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H Royden Jones

Publications and source records attributed to H Royden Jones.

16 recordsLinked to original sources
PubMed2006

Inappropriate surgeries resulting from misdiagnosis of early amyotrophic lateral sclerosis.

Initial symptoms of amyotrophic lateral sclerosis (ALS) may mimic radiculopathy, myelopathy, mononeuropathy, or arthropathy. A retrospective review of 260 consecutive patients with ALS evaluated between 1996 and 2004 revealed that 55 (21%) had had surgery within the 5 years prior to ALS diagnosis. Thirty-four of these 55 (61%) had surgery for symptoms and signs that retrospectively were attributable to early manifestations of ALS. Misdiagnosis of early ALS may lead to unnecessary surgeries with their attendant potential complications.

Aged↗
PubMed2004

Thalidomide neuropathy in childhood.

Thalidomide was withdrawn from world markets in 1961 following recognition of its teratogenic effects. More recently, however, thalidomide treatment has been reintroduced to adult and paediatric practice for a variety of dermatologic, immunologic, rheumatologic and neoplastic disorders. Neuropathy is a significant side effect of thalidomide therapy, which may limit its clinical use. We report four cases of sensorimotor axonal neuropathy in children aged 10-15 years, treated with thalidomide for myxopapillary ependymoma, Crohn's disease and recurrent giant aphthous ulceration. Thalidomide neuropathy is often associated with proximal weakness and may progress even after discontinuation of treatment, in the phenomenon of 'coasting'. Children treated with thalidomide should undergo regular neurophysiologic studies in order to detect presymptomatic or progressive peripheral neuropathy.

Action Potentials↗
PubMed2004

Mogul-clonus.

Explore the source record for details and available documents.

Adult↗
PubMed2004

Multifocal slowing of nerve conduction in metachromatic leukodystrophy.

Polyneuropathy is invariably associated with the late-infantile form of metachromatic leukodystrophy (MLD), and occurs frequently in the early juvenile, juvenile, and adult variants. Uniform slowing of nerve conduction velocity is the neurophysiologic hallmark of metachromatic leukodystrophy and other inherited demyelinating polyneuropathies. To evaluate the consistency of this principle, we reviewed nerve conduction studies in 9 children with late-infantile or early-juvenile metachromatic leukodystrophy. Each child had significant slowing of motor nerve conduction velocity (NCV). The compound muscle action potentials showed abnormal temporal dispersion in 3 of the 9 children, which is usually regarded as the hallmark of acquired demyelinating polyneuropathies. There are reports of multifocal slowing in other hereditary processes including X-linked Charcot-Marie-Tooth disease, hereditary neuropathy with liability to pressure palsies, and adrenomyeloneuropathy. Although multifocal NCV slowing in a child with polyneuropathy is seen most commonly in acquired conditions, a hereditary process, including MLD, cannot always be excluded in this setting.

Action Potentials↗
PubMed2004

Myasthenia gravis and premature ovarian failure.

We describe a patient who developed seropositive myasthenia gravis 16 years after she was diagnosed with autoimmune premature ovarian failure with antibodies to the receptor for follicle-stimulating hormone (FSH). Although thymectomy led to improvement of her myasthenic symptoms, menses did not resume. Such combined seropositivity for antibodies to acetylcholine and ovarian hormone receptors in a patient with myasthenia gravis and premature ovarian failure may reflect common disease mechanisms, although the precise pathogenesis of these disorders remains ill-defined.

Acetylcholine↗
PubMed2004

Neuromuscular problems of the critically ill neonate and child.

This review presents insights gained over 24 years of clinical and laboratory evaluations of children, newborn to 18 years of age, who present with acute weakness in the intensive care setting. The differential diagnosis of neuromuscular disorders in these cases begins with recognition of three categories-the infant and toddler, the older child and adolescent, and the child with critical illness-within which predisposition to specific disorders may be identified. Disorders originating from anterior horn cell, peripheral nerve, neuromuscular junction, and muscle cell are discussed with emphasis on presentation and electrophysiologic findings. Nerve conduction studies, electromyography, electroencephalography, cerebrospinal fluid analysis, and magnetic resonance imaging each play important diagnostic roles in the differentiation of neuromuscular disorders in the critically ill child. Case studies suggest the wide range of presentations these disorders may make to the pediatrician or pediatric neurologist.

Adolescent↗
PubMed2003

Oculobulbar involvement is typical with Lambert-Eaton myasthenic syndrome.

Oculobulbar symptoms and/or signs were present in 18 of 23 (78%) of Lambert-Eaton myasthenic syndrome (LEMS) patients evaluated at the Lahey Clinic (Table). Sixty-five percent (15 of 23) of our patients had ptosis and/or diplopia, each present in 11 individuals. Bulbar signs and symptoms, including dysarthria in 10 and dysphagia in 8 patients, also were observed among our LEMS population. More than one prereferral oculobulbar feature occurred in 13 of our LEMS patients. Prereferral diagnostic considerations included myasthenia gravis, myopathies, and psychiatric disorders. These findings suggest that these atypical characteristics served to dissuade some colleagues from a diagnosis of LEMS. Thus, the presence of oculobulbar symptoms and signs cannot be used to exclude LEMS from the differential diagnosis.

Adult↗
PubMed2003

Diagnostic value of electromyography and muscle biopsy in arthrogryposis multiplex congenita.

