[Therapeutic Plasma Exchange in a Patient with Chronic Hemodialysis and a New Diagnosis of Myasthenia Gravis].

Case ReportC.S.T. (♂, 71 years old) is a patient with multiple and severe comorbidities, undergoing thrice-weekly chronic hemodialysis since 2008 due to the progression of post-lithiasic uropathy. Over the past 2 months, the patient had been experiencing progressive ptosis of the eyelids, muscle weakness, and ultimately dysphagia and dysarthria that emerged in the last few days. Urgently admitted to the Neurology department, electromyography (EMG) was performed, leading to a diagnosis of predominant cranial myasthenia gravis (with borderline anti-acetylcholine receptor antibody serology). Prompt treatment with pyridostigmine and steroids was initiated. Considering the high risk of acute myasthenic decompensation, therapeutic plasma exchange (TPE) with centrifugation technique was promptly undertaken after femoral CVC placement. TPE sessions were alternated with hemodialysis. The patient's condition complicated after the third TPE session, with septic shock caused by Methicillin-Sensitive Staphylococcus Aureus (MSSA). The patient was transferred to the Intensive Care Unit (ICU). Due to hemodynamic instability, continuous veno-venous hemodiafiltration (CVVHDF) with citrate anticoagulation was administered for 72 hours. After resolving the septic condition, intermittent treatment with Acetate-Free Biofiltration (AFB) technique was resumed. The patient completed the remaining three TPE sessions and, once the acute condition was resolved, was transferred back to Neurology. Here, the patient continued the treatment and underwent a rehabilitation program, showing significant motor and functional recovery until discharge. Conclusions. The multidisciplinary interaction among Nephrologists, Neurologists, Anesthesiologists, and experts from the Immunohematology and Transfusion Medicine Service enabled the management and treatment of a rare condition (MG) in a high-risk chronic hemodialysis patient.

Thyroid hormones in epileptic children receiving carbamazepine and valproic acid.

Carbamazepine and valproic acid are effective antiepileptic drugs for treating many types of epilepsy. Although they are well tolerated, many effects on endocrine function have been reported. Changes in serum thyroid hormones levels in 37 children with epilepsy during carbamazepine and valproic acid therapy were analyzed, and the thyroid hormone concentration after thyrotropin-releasing hormone test was evaluated. Serum thyroxine and free thyroxine levels were significantly lower in patients treated with carbamazepine and carbamazepine plus valproic acid than in the control subjects; serum thyroxine and free thyroxine concentrations were unaffected by valproic acid monotherapy. Serum triiodothyronine and free triiodothyronine concentrations were similar in the three groups of patients studied. Thyroid-stimulating hormone serum levels were normal in all patients, and the thyrotropin responses to the thyrotropin-releasing hormone were similar to control group. Our data suggest that children treated with carbamazepine may have subclinical signs of hypothyroidism, and these changes are more evident if carbamazepine is given in association with valproic acid, while no alteration in thyroid hormones can be found with valproic acid monotherapy. Thyroid-stimulating hormone and thyrotropin-releasing hormone levels do not seem to be affected by these drugs, suggesting that hypothalamic function is not affected in these children.

Discontinuation of anticonvulsant therapy in children with partial epilepsy.

To evaluate when it is possible to discontinue anticonvulsant treatment in children with cryptogenic partial epilepsy, the authors studied 89 epileptic children divided into two groups: Group A, 45 children whose therapy was discontinued after 1 year from the last seizure; and Group B, 44 children whose therapy was stopped after 2 years from the last seizure. After 5 years of follow-up, the recurrence rate was similar in the two groups of patients (Group A, 28.8%; Group B, 25%). It is safe to discontinue the anticonvulsant therapy in children with cryptogenic partial epilepsy who were seizure free for only 1 year.

Predictive value of EEG monitoring during drug withdrawal in children with cryptogenic partial epilepsy.

In order to evaluate the predictive value of the interictal EEG after discontinuation of anticonvulsant therapy in children with cryptogenic partial epilepsy, a prospective study was carried out on 84 children and adolescents who had been seizure-free for at least 2 years. Twenty-four children (28.6%) presented one or more relapses. EEG monitoring during drug withdrawal showed abnormalities in 67% of the patients (16/24) who relapsed with seizures, and in 10% of the patients (6/60) who did not relapse. Our study suggests that in children who have suffered from partial epilepsy, the detection of paroxysmal abnormalities during drug withdrawal, in a previously normal EEG is a risk factor for recurrence of seizures. It is therefore advocated that periodic EEG monitoring be carried out during the drug withdrawal period, because the presence of EEG abnormalities is associated with a high probability of seizures occurring during or after drug discontinuation.

Carbamazepine-induced hypersensitivity syndrome in a child with epilepsy.

Carbamazepine is an effective anticonvulsant and is considered the drug of first choice for the treatment of partial and secondarily generalized seizures. Although carbamazepine is well tolerated, many side effects have been reported in the literature. The majority of these adverse effects are transient and do not lead to the discontinuation of the therapy. We present a case of a female child, aged 11 years and 6 months, who showed an anticonvulsant hypersensitivity syndrome induced by carbamazepine. This syndrome is a rare, potentially life-threatening adverse drug reaction. The patient developed a cutaneous nonpruritic rash, associated with high fever, diffuse lymphadenopathy, and arthralgias on the knees and the ankles with local signs of arthritis. Laboratory examination showed a lymphocytosis, mild thrombocytopenia, marked eosinophilia, and high transaminases. Corticosteroid therapy (betametasone 0,5 mg x 3 day) was started and carbamazepine was gradually withdrawn changing to valproic acid, with complete control of the seizures. The fever and the rash reduced gradually, beginning from the face and then disappearing completely after 10 days. Laboratory results showed a clear improvement: after 7 days the patient showed a complete normalization of the above parameters, except for transaminases. The complete normalization of these enzymes was observed after 2 weeks from the disappearance of the skin rash.

Serum leptin changes in epileptic patients who gain weight after therapy with valproic acid.

Weight gain has been recognized as an adverse effect of valproic acid therapy, but there are are no data about serum leptin levels in patients receiving this drug. To evaluate if valproic acid treatment in epileptic patients in whom obesity develops modifies serum levels of insulin and leptin, 40 female patients with epilepsy were evaluated before therapy and after 1 year of therapy. At the end of follow-up, 15 patients were obese and showed higher serum leptin and insulin levels than patients who did not gain weight. As in other types of obesity, elevation of serum leptin concentrations is related to the increase in body mass index.