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feed themselves Autonomic failure was another feature in a few, and one of ours became deaf Extensive examinations for an occult cancer, paraproteinemia, Sjogren disease, Refsum disease, auto immune diseases, and all potential causes of an ataxic neuropathy, proved to be frustratingly negative Of course, it is possible that some patients had an as yet undiscovered tumor Yet other instances have had all the features of a truncal-limb sensory neuropathy, with little or no ataxia and only muted re exes; these have had a more benign course but still no cause was found (why most of our patients have been male physicians, as reported by Romero, is entirely unclear) Illa and colleagues, in a review of 17 patients with idiopathic sensory ataxic neuropathy found antibodies against the ganglioside GD1 in only one case, and concluded that the majority were not due to an immunologic mechanism The motor nerve conduction studies have been normal or slightly impaired, while the sensory potentials were eventually lost (but they may at rst be normal) A puzzling feature in two patients has been an unexpected preservation of many sensory nerve potentials even after a year of illness In these cases the process presumably lay in the dorsal roots rather than in the ganglia In a few instances the MRI has shown a change in the posterior columns of the spinal cord, presumably secondary to root disease The spinal uid has generally contained a slightly elevated protein concentration with few or no cells, up to 18 /mm3 in our cases Pathologic examination of the sensory ganglia in a few cases has exposed an in ammatory process identical to that of Sjogren disease Our attempts at treatment using plasma exchanges, IVIG, corticosteroids, and immunosuppressive agents have been mostly unsuccessful Idiopathic Autonomic Neuropathy Under this term is collected a group of dysautonomias that on extensive evaluation cannot be attributed to diabetes or amyloidosis A fair number, almost half in the series of Suarez and colleagues, are acute in onset and conform to the pure pandysautonomia condition described by Young and colleagues and discussed earlier as a variant of GBS The others, however, pursue a subacute or chronic course and about one quarter of these has an associated serum antibody that is directed against the acetylcholine receptor of sensory ganglia (Klein et al) Orthostatic hypotension is the leading feature and in those with the previously mentioned antibody, pupillary changes and dif culty with accommodation, dry mouth and dry eyes, and gastrointestinal paresis were the most common ndings according to Sandroni and colleagues Perhaps another subgroup is in some way related to Sjogren syndrome since sicca symptoms are prominent but these later features could just as well be a component of the autonomic failure and the appropriate antibodies have been found only rarely There is not enough information to determine if all these cases are accounted for by one process or to judge the effects of various immune treatments Migrant Sensory Neuritis (Wartenberg Syndrome) The de ning feature of this syndrome is a searing and pulling sensation involving a small cutaneous area of a limb evoked by extending or stretching the limb, as happens when reaching for an object with the extended arm and hand, or when kneeling, or pointing with the foot The pain is momentary but leaves in its wake a patch of numbness Cutaneous sensory nerves must be involved in some way and are irritated during such a maneuver Often the area involved is proximal to the most terminal sensory distribution of the nerve, encompassing, for example, a patch on the lateral side of.

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the hand and the proximal fth nger or a larger region over the patella (the sites affected in three of our patients) Recovery of the patch of numbness takes several weeks but it may persist if the symptoms are induced repeatedly Except for these patches of cutaneous analgesia, the clinical examination is normal Selected sensory nerves may show abnormalities in conduction Matthews and Esiri have listed the many areas that may be affected in a single patient and have described an increase in the endoneurial connective tissue in a biopsied sural nerve The process may come in episodes over many years, without symptoms between attacks A spurious diagnosis of multiple sclerosis is often made The pathology is not known but some form of brosis or in ammation of cutaneous nerves has been suggested, perhaps in some way similar to the condition described below Sensory Perineuritis Under this title, Asbury and colleagues described a patchy, painful, partially remitting distal cutaneous sensory neuropathy The pathologic picture was one of in ammatory scarring restricted to the perineurium, with compression of the contained nerve bers As with the Wartenberg syndrome earlier, the re exes and motor function were unaffected Digital nerves as well as the medial and lateral branches of the super cial peroneal nerve were the ones most often involved Matthews and Squier have described a trigeminal and occipital distribution of painful sensory symptoms, and one of the patients of Asbury and coworkers also had symptoms on the scalp A Tinel sign is characteristically elicited by tapping the skin overlying the involved cutaneous nerves and is indicative of partial nerve damage and regeneration The differential diagnosis includes numerous other forms of painful sensory neuropathy but the patchy and painful and often burning quality of symptoms distinguishes this process The diagnosis can only be established with certainty by biopsy of a distal cutaneous branch of a sensory nerve Perhaps some of the large group of patients with burning feet may have a small- ber neuropathy that affects intradermal nerve bers in a similar way (see further on) Since the original report, the brosing perineurial pathologic changes that characterize perineuritis have been described in a number of polyneuropathies, mainly in diabetic patients but also in those with cryoglobulinemia, nutritional diseases, and malignancies (Sorenson et al) However, these patients displayed a diversity of clinical patterns of neuropathy, mainly mononeuritis multiplex and demyelinating neuropathy These ndings indicate that the pathologic feature of perineuritis may be less speci c than initially thought but the perineuritis clinical syndrome is still a useful concept A proportion of the idiopathic cases respond to corticosteroids Celiac-Sprue Disease Among the multitude of odd neurologic manifestations attributed to this disease (see pages 977 and 1001) the best known are a cerebellar ataxia and myoclonus In addition, Hadjivassiliou and colleagues have reported patients with a range of neuromuscular disorders in whom the neurologic symptoms antedated the diagnosis of the bowel disorder A nondescript sensorimotor neuropathy was the most frequent complication, but one patient was said to have a mononeuritis multiplex In a small prospective survey of treated celiac disease, Luostarinen and colleagues found 23 percent with evidence of a polyneuropathy by EMG testing but the clinical ndings were scant Antigliadin antibodies (simple antibodies directed against gluten), as well as more speci c antitransglutaminase antibodies and histologic examination of a duodenal biopsy are con rmatory of the diagnosis Luos-.

ferent ber connections The occulonodular lobe receives special proprioceptive impulses from the vestibular nuclei and is therefore also referred to as the vestibulocerebellum; it is concerned essentially with equilibrium The anterior vermis and part of the posterior vermis are referred to as the spinocerebellum, since projections to these parts derive to a large extent from the proprioceptors of muscles and tendons in the limbs and are conveyed to the cerebellum in the dorsal spinocerebellar tract (from the lower limbs) and the ventral spinocerebellar tract (upper limbs) The main in uence of the spinocerebellum appears to be on posture and muscle tone The neocerebellum derives its afferent bers indirectly from the cerebral cortex via the pontine nuclei and brachium pontis, hence the designation pontocerebellum; this portion of the cerebellum is concerned primarily with the coordination of skilled movements that are initiated at a cerebral cortical level These divisions are necessarily incomplete, because it is now appreciated that certain portions of the cerebellar hemispheres are also involved to some extent in tactual, visual, auditory, and even visceral functions On the basis of ablation experiments in animals, three characteristic clinical syndromes corresponding to these major divisions of the cerebellum have been delineated Lesions of the nodulus and occulus have been associated with a disturbance of equilibrium and frequently with a positional nystagmus; individual movements of the limbs are not affected Anterior lobe ablation in primates results in increased shortening and lengthening reactions, somewhat increased tendon re exes, and an exaggeration of the postural re exes, particularly the positive supporting re ex, which consists of an extension of the limb in response to light

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