of a levodopa-nonresponsive PD patient who failed to improve after pallidotomy also failed to improve after DBS STN. 10 Our patients were all levodopa-responsive and, aside from a single patient, improved after their pallidotomy.There are several reasons why postpallidotomy STN DBS may result in less robust motor improvement. First, there is an obvious referral bias toward patients who were not satisfied after their pallidotomy, either for objective or subjective reasons. This may represent a more aggressive disease process or more atypical course. The pre-and postpallidotomy off drug UPDRS scores, however, were similar in this group of pallidotomy DBS patients compared to our pallidotomy population in general (n ϭ 89). 1 Dyskinesia scores were also similar. Second, electrophysiological recordings, on which we greatly rely for placement, can be altered in the STN following GPi ablation, possibly resulting in suboptimal placement. 11 Random suboptimal placement is possible in either group. Third, there could be redundant physiological effects that would mitigate against subsequent improvement after the second procedure. A single study that simultaneously implanted GPi and STN DBS found that combined stimulation was no more effective than STN stimulation alone. 12 Fourth, we present a relatively small number of patients and the results could be different with a larger sample.Overall, the small corpus of literature on the efficacy and safety of postpallidotomy STN DBS is mixed. We recommend prudence when considering DBS in this population. REFERENCES

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Although positive sensory symptoms are classically considered a hallmark of acquired neuropathies, sensory manifestations, in particular, pain, are not uncommon in Charcot‐Marie‐Tooth disease (CMT). We investigated the occurrence of sensory manifestations in 35 CMT patients (15 with CMT1, 20 with CMT2). Positive sensory manifestations were reported by 18 patients (51%), and were prominent in 7 patients (20%), representing an onset symptom and/or a main feature. Non‐neuropathic (musculo‐skeletal) pain was also frequent, occurring in 48% of patients. Comparison between CMT1 and CMT2 showed that positive sensory symptoms were significantly more frequent in CMT2 (70% versus 27%; p = 0.018), representing a prominent clinical feature in 25% of CMT2 patients. Non‐neuropathic pain was more frequent in CMT1 (60%) than in CMT2 patients (40%), and represented an onset symptom and/or a main feature in 33% and 25% of patients, respectively. Sensory manifestations in CMT seem more frequent than previously thought, however their frequency may be different in the genetic subtypes of the disease. The occurrence of positive symptoms in CMT2 raises diagnostic problems with regard to acquired axonal neuropathies, and, in particular, chronic idiopathic axonal polyneuropathy (CIAP). Sensory symptoms, in particular, pain, may represent an important issue in the management of CMT patients, especially in a physical medicine approach.

Involvement of sensory nerves in Charcot-Marie-Tooth (CMT) disease is well known, however, sensory symptoms are usually overlooked. To assess the frequency and features of sensory symptoms in a cohort of patients with CMT, we investigated in a prospective study 52 consecutive CMT patients, diagnosed on the basis of clinical, neurophysiological, and genetic features and classified in CMT type 1 (CMT1) (20 patients, including 14 with CMT1A) and CMT type 2 (CMT2) (32 patients). Positive sensory symptoms were reported by 28 patients (54%), including neuropathic pain in 6 patients. Pain, either neuropathic or nociceptive, was present in 29 patients (56%) and in 15 patients as a main symptom. Positive sensory symptoms were present in 24 of 32 CMT2 patients (75%) and in 4 of 20 CMT1 patients (20%) (p < 0.001); there was a presenting manifestation in 11/32 CMT2 patients vs. 1/20 in CMT1 patients (p = 0.018), and one of the main features in 6/32 CMT2 patients vs. 1/20 CMT1 patients. Frequency of positive sensory symptoms in CMT1A patients was similar to that of the entire CMT1 group. Within the CMT2 group, patients with positive sensory symptoms as a main or onset feature (11 patients) had significantly later onset (median 57 vs. 25 years; p = 0.042) and less severely impaired motor action potentials than CMT2 patients without positive sensory symptoms (8 patients). Nociceptive pain was especially frequent in CMT1A patients (10/14, 71%). Sensory manifestations in CMT seem more frequent than previously thought, especially in CMT2; however, their frequency may be different in the genetic subtypes of the disease and/or an expression of phenotypic variability. Sensory symptoms, and in particular pain, may represent an important issue in the management of CMT patients, especially in a physical medicine approach.

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In OHT recipients, pathologic evaluation of the heart during acute rejection can show involvement of both the conduction system and the myocardium. We here describe the cases of a 9-year-old male with DCM and a 13-year-old female with RCM, who developed third-degree PAVB associated with acute rejection 36 months and 24 months after OHT, respectively. We conclude that PAVB could be considered an early sign of acute rejection after OHT in children who exhibit post-transplantation presyncope or syncope.