr, cavities surrounded by dense neuroglia are found
behind the central canal, and are believed to be due to absorption of
gliomata. Sclerosis of the posterior horns and columns of the cord has

¹ Jacquet, part vi, plate xviii, International Atlas, 1891.

² Prouff, Gaz. hebdom., 1887, p. 249 (lasted over forty years).

³Zambaco, Trans, of First Internat. Leprosy Conference, Berlin, 1898; see also his
paper, “Lepra anesthetique et syringomyelie,” Gaz. hebdom., 1891, vol. xxviii, p. 196.

⁴ Dyer, “Syringomyelia and Lepra nervorum,” New Orleans Med. and Surg. Jour.,
1893-94, vol. xxi, p. 81; see also Cagney, “Syringomyelia and Leprosy,” Brit. Jour.
Derm., 1894, p. 375; and Jeanselme‘s paper, “La Lepre,” La Presse méd., 1897, Nos.
84 and 85 (containing suggestive cases, with illustrations); also von Düring, “Die
Schwierigneiten in der Diagnose nervösen Lepraformen, insbesondere in Beziehung auf
die Syringomyelie,” Archiv, 1898, vol. xliii, p. 137; Pick‘s Fetschrift, part i (with 3
plates and references).

MORVAN‘S DISEASE

633

also been noted. Gombault and Joffroy¹ examined the peripheral
nerves and found sclerosis and neuritis. Sachs and Armstrong² suggest
that the earliest pathologic process may be seated in the peripheral
nerves, and that the central changes are secondary. This would be in
consonance with the belief that some cases seem to follow a local
peripheral traumatism. Both the pathologic findings and clinical
symptoms are in accord with the view that the malady is identical with
syringomyelia.

Diagnosis.—As already remarked, the clinical features of the
disease at times bear a close resemblance to some cases of leprosy of
the anesthetic type; but the absence of the bacillus, as well as other
differences, are usually sufficient to distinguish. There is occasionally
a slight similarity in some of the hand symptoms in certain cases of sclero-
derma, but that is the whole extent of the resemblance, so that a mistake
is scarcely possible if the usual and more extensive symptoms of sclero-
derma are kept in view.³

Prognosis and Treatment.—The outlook for a cessation or
cure of the malady is not promising. Its progress, as already stated,
is slow, ten, fifteen, sometimes more years elapsing before serious con­
sequences, as regards life, ensue. The resulting deformity and mutila­
tion are gradual. Treatment is essentially symptomatic. The parts
are to be protected against injury, and the whitlows and ulcerations
treated upon ordinary antiseptic surgical principles. The patient‘s
general health should receive attention. Arsenic, strychnin, silver
nitrate, and other remedies have been tried, but no estimation as to
their possible value can be definitely stated. Application of the galvanic
current up and down the spine, and also along the main peripheral trunks
and branches, has been suggested as of benefit.⁴

¹ Gombault, in Monod and Reboul‘s paper, Arch. gen. de méd., 1888, ii, p. 28;
ProufT and Gombault, “Un cas de mal. de Morvan suivi d‘autopsie,” Gaz. hebdom.,
1889, pp. 308 and 318; Joffroy and Achard, “Un cas de mal. de Morvan aved autopsie,”
Arch, de med. exper., 1890 (with 7 histologic cuts).

² Sachs and Armstrong, “Morvan‘s Disease,” New York Med. Jour., 1892, vol. lv,
p. 482 (with bibliography).

³ Pospelow, in a recent valuable exhaustive paper, “Trophische Störungen der
Haut bei spinaler Gliomatose oder Syringomyelie,” Archiv (Pick‘s Festschrift, part ii),
1898, vol. xliv, p. 91, believes, from his study of the subject, that scleroderma is occa­
sionally associated with the malady.

⁴ Other valuable contributions on the disease are: Bruhl, Contribution a l'étude de
la syringomyelie, Paris, 1890 (an exhaustive monograph with bibliography); Thibiérge,
“Les alterations cutanées de la syringomyelie,” Annales, 1890, p. 799; Schlesinger,
Die Syringomyelie, Liepzig and Vienna, 1895 (a complete exposition with bibliography).

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