Background and AimPure sensory neuropathies involving the dorsal root ganglia are commonly referred to as sensory ganglionopathies (SG). Causes of SG can be inherited (as seen in Friedreich's ataxia) or acquired (e.g. immune‐mediated or paraneoplastic). Diagnostic criteria for confirming SG have been published and consist of a combination of clinical and neurophysiological parameters. The aim of our study was to develop a neurophysiological method for rapid screening for diagnosis of SG.MethodsFor each subject we obtained the sensory nerve action potentials (SNAPs) of five nerves (median, ulnar, radial, sural and superficial peroneal) bilaterally. In the presence of an entrapment neuropathy we obtained the SNAP of the medial antebrachial cutaneous nerves bilaterally. We estimated the number of pairs of nerves showing a SNAP asymmetry of >50% (difference of SNAPs/ lower SNAP).ResultsSixty‐eight subjects, 34 patients with SG and 34 age and sex‐matched controls, participated in the study. Among all subjects using a receiver operating characteristic (ROC) curve analysis, the area under the curve was 0.984 (95% CI, 0.960–1.000; SE, 0.012; p < .001). In order to detect SG, presence of SNAP asymmetry of >50% in 2 pairs of nerves, not explained by an entrapment neuropathy, shows a sensitivity of 97.1%, a specificity of 94.1%, a positive predictive value of 94.3% and a negative predictive value of 97.0ConclusionThe number of pairs of nerves showing a SNAP asymmetry of >50% may be used as a novel rapid screening tool of patients with SG.

ABBREVIATIONSGTCS Generalized tonic-clonic seizures HIHARS Hyperventilation-induced highamplitude rhythmic slow activity with altered awarenessHyperventilation-induced high-amplitude rhythmic slow activity with altered awareness (HIHARS) is increasingly being identified in children and is thought to be an age-related non-epileptic electrographic phenomenon. We retrospectively investigated the clinical outcome in 15 children (six males, nine females) with HIHARS (mean age 7y, SD 1y 11mo; range 4y 6mo-11y). The presenting feature in 11 cases was blank spells -two of these children also had generalized tonic-clonic seizures (GTCS) -and in one individual the main concern was deteriorating school performance. Three children had symptoms suggestive of focal motor seizures. Of the nine children presenting solely with blank spells, further follow-up (mean duration 18mo, SD 21mo) revealed full resolution of symptoms in six, but three had persistent symptoms. In our study, the symptoms of children with HIHARS presenting with blank spells in isolation appeared to resolve spontaneously and did not evolve into convulsive seizures or other paroxysmal events considered to be clearly epileptic. Children (with HIHARS) who presented with clinical features suggestive of GTCS or focal motor seizures (with or without blank spells) and ⁄ or had epileptiform discharges on interictal electroencephalography were subsequently diagnosed with epilepsy.

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