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Biomedical subjects

D J Lange

Publications and source records attributed to D J Lange.

At least 19 recordsLinked to original sources
PubMed1992

Sensory nerve abnormalities in brachial plexopathy.

Sensory nerve action potential (SNAP) amplitude should be abnormal in brachial plexopathies (BP) which cause axonal degeneration in distal segments. Fifty-six patients with BP were identified. In diffuse BP, 22/25 (88%) showed low amplitude or absent median or ulnar SNAP. Three of 5 patients with upper trunk BP had low amplitude or absent SNAP (1 median, 1 radial, 1 lateral antebrachial cutaneous). Seventy-five percent of patients with lower trunk/medial cord BP had low amplitude or absent SNAP (8/24 median, 18/24 ulnar). Overall, 82.5% of patients had low amplitude or absent SNAP when a sensory nerve in the distribution of signs was studied. Testing multiple sensory nerves to include symptomatic regions enhances the diagnostic yield of SNAP in BP.

Action Potentials↗
PubMed1992

Multifocal motor neuropathy with conduction block: is it a distinct clinical entity?

We studied 169 patients with motor neuron disease. Seventeen showed abnormal amplitude reduction of the compound muscle action potential. Ten had focal loss of both amplitude and area across a specific segment (conduction block). Eight of the 10 had slowing of conduction across that segment. Nine were men and had prominent hand involvement. Six had probable or definite upper motor neuron signs. Five of the 10 showed immunologic abnormalities (elevated GM1 antibody titers or paraproteinemia), and eight had had symptoms for more than 4 years. Seven of the 17 patients showed loss of amplitude without corresponding loss of area and focal slowing of conduction (temporal dispersion). Five of the seven were men, five had prominent hand involvement, and five had definite or probable upper motor neuron signs. Two had immunologic abnormalities, and ony one had had symptoms for longer than 4 years. Among 152 patients with no abnormality of conduction, 64% wee men, hands were dominantly involved in 34%, upper motor neuron signs were definite or probable in 72%, and 3% had immunologic abnormalities. None had symptoms for more than 4 years. Because there were so many exceptions, we could not define a unique syndrome by criteria involving conduction block, GM1 antibodies, or lack of upper motor neuron signs. The clinical syndrome associated with multifocal conduction block seemed uniform, however, and patients with conduction block had slower progression if there were no upper motor neuron signs.

Action Potentials↗
PubMed1992

Neuropathies.

Explore the source record for details and available documents.

Diabetic Neuropathies↗
PubMed1991

Lymphoma, motor neuron diseases, and amyotrophic lateral sclerosis.

We studied 9 patients with motor neuron disease and lymphoma. The following several observations have not been recognized in the past: (1) Motor neuron syndromes are associated with either Hodgkin's disease or non-Hodgkin's lymphoma. (2) The syndromes are not restricted to lower motor neuron disorders; 8 of 9 patients had definite or probable upper motor neuron signs as well, qualifying for the diagnosis of amyotrophic lateral sclerosis. Corticospinal tracts were affected in both postmortem examinations. (3) The combination of motor neuron disease and lymphoma is often accompanied by paraproteinemia (3 of 7 patients studied), increased cerebrospinal fluid protein content (6 of 9 patients), and cerebrospinal fluid oligoclonal bands (3 of 9 patients). (4) In 2 patients, asymptomatic non-Hodgkin's lymphoma was found only because the discovery of paraproteinemia gave impetus to examine the bone marrow. (5) Patients with both upper and lower motor neuron signs (amyotrophic lateral sclerosis) may show physiological evidence of conduction block in peripheral nerves or autopsy abnormalities in peripheral nerves. The cause of this syndrome is not known. Both lymphoma and motor neuron disease could have a common cause, possibly a retroviral infection. The frequency of paraproteinemia suggests that an immunological disorder may play a role in the pathogenesis of the neurological disorder.

Aged↗
PubMed1991

Distant effects of locally injected botulinum toxin: a double-blind study of single fiber EMG changes.

