ABSTRACT
Peripheral neuropathy is one of the rarely reported neurological complications of interferon therapy. The authors report such a case in a 15-year-old boy during alpha-interferon therapy for Hodgkin's disease. He received alpha-interferon at a dose of 1.8 million units/day 5 times a week by subcutaneous injections as adjuvant immunotherapy post autologous stem cell transplant. Twenty months after the initiation of therapy, he complained of severe pain in his lower distal extremities. Neurological examination revealed the absence of deep tendon reflexes. A nerve conduction study showed a sensorial, polyneuropathic involvement in the lower extremities. Within 4 weeks after the stopping of interferon, his pain improved, and recovery was also seen by nerve conduction studies. Symptoms reappeared at the resumption of interferon treatment. This study suggests that peripheral neuropathy may rarely occur in patients given long-term interferon treatment at high cumulative dosage.
Subject(s)
Hodgkin Disease/drug therapy , Interferon-alpha/adverse effects , Peripheral Nervous System Diseases/chemically induced , Adolescent , Humans , Interferon-alpha/therapeutic use , MaleABSTRACT
OBJECTIVE: The present study has examined the effect of vitamin E, the principal modulator of free radical activity, on electrophysiological parameters in patients with diabetic peripheral sensorimotor polyneuropathy, matched for duration of disease and metabolic control. RESEARCH DESIGN AND METHODS: A total of 21 subjects with type 2 diabetes were enrolled in this double-blind randomized placebo-controlled study (vitamin E, 11 patients; placebo, 10 patients). Patients were randomly assigned to receive either 900 mg vitamin E or placebo for 6 months. The average dietary vitamin E consumption of the subjects was similar during the study. The main outcome measure was the electrophysiological tests assessing nerve conduction. Fasting plasma glucose, HbA1, postprandial plasma glucose, and electrophysiological parameters in the basal state and after 6 months of treatment were studied. RESULTS: Glycemic indexes did not show any significant changes during the study, whereas nerve conduction improved significantly in 2 of the 12 studied electrophysiological parameters after 6 months in patients on vitamin E supplementation. The changes in the electrophysiological parameters were obvious in the median motor nerve fibers and tibial motor nerve fibers. Nerve conduction velocity in the median motor nerve fibers (P = 0.0019) and tibial motor nerve distal latency (P = 0.0284) improved significantly after 6 months of vitamin E supplementation. CONCLUSIONS: This study shows that defective nerve conduction in diabetic subjects with mild-to-moderate peripheral neuropathy may be improved by pharmacological doses of vitamin E supplementation. Further studies with a larger number of patients for longer periods of time are needed.
Subject(s)
Antioxidants/therapeutic use , Diabetes Mellitus, Type 2/diet therapy , Diabetic Neuropathies/diet therapy , Dietary Supplements , Neural Conduction/drug effects , Vitamin E/therapeutic use , Blood Glucose/metabolism , Diabetes Mellitus, Type 2/physiopathology , Diabetic Neuropathies/physiopathology , Double-Blind Method , Electrophysiology , Female , Glycated Hemoglobin/analysis , Humans , Male , Middle Aged , Motor Neurons/physiology , Postprandial PeriodABSTRACT
The efficacy of recombinant interferon-alpha (rIFN alpha), on natural killer (NK) cell cytotoxic activity, CD3+, CD4+, CD8+, CD56+, HLA-DR+ lymphocyte counts, anti-acetylcholine receptor antibody (AChR Ab) levels, single fibre electromyography findings (SFEMG) and clinical course were evaluated in patients with myasthenia gravis (MG). During the IFN alpha treatment (3 mu, subcutaneous, 3 times a week), NK cell cytotoxicity and CD4+/8+ ratio increased, NK cell count remarkably decreased, and no significant clinical or SFEMG changes were observed. This preliminary open study in MG patients has demonstrated enhanced NK activity per unit NK cell after IFN alpha therapy. Although lymphocyte phenotypes and NK function approached normal levels during therapy, a higher dose of IFN alpha may be required for a significant clinical response. It has been also concluded that 6 months of IFN alpha therapy seems to be safe in MG, though in patients with malignancy, IFN alpha may cause increased autoimmunity, AChR positivity and MG.
