There remains to be considered a neuropathic syndrome that almost certainly is nutritional in origin but does not conform clinically to beriberi or pellagra, the classic deficiency diseases. This syndrome was originally observed by Strachan in 1897 among Jamaican sugarcane workers. The main symptoms in his patients were pain, numbness, and paresthesias of the extremities; objectively there was ataxia of gait, weakness, wasting, and loss of deep tendon reflexes and sensation in the limbs. Dimness of vision and impairment of hearing were common findings, as were soreness and excoriation of the mucocutaneous junctions of the mouth. This disorder, originally known as “Jamaican neuritis,” was quickly recognized in other parts of the world, particularly in the undernourished populations of tropical countries. Subsequently, many cases of this syndrome were observed in the besieged population of Madrid during the Spanish Civil War and during World War II among prisoners of war in North Africa and the
Far East.
The clinical descriptions from these varied sources are not entirely uniform, but certain features are common to all of them and occur with sufficient frequency to allow the delineation of the neurologic syndrome; it appears to be almost identical to the one described by Strachan. The core disorder is combined optic and peripheral neuropathy. The latter consists mainly of sensory symptoms and signs, and the former of the subacute evolution of failing vision, which, if untreated, progresses to complete blindness and pallor of the optic discs. Deafness and vertigo are uncommon, but in some outbreaks among prisoners of war, these symptoms were frequent enough to earn the epithet “camp dizziness.” In all these respects, the syndrome differs from beriberi. Along with the neurologic signs there may be varying degrees of stomatoglossitis, corneal degeneration, and genital dermatitis (the orogenital syndrome). The mucocutaneous lesions are unlike those of pellagra and riboflavin deficiency.
There have been only a few neuropathologic studies of this syndrome. Aside from the changes in the papillomacular bundle of the optic nerve, which are similar to the deficiency amblyopia discussed previously, the most consistent abnormality has been a loss of myelinated fibers in each column of Goll adjacent to the midline. Fisher interpreted this change to indicate a degeneration of the central processes of the bipolar sensory neurons of the dorsal root ganglia (i.e., the dorsal roots). The fact that the primary sensory neuron is the main site of the neuropathic disorder is consistent with the predominantly sensory symptomatology. We find it difficult to draw a sharp dividing line between the nutritional peripheral (and optic) neuropathy described previously and the Strachan syndrome.
This term refers to a common and uniform type of degeneration of the vermian and anterior lobes of the cerebellum in alcoholics. Its incidence was in the past about twice that of Wernicke disease, and like the latter, it is considerably more frequent in men than in women. It is characterized clinically by a wide-based stance and gait, varying degrees of instability of the trunk, and ataxia of the legs, the arms being affected to a lesser extent and often not at all. Nystagmus and dysarthria are infrequent. In addition to an ataxic (intention) tremor, there may be a tremor of the fingers or hands resembling 1 of the 2 types of parkinsonian tremor but appearing only when the limbs are placed in certain sustained postures. Mauritz and coworkers demonstrated that the instability of the trunk in these cases consists of a specific 3-Hz rhythmic swaying in the anteroposterior direction; by contrast, patients with lesions of the cerebellar hemispheres show only slight postural instability without directional preponderance.