We used single fiber electromyography (SFEMG) to study 42 patients who had enrolled in a double-blind, placebo-controlled trial undertaken to assess the efficacy of botulinum toxin (BTX) injection of neck muscles to treat torticollis. SFEMG in a limb muscle was performed before treatment, 2, and 12 weeks after injection of placebo or BTX. Before treatment, the mean jitter was 26.8 microsec in patients who were to receive BTX, and 25.7 microsec in the placebo group. Two weeks after injection, mean jitter in the group receiving BTX was 43.6 microsec. In the placebo group, it was 26.5 microsec (P = less than .05). Twelve weeks after injection, mean jitter in the BTX group was 35.5; for the placebo group it was 24.5. Fiber density did not change in any patient during the study. There were no remote clinical effects of BTX. Injection of BTX into muscles affected with focal dystonia is a promising and safe treatment, but there are subclinical effects on uninjected muscles.

Adult↗
PubMed1991

Chronic inflammatory demyelinating polyneuropathy in childhood: clinical and electrophysiological features.

Five children with chronic progressive polyneuropathy but no familial history of it showed electrophysiological evidence of demyelination with partial conduction block, temporal dispersion, and focal slowing of nerve conduction velocities in multiple nerves. These findings are indicative of an acquired demyelinating polyneuropathy that is chronic and inflammatory and differentiate this condition from most of the inherited neuropathies. It is very important to recognize this entity because of the availability of various treatments.

Child↗
PubMed1990

X-linked spinal muscular atrophy (Kennedy's syndrome). A kindred with hypobetalipoproteinemia.

Kennedy's syndrome, X-linked adult-onset bulbospinal muscular atrophy, has been described in over 30 families. The characteristic distribution of weakness creates a recognizable syndrome, augmented by frequent findings of testicular atrophy and gynecomastia. Type IV or type II hyperlipoproteinemia has been found in some families. We have studied another family with Kennedy's syndrome, this one with hypobetalipoproteinemia. The diversity of serum patterns suggests that lipoprotein abnormalities are not causally related to either the endocrinopathy or the spinal muscular atrophy. However, gene linkage studies indicate proximity of the gene for Kennedy's syndrome and the gene encoding the androgen receptor, which could explain the combination of a motor neuron disorder and the endocrine abnormalities.

Adult↗
PubMed1990

IgM deposits at nodes of Ranvier in a patient with amyotrophic lateral sclerosis, anti-GM1 antibodies, and multifocal motor conduction block.

We studied a patient with amyotrophic lateral sclerosis, multifocal motor conduction block, and IgM anti-GM1 antibodies. A sural nerve biopsy demonstrated deposits of IgM at nodes of Ranvier by direct immunofluorescence. The deposits were granular and located in the nodal gap between adjacent myelin internodes, and in some instances, they extended along the surface of the paranodal myelin sheath. When injected into rat sciatic nerve, the serum IgM bound to the nodes of Ranvier, and the binding activity was removed by preincubation with GM1. These observations suggest that anti-GM1 antibodies may have caused motor dysfunction by binding to the nodal and paranodal regions of peripheral nerve.

Adult↗
PubMed1990

Long-duration polyphasic motor unit potentials in myopathies: a quantitative study with pathological correlation.

In most myopathies, the duration of motor unit action potentials (MUAPs) is shorter than normal. However, polyphasic MUAPs of duration longer than 20% of the control mean, (long-duration polyphasic potentials, or LDPPs) may be seen. We therefore analyzed the incidence and meaning of LDPPs using quantitative MUAP analysis in 41 patients with different myopathies. The mean duration of all potentials was reduced in only 64% of patients because LDPPs increased the mean. When only simple potentials were considered, however, the mean duration was decreased in 95% of patients. This observation confirms the need to exclude LDPPs when calculating the mean duration of MUAPs for diagnosis. We found LDPPs most often in chronic polymyositis and in one patient with Becker Muscular Dystrophy. LDPPs are attributed to desynchronization of single-fiber potentials within the MUAP and may be due to slow conduction in regenerating muscle fibers.

Action Potentials↗
PubMed1990

Motor neuron disease and amyotrophic lateral sclerosis: relation of high CSF protein content to paraproteinemia and clinical syndromes.