Subject(s)
Interferon-alpha/therapeutic use , Killer Cells, Natural/physiology , Myasthenia Gravis/therapy , Adult , Antibodies/analysis , Cytotoxicity, Immunologic , Female , Humans , Interferon-alpha/adverse effects , Leukocyte Count , Lymphocyte Subsets/pathology , Male , Myasthenia Gravis/pathology , Myasthenia Gravis/physiopathology , Receptors, Cholinergic/immunology , beta 2-Microglobulin/analysisABSTRACT
A case of progressive encephalomyelitis with rigidity (PEWR) associated with hepatitis C virus (HCV) is reported. A 58 year-old woman presented with a clinical picture of progressive quadriparesis, sensory loss, sphincter dysfunction, painful muscle spasms in the upper and lower limbs and continuous muscle unit activity in electromyography. She developed hepatitis, pancreatitis and HCV-RNA was detected in the plasma by reverse transcription-polymerase chain reaction (RT-PCR). Postmortem histopathological examination showed encephalomyelitis with perivascular lymphocyte cuffing, infiltration and neuronal loss mainly affecting the brainstem and cervical spinal cord. The RT-PCR analysis of the postmortem brain, brainstem, liver, pancreas, plasma and CSF samples revealed the presence of HCV genome in all specimens except CSF. Clinical features, postmortem histopathology and PCR results and the possible etiopathogenesis of PEWR are briefly discussed.
Subject(s)
Encephalomyelitis/virology , Hepacivirus/genetics , Hepatitis C/virology , Muscle Rigidity/virology , Polymerase Chain Reaction , Brain Stem/pathology , Brain Stem/virology , Encephalomyelitis/diagnosis , Encephalomyelitis/pathology , Fatal Outcome , Female , Genome, Viral , Hepatitis C/diagnosis , Hepatitis C/pathology , Humans , Middle Aged , Muscle Rigidity/diagnosis , Muscle Rigidity/pathology , Neurologic Examination , Spinal Cord/pathology , Spinal Cord/virologyABSTRACT
Eleven patients with congenital and five with juvenile myasthenia gravis, aged 5 to 24 years, were given 3,4-diaminopyridine in a double-blind, placebo-controlled, crossover study. Clinical improvement was observed in 5 of 11 congenital myasthenia patients, and placebo effect, in 3 of 11. Juvenile myasthenia patients did not respond. Single-fiber electromyographic studies did not reveal any changes correlating with the clinical status of the patient. This study demonstrates the importance of double-blind and placebo-controlled studies to determine the effect of 3,4-diaminopyridine in congenital myasthenia. This drug may have different effects on various presynaptic and postsynaptic defects of neuromuscular transmission resulting in congenital myasthenia syndromes.
Subject(s)
4-Aminopyridine/analogs & derivatives , Myasthenia Gravis/drug therapy , 4-Aminopyridine/therapeutic use , Adolescent , Adult , Amifampridine , Child , Child, Preschool , Cross-Over Studies , Double-Blind Method , Electromyography/drug effects , Female , Humans , Male , Myasthenia Gravis/congenital , Myasthenia Gravis/diagnosis , Neurologic Examination/drug effectsSubject(s)
Antibodies , Electromyography , Myasthenia Gravis/diagnosis , Receptors, Cholinergic , Adult , Child , Female , Humans , MaleABSTRACT
We report a male with cerebellar ataxia, hypogonadism and chorioretinopathy. The age of onset was 12. The parents were first cousins. Endocrinologic studies demonstrated hypogonadotropic hypogonadism due to pituitary dysfunction. The ocular disorder involved the choriocapillaris and the retina. The association may represent a separate syndrome, seldom recognized in the past.
Subject(s)
Chorioretinitis/diagnosis , Gonadotropins, Pituitary/deficiency , Hypogonadism/diagnosis , Spinocerebellar Degenerations/diagnosis , Adult , Atrophy , Cerebellum/pathology , Chorioretinitis/genetics , Consanguinity , Humans , Hypogonadism/genetics , Magnetic Resonance Imaging , Male , Pituitary Function Tests , Spinocerebellar Degenerations/genetics , Syndrome , Tomography, X-Ray ComputedABSTRACT
The clinical and EEG features of 10 patients with hot water epilepsy were presented. Eight of the 10 cases were male. The mean age of onset was 4.7 years. In four patients partial and in six cases generalized seizures were seen. Three patients also had non-hot water precipitated seizures. Interictal EEGs showed generalized abnormalities in eight and focal abnormalities in two cases. CT was performed and found normal in two patients. Anticonvulsant therapy was instituted for all patients. In six cases with adequate follow-up, complete remission was obtained in four and the frequency of the seizures was decreased in two, with anticonvulsant therapy.
Subject(s)
Epilepsy/etiology , Hot Temperature/adverse effects , Water , Child , Child, Preschool , Electroencephalography , Epilepsy/physiopathology , Female , Humans , Infant , MaleABSTRACT
Monomelic amyotrophy is a rare, benign motor neuron disorder. Electrophysiologic studies are suggestive of localized chronic anterior horn cell disease. Two young siblings are reported with monomelic amyotrophy who had proximal muscle weakness confined to one arm. We propose that monomelic amyotrophy, at least in this family, is inherited as an autosomal recessive trait.