Marie Neuritis

MARIE NEURITIS, syn. Marie-Boveri neuritis (Marie, Boveri), a type of familial hypertrophic neuritis, described by Marie in 1906 as "forme speciale de nevrite interstitielle et progressive de l'enfance." In etiology, heredity is of primary importance, and the disease does not manifest in its full form in all cases, but is often expressed only abortively. At autopsy, changes are found in the spinal cord (in the posterior columns, in the pyramidal tracts, in the Flechsig tracts) and typical changes in the peripheral nerves. The latter are thickened, the axis cylinders show phenomena of degeneration in various stages; sleeve-like neoplasms are characteristic, consisting of cells and fibers, which are probably the product of the proliferation of Schwann sheaths and their nuclei. Bielschowsky considers these neoplasms to be a "peritubular form of neurinomatosis" and views the entire disease as a peculiar subform of Recklinghausen's disease. The course of the disease is very chronic, slowly progressive, often with long periods of arrest. The symptomatology is complex. The most characteristic symptom is the thickening of the peripheral nerves, clearly visible to the eye and upon palpation. Next, one should mention the absence of tendon reflexes, muscular atrophy in the lower extremities with a strong decrease in excitability to galvanic and faradic currents, sensory disturbances, bone changes (kyphoscoliosis and pes varo-equinus excavatus), intention tremor, and scanning speech. The latter two symptoms give the clinical picture of Marie-Boveri disease a resemblance to the clinical picture of multiple sclerosis and distinguish this form from Dejerine-Sottas hypertrophic neuritis, in which, however, there are changes in the pupils, as well as shooting pains. However, it is necessary to keep in mind that all described cases of Marie-Boveri hypertrophic neuritis concerned only one family and even only one generation of siblings in this family. Thus, it is not yet possible to say whether the Marie-Boveri clinical type is an accidental variation of Dejerine-Sottas hypertrophic neuritis or an independent form, which can be resolved only by further observations. The pathological-anatomical picture in Marie-Boveri neuritis does not differ in any way from the pathological-anatomical picture in Dejerine-Sottas neuritis. The genetics of the family studied by Marie-Boveri have not been well investigated, so it is still impossible to comment on one or another type of hereditary transmission in this disease. The prognosis regarding a cure is hopeless. Rational therapy, as with all other hereditary diseases of the nervous system, does not exist. Desirable preventive measures follow from the etiology of the disease.