Arthrogryposis multiplex congenita (AMC), a clinical syndrome characterized by multiple congenital joint contractures, frequently is caused by lesions in the peripheral nervous system. Two standard tests for the evaluation of the motor unit are nerve conduction studies/electromyography (NCS/EMG) and muscle biopsy. We reviewed the diagnostic value of these two studies in the evaluation of AMC over a 23-year period, analyzing 38 patients with AMC who had NCS/EMG, muscle biopsy, or both. Final diagnoses were classified as neurogenic (8 patients), myopathic (10 patients), "other" (12 patients), or unknown (8 patients). Neither test alone had consistently high sensitivities, positive predictive values, or specificities. However, when NCS/EMG and muscle biopsy were concordant for neurogenic or myopathic findings, they were more accurate than either test alone, especially for neurogenic diseases. Test results were most commonly discordant in patients with "other" or unknown diagnoses. These findings suggest that when the clinical evaluation indicates a specific syndromic, developmental, or exogenous cause, NCS/EMG and muscle biopsy are not helpful and may not need to be performed. When the history, examination, and genetic evaluation are unrevealing, NCS/EMG and muscle biopsy together provide valuable diagnostic information.

Arthrogryposis↗
PubMed2003

Paediatric mononeuritis multiplex: a report of three cases and review of the literature.

Mononeuritis multiplex results from disease processes causing multifocal injury to the peripheral nerves, with or without involvement of one or more cranial nerves. Most reported cases of paediatric mononeuritis multiplex have been related to autoimmune disorders. We present clinical and neurophysiologic findings in three cases of multiple mononeuropathy occurring in adolescence, with onset at ages 16, 17 and 13 years. Two of these cases were related to systemic vasculitis, one possibly representing a paediatric case of non-systemic vasculitis. Sural nerve biopsy confirmed the diagnosis of vasculitis in one patient with systemic lupus erythematosus, but was non-diagnostic in another case. Mononeuritis multiplex is an extremely uncommon form of acquired peripheral neuropathy in childhood. We discuss the differential diagnosis and review previous descriptions of this rare condition.

Adolescent↗
PubMed2003

Current therapeutic strategies for patients with polyneuropathies secondary to inherited metabolic disorders.

Supportive care, symptomatic treatment, and patient education should be provided for patients with inherited or acquired polyneuropathies. In addition, specific treatment is available for many of the acquired polyneuropathies. Genetic counseling is valuable for many patients with inherited polyneuropathies, but only rarely is specific treatment an option for these patients. However, specific treatments are available for many of the rare and devastating systemic disorders associated with polyneuropathies. Thus, clinicians must promptly diagnose these inherited disorders so that specific treatment may be initiated. The clinical features of these rare inherited disorders are emphasized.

Adult↗
PubMed1996

Pediatric ulnar mononeuropathy: report of 21 electromyography-documented cases and review of the literature.

Pediatric ulnar mononeuropathies are the most frequent upper extremity mononeuropathies seen in the electromyography laboratory at The Children's Hospital, Boston. Twenty-one children (12 boys and nine girls) with pediatric ulnar mononeuropathy, aged 5 to 18 years, were seen from 1979 to 1991. The causes included acute trauma in 11 children (52%), compression in five children (24%), entrapment in three children (14%), and indeterminate in two children (10%). The sites of nerve injury included the elbow in 10 children (48%), forearm in three children (14%), wrist in five children (24%), hand in one child (4%), and indeterminate in the remaining two children (10%). Prognosis is more favorable in nontraumatic (83% improved) pediatric ulnar neuropathies than with traumatic lesions (56% improved), with at least a 1-year follow-up.

Adolescent↗
PubMedDate not supplied

Severe ataxia, myelopathy, and peripheral neuropathy due to acquired copper deficiency in a patient with history of gastrectomy.

BACKGROUND: In animal studies, copper absorption has been demonstrated to occur in the proximal gut via duodenal enterocytes. Acquired copper deficiency is known as "swayback" in ruminant animals and Menkes' disease in humans. Copper is an essential micronutrient necessary for the hematologic and neurologic systems. Acquired copper deficiency in humans has been described, causing a syndrome similar to the subacute combined degeneration of vitamin B(12) deficiency. METHODS: This is a single case report. Our patient developed a neurologic constellation of ataxia, myelopathy, and peripheral neuropathy similar to vitamin B(12) deficiency many years after gastrectomy for severe peptic ulcer disease. The patient was maintained for decades with enteral feedings via jejunostomy that provided the recommended dietary allowance (RDA) for copper. RESULTS: Copper deficiency was suspected, identified, and treated. Over 3 months of follow-up, serum copper levels increased from 4 microg/dL to 20 microg/dL (70-150 microg/dL), and ceruloplasmin increased from 6 mg/dL to 8 mg/dL (14-58 mg/dL). During this short time of follow-up, the patient has had no further progression of his neurologic symptoms. CONCLUSIONS: Ataxia and myelopathy secondary to acquired copper deficiency are rare complications of major gastric resection. This is quite similar to the syndrome of vitamin B(12) deficiency. Vitamin B(12) repletion does not improve symptoms. Bariatric procedures such as gastric bypass surgery result in a similar functional anatomy of the proximal gut and may place more patients at increased risk. Early recognition and therapy with oral or parenteral copper may lead to a decrease in both neurologic and hematologic consequences.

Aged↗