Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Favorable Outcome Despite Prolonged Status Epilepticus

Background

To describe a case of auto-immune encephalitis in an adolescent with favorable outcome despite prolonged status epilepticus.

Methods

A 17 year old Asian man without previous medical history developed alteration of consciousness and partial seizures. The diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was confirmed by the detection of specific antibodies in both cerebrospinal fluid and serum.

Results

The clinical course was complicated by prolonged status epilepticus which was refractory to a large number of antiepileptic drugs, including barbiturate coma. Immunomodulatory therapy included steroids, plasma exchanges, and intravenous immunoglobulins. After 86 days of intensive therapy, the patient regained consciousness progressively. Brain magnetic resonance imaging never demonstrated any lesion. Extensive search for a tumor was negative. At 12 month follow-up, the patient had made an excellent recovery.

Conclusion

Auto-immune encephalitis is likely underdiagnosed in adolescents. In their most severe presentation, seizures may be resistant to a large number of anti-epileptic drugs, and the clinical improvement seems to be mainly because of the immunomodulatory therapy. Relapse is possible, as well as the delayed development of a teratoma or other tumor.     

Testosterone Levels Following Decreases in Serum Osteocalcin

Recent preclinical studies suggest that osteoblasts are able to induce testosterone production by the testis, a process mediated by osteocalcin. Bisphosphonates substantially reduce osteocalcin levels. If osteocalcin is an important regulator of testosterone levels in adult men, it would be expected that the substantial reductions in osteocalcin induced by zoledronate would impact negatively on testosterone levels. Previously, we carried out a 2-year randomized, controlled trial of annual 4 mg zoledronate in 43 HIV-infected men. To explore the relationship between osteocalcin and testosterone further, we measured serum testosterone at baseline, 3 months, and 2 years; luteinizing hormone at 3 months and 2 years; and total osteocalcin at 2 years in 28 trial participants with available blood samples. At 2 years, total osteocalcin was 39 % lower in the zoledronate group than the placebo group (zoledronate mean 10.1 [SD 3.0] μg/L, placebo 16.5 [SD 4.9] μg/L, P = 0.003). Despite these substantial differences in osteocalcin levels, testosterone levels did not change over time in either group and there were no between-group differences over time, P = 0.4 (mean change at 2 years [adjusted for baseline levels] in zoledronate group ?0.4 nmol/L, 95 % CI ?2.5 to 1.6; placebo group 0.4 nmol/L, 95 % CI ?1.6 to 2.5). Luteinizing hormone was within the normal range and did not differ between the groups at either 3 months or 2 years. Thus, the absence of a change in testosterone despite a substantial reduction in osteocalcin following zoledronate treatment argues against a biologically significant role for osteocalcin in the regulation of testosterone in adult men. This provides reassurance that men receiving potent antiresorptive drugs are not at risk of iatrogenic hypogonadism.     

Patient satisfaction and suggestions for improvement of remote ICD monitoring

Purpose

The study aim was to evaluate patient acceptance and content with remote follow-up (FU) of their implantable cardioverter defibrillator (ICD) and to estimate patients?? wish for changes in remote follow-up routines.

Methods

Four hundred seventy-four ICD patients at the device follow-up clinic at Rigshospitalet using CareLink? (Medtronic) remote follow-up, who had made ??2 transmissions, received a questionnaire.

Results

Three hundred eighty-five patients (81.2%) answered. Mean time with ICD was 56?±?45?months and mean age was 62?±?13?years; 80% was male. Diagnosis related to ICD implant was: ischemic heart disease in 56% and dilated cardiomyopathy in 21%. Twenty-six percent had primary prophylactic indication. Mean time on remote FU was 16.4?±?6.9?months. Mean time spent on in-clinic FU (two-way transport and FU) was 4?h and 36?min?±?7?h and 50?min, excluding 12 patients from Greenland and Faroe Islands. Ninety-five percent of the patients was very content or content with remote FU compared to in-clinic FU; 3% was less content and 2% was not content. For scheduled transmissions, 21% of the patients wished for a faster reply (sms or e-mail) compared to current practice with a letter. Eighty-four percent preferred more detailed information concerning ICD leads, battery status, and ICD therapies. A total of 96 patients (25%) had performed extra unscheduled remote transmissions: 20 due to shock, 20 due to alarm, 35 due to palpitations, and 18 for other or combined reasons.

Conclusion

Ninety-five percent of the patients were content with the remote FU. Only 25% had unscheduled transmissions and most unscheduled transmissions were for appropriate reasons. Eighty-four percent of the patients wished for a more detailed response and 21% wished for a faster reply after routine transmissions.