(PERONEAL TYPE) This condition, which is peculiar to childhood, has been known for a long time (liulenberg, 1856), but its accurate definition we owe to Charcot, Marie, Tooth and, particularly, Hoffmann and Bernhardt. Hoffmann originated the tern) progressive neurotic (or neural) muscular atrophy, while Bernhardt called the disease spinal neuritic atrophy. It is distinctly' hereditary or rather familial, and occurs chiefly in later childhood, somewhat more frequent in boys than in girls. The atrophy' appears first in the peroneal muscles or sometimes in the other muscle groups of the legs. There results a peculiar disturbance of the gait (stepping gait). The foot is raised very high, the toes are extended and point downward, and are the first to come in contact with the ground as the foot is put down. Sometimes a similar disease appears at the same time in the arms, particularly in the inuscles of the hands and in the extensors, producing the deformity' known as claw-hand. The paralysis sometimes extends to other muscles of the extremities, but the rest of the muscular system usually escapes. The patellar and Achilles tendon reflexes diminish as the disease goes on and usually disappear altogether. Fibrillary twitching in the diseased muscles or those about to be attacked is quite frequent. The electric irritability of the atrophic muscles is variable. As a rule the reactions of degenera tion are present, or there is complete absence of reaction to either the galvanic or faradic current. Sometimes muscles that are still perfectly capable of functionating, and are not atrophic, exhibit marked changes in their electric behavior. Sensory disturbances are not rare in neural muscular atrophy and manifest themselves in attacks of pain, hyper aisthesia, or diminution of the tactile and pain sense. Vasomotor phenomena have also been observed. The disease is extremely slow and often does not progress for years beyond paralysis of the legs. The
progress of the disease may be interrupted at any time by remissions and exacerbations (according to Goldenberg always in the spring).
The pathology of the progressive muscular atrophy has never been fully cleared up. According to Hoffmann's view the peripheral nerves appear to take an active part in the disease process, for they are found to be distinctly degenerated. But the spinal cord also shows changes which can be attributed only in part to the primary nervous disease. Hence, it is at least a question whether both spinal or neural changes may' not take part simultaneously in the pathology of this disease. For this reason Bernhard designated the disease "spinal neuritic atrophy." If we include the eases in which a primary muscular change must be assumed (myositic form of progressive muscular atro PhY), si-e have sufficient proof that the original expectation of finding in this form of muscular atrophy a disease which should form a neuro genic connecting link between the myelogenic and the myogenic forms has not been realized.
A deviation from the normal type of this disease is found in a conclition first described by Dejerine and Sottas and carefully studied by Brasch. In the main this condition resembles the above-described form of the disease, with the addition of pupillary rigidity, miosis, Romberg's sign, ataxia and other symptoms. The two French authors demonstrated both clinically and pathologically a thickening of the nerve trunks in this, disease, which they accordingly named interstitial hypertrophic progressive neuritis. But the pathology of these cases is still in doubt, and most authors include them under the head of neural muscular artophy, the association of symptoms belonging to hereditary ataxia being a feature of great interest.