From 1984 to 1988, 11 of 120 patients (9%) with motor neuron disease (MND) had paraproteins detected by serum immunofixation electrophoresis (IFE), compared with 4 (3%) by cellulose acetate gels: 1 patient had progressive spinal muscular atrophy, 5 patients had amyotrophic lateral sclerosis (ALS), and 5 patients had ALS with probable upper motor neuron signs. Four of 5 patients (80%) with cerebrospinal fluid (CSF) protein content above 75 mg/dl had paraproteins, as did 6 of 30 with values above 50 mg/dl. Four of 14 patients with cerebrospinal oligoclonal bands (OCB) also had paraproteins. Two patients with ALS, CSF protein content above 75 mg/dl, and paraproteinemia had lymphoma. We conclude the following about patients with MND: high CSF protein content (especially above 75 mg/dl) or CSF OCB makes paraproteinemia more likely; some of these patients may have lymphoma; there is an inordinately high occurrence of paraproteinemia in MND; and IFE on agarose is more sensitive than electrophoresis on cellulose acetate in detecting paraproteins. Syndromes of paraproteinemia and high CSF protein are not restricted to the lower motor neuron but qualify as "ALS" with coexisting upper motor neuron signs.

Adult↗
PubMed1990

Infectious agents and disease of peripheral nerve.

An organism by organism review is presented of infectious agents that may result in focal, multifocal, or diffuse peripheral neuropathy by either direct or, more often, indirect means. Therapy is discussed and is directed at the primary infection as well as at the indirect cause if possible (for example, replacement therapy in vitamin deficiency).

Anti-Infective Agents↗
PubMed1990

Disorders of peripheral nerves associated with HIV infection.

The clinician with interest in neuromuscular disease must become familiar with the clinical manifestations of HIV infection. It is important to realize that not everyone who is infected with HIV will develop clinical AIDS. This includes patients with clinical manifestations related to HIV infection, for example, neuropathy. Thus, if treatment is successful, patients can continue a normal life. HIV infection should be considered in almost any neuromuscular syndrome, especially neuropathies with features of demyelination, which may be the first manifestation of HIV infection. Plasmapheresis may be the treatment of choice for these disorders. Steroids should be used with caution. AZT seems to be a promising new agent to combat AIDS.

Biopsy↗
PubMed1989

Postpolio muscular atrophy. Diagnostic utility of macroelectromyography.

Muscles recovered from previous poliomyelitis may exhibit new weakness and wasting years after the original infection (postpolio muscular atrophy). Routine electrophysiological and morphological techniques may not differentiate weakening muscles from previously affected but stable muscles. We used quantitative motor unit potential analysis, single-fiber electromyography, and macroelectromyography (macro-EMG) to determine if these techniques could identify weakening muscles. We classified 18 previously affected muscles according to strength from 12 patients who had had poliomyelitis 18 to 50 years earlier. Muscles of normal strength had large-amplitude macro-EMG signals (1000 to 4110 microV; normal level, less than 390 microV). Muscles with new weakness and atrophy had smaller than expected macro-EMG amplitude (130 to 450 microV). Two muscles with weakness and atrophy unchanged since the original childhood infection had markedly increased macro-EMG amplitude (1710 and 1860 microV), suggesting the increased macro-EMG amplitude is due to stable reinnervation not strength. Mean motor unit potential duration and amplitude were lower in newly weakened muscles, but jitter, fiber density, and blocking were similar in all muscles regardless of strength. We conclude that low-amplitude macro-EMG signals may be useful in the identification of muscles weakened by postpolio muscular atrophy.

Adult↗
PubMed1989

Ring finger testing in carpal tunnel syndrome: a comparative study of diagnostic utility.

Because digit 4 (D4) has dual innervation, median and ulnar sensory latencies can be determined over identical distances. To determine if D4 testing is more sensitive than other commonly used techniques to diagnose carpal tunnel syndrome (CTS), we examined 42 hands with clinical evidence of carpal tunnel syndrome and 43 control hands. D4 latency was significantly longer than controls more often than digit 2 (D2) in patients with CTS. Comparing median to ulnar latencies from D4 was the most sensitive method to make the diagnosis of CTS. In mild CTS, a characteristic double peak potential was seen in recordings from the median nerve after stimulating D4. This potential provided immediate visual confirmation of the diagnosis of CTS. Comparing D4 latency along median and ulnar nerves is useful for detecting mild CTS and should be used whenever there are clinical signs and symptoms of CTS but electrodiagnostic studies are normal or borderline.

