In addition to the well-known chronic sensory polyneuropathy associated with chronic renal failure that is discussed later in the chapter, there is a more rapid (“accelerated”) process that has not been widely appreciated as a cause of acute and subacute weakness. Most patients in our series were diabetics with stable end-stage renal failure who had been treated by peritoneal dialysis for their long-standing kidney disease (Ropper, 1993). In contrast to the better characterized and less severe chronic uremic neuropathy, generalized weakness and distal paresthesias progress over 1 or more weeks until a bedbound state is reached. The illness simulates subacute GBS. More aggressive dialysis or a change to hemodialysis has little immediate effect, although kidney transplantation is curative. Electrophysiologic studies show demyelinating features (slowing of conduction velocity), but usually not conduction block. There is mildly or moderately raised CSF protein concentration (not unexpectedly, for there is usually an element of diabetic neuropathy). A few reported cases have responded to plasma exchange or gamma globulin. As with the more common chronic uremic neuropathy, the cause of the acute form is unknown.
Attention was initially drawn to this entity by Sterman and colleagues in a report of 3 adult patients with rapidly evolving sensory ataxia, areflexia, numbness, and pain, beginning in the face and spreading to involve the entire body. In those instances, the symptoms began following the institution of penicillin for a febrile illness (antibiotics were subsequently shown not to be associated with this process). Proprioception was profoundly reduced, but there was no weakness or muscle atrophy, despite generalized areflexia. The sensory deficit attained its maximum severity within a week, after which it stabilized and improved very little.
Electrophysiologic studies showed absent or slowed sensory conduction, but there were no abnormalities of motor nerve conduction or signs of denervation. In two patients, the CSF protein content was elevated to 126 and 175 mg/dL. Followup observations (for up to 5 years) disclosed no neoplastic or immunologic disorder, the usual identifiable causes of such a sensory neuronopathy. Lacking pathologic material, it was assumed from the permanence of the condition that sensory neurons were destroyed (sensory neuronopathy). A subsequent series of 42 patients reported by Windebank and colleagues emphasized an asymmetrical and brachial pattern of symptoms in some patients and initial involvement of the face in others. In contrast to Sterman’s cases, the CSF was usually normal and most patients had some improvement or a spontaneous resolution of symptoms. In this and subsequent reports, as mentioned, antibiotics were not implicated.
This clinical pattern should be viewed as a syndrome rather than as a disease. There are two main presentations: limb ataxia that does not have accompanying dysarthria or nystagmus, thus distinguishing it from a cerebellar disorder; and generalized facial and truncal numbness that involves proximal and distal sensory areas and may include the top of the head, trunk, buttocks, scrotum, and oral mucosal membranes. The latter syndrome must be delineated from an evolving polyneuropathy, the early proximal symptoms being the most salient identifying feature for a ganglionopathy as mentioned in the introductory sections of this chapter. All the just described processes are accompanied by areflexia, but this may not be fully developed in the case of ganglionopathy for several days or longer.