Adolescent↗
PubMed1989

Recurrent myoglobinuria and HIV seropositivity: incidental or pathogenic association?

There have been few cases of polymyositis in patients with AIDS, and polymyositis is rarely a cause of myoglobinuria. We studied a 20-year-old homosexual man with recurrent myoglobinuria. He was asymptomatic between episodes. Each episode was accompanied by muscle pain, limb weakness, high serum levels of creatine kinase, and pigmenturia. Muscle biopsy showed active necrosis without inflammation or abnormalities of glycolytic or other energy-generating enzymes. Antibodies to HIV were present in serum. Clinical evidence of AIDS has not developed in 2 years. Recurrent myoglobinuria may be another consequence of HIV infection.

Adult↗
PubMed1989

Neuromuscular involvement in mild, asymptomatic primary hyperparathyroidism.

PURPOSE: The classic neuromuscular abnormalities of primary hyperparathyroidism are based on observations from an earlier time when the disease presented commonly with signs and symptoms. The purpose of the present study was to test for neuromuscular abnormalities among patients currently seen with primary hyperparathyroidism, in whom the vast majority are asymptomatic. PATIENTS, METHODS, AND RESULTS: Forty-two patients (30 women, 12 men) were studied. Serum calcium level was 11.1 +/- 0.1 mg/dL (normal, 8.7 to 10.7 mg/dL); phosphorus level was 2.8 +/- 0.1 mg/dL (normal, 2.5 to 4.5 mg/dL). Parathyroid hormone levels by radioimmunoassay for N-terminal and for mid-molecule regions were 32.6 +/- 2.4 pg/mL (normal, 8 to 24 pg/mL) and 831 +/- 127 pg/mL (normal, 50 to 330 pg/mL), respectively. Although generalized weakness and easy fatigability were common complaints, they were not seen on neurologic examination. No patient showed typical neuromuscular findings such as muscle weakness or atrophy, hyperreflexia, abnormal gait, tongue fasciculations, or objective changes in mental status. On the other hand, paresthesias and muscle cramps were reported by 22 patients (52%). Neurologic examination in 12 patients (29%) demonstrated findings consisting of either stocking-glove loss of pain sensation or loss of vibratory sensation with diminished reflexes. Creatine phosphokinase levels were normal in all patients. Electromyography and nerve conduction studies were performed in nine patients. None showed myopathy or signs of motor unit denervation. Isolated nerve abnormalities (n = 6) and nonspecific sensory neuropathy (n = 2) were observed. CONCLUSION: We conclude that the classic neuromuscular syndrome of primary hyperparathyroidism is rarely seen among a typical modern-day group of patients with primary hyperparathyroidism. The results suggest that the neurologic component of primary hyperparathyroidism should be included among those features of the disease that have changed along with other aspects of the clinical profile of primary hyperparathyroidism.

Aged↗
PubMed1988

The neuromuscular manifestations of human immunodeficiency virus infections.

We studied 14 patients with neuromuscular disorders and concomitant infection with human immunodeficiency virus to define clinical syndromes and prognosis. Eight patients had painful sensorimotor peripheral neuropathy; two, chronic inflammatory demyelinating polyneuropathy; two, mononeuropathy or mononeuropathy multiplex; one, recurrent myoglobinuria; and one, chronic proximal weakness and elevated creatine kinase levels. All eight patients with painful neuropathy had overt symptoms of acquired immunodeficiency syndrome. Chronic inflammatory demyelinating polyneuropathy was the first manifestation of acquired immunodeficiency syndrome in both patients with this syndrome. Both died from overwhelming sepsis within six months of the neuropathy's onset. Patients with mononeuropathy multiplex had a variable course. Immunosuppressant medication had no effect in two patients.

Acquired Immunodeficiency Syndrome↗