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PubMed1994

[What is left of Morvan's fibrillary chorea?].

In 1890, Morvan described a syndrome of myokimia associated with muscle pain, excessive sweating and sleep disorders. The course was severe and the patient died five weeks after the onset. Several cases were published after this first report in the French literature. The last for 10 years the disease seems to have disappeared. In fact, it seems that the progress in electromyography introduced clear definitions of spontaneous muscle discharges (myokimia, neuromyotonic discharges, fibrillations, fasciculations) allowing recognition of a disease characterized by a syndrome of spontaneous and continuous muscle fiber activity associated with cramping, slow relaxation (pseudomyotonia), excessive sweating and stiffness. Different terms have been used to describe this entity: neuromyotonia (Mertens and Zschocke, 1965) or continuous muscle fibre activity (Isaacs, 1961). Classification of these syndromes has to distinguish inherited from acquired forms, idiopathic cases from cases where neuromyotonia is associated with a peripheral neuropathy, a cancer or an immunological disease. Moreover, the frequent occurrence of this later association and recent reports of improvement after plasma exchanges suggest a possible autoimmune aetiology for this group. Finally, we suggest that the term of "Maladie de Morvan" must be used instead of chorée fibrillaire but only concerning patients who exhibit central disorders including insomnia, hallucinations and altered behaviour.

Acrodynia↗
PubMed1994

The carpal tunnel syndrome. Relationship between median distal motor latency and graded results of needle electromyography.

Needle Electromyography (NEMG) was performed in 228 patients who had been diagnosed by means of motor and sensory median nerve conductions of having Carpal Tunnel Syndrome (CTS). Evaluating abnormal spontaneous muscle activity (fibrillations, positive sharp waves, fasciculation potentials and myokymic discharges) and recruitment pattern, 51.7% of them showed abnormal NEMG. According to the amount of abnormality found, the studies were classified into: normal, grade 1, grade 2 and grade 3. Median Distal Motor Latency (DML) to APB was prolonged beyond the upper confidence limit in 155 (68%) hands, showing a mean value of 5.69 +/- 2.79 msec. Statistically significant difference (p < 0.001) of DML mean value among patients with a NEMG "normal", "grade 1" or "grade 2" have been found. We revise previous reports about NEMG on the CTS and discuss practical consequences of our findings.

Action Potentials↗
PubMed1996

Differential diagnosis of limb girdle syndromes.

The term limb girdle syndrome includes a variety of neuromuscular disorders like the scapulohumeral and pelvifemoral types of muscular dystrophy, quadriceps myopathy and Wohlfart-Kugelberg-Welander syndrome. There may be considerable difficulty in distinguishing between different types of limb girdle syndrome, even with the aid of electromyographic and muscle biopsy examinations. The aim of this investigation was to reestablish the clues for distinguishing between different types of limb girdle syndrome. Fifty-four patients with limb girdle syndromes took part in this investigation. They were subdivided into two groups according to EMG and muscle biopsy data. The first group consisted of 39 patients with limb-girdle type of muscular dystrophy and the second group consisted of 15 patients with juvenile type of spinal muscular atrophy. Our results revealed that neurological examination was not enough to distinguish the different types of limb girdle syndrome. The most important electromyographic findings in patients with muscular atrophy are the neurogenic action potentials and fasciculations. Fibrillations, positive sharp waves and bizzare discharges may be found also in some patients with muscular dystrophy. Myogenic action potentials are found in some patients with muscular atrophy and so may cause confusion. Muscle biopsy may also reveal some myogenic features in patients with muscular atrophy. In conclusion electromyography and muscle biopsy are useful in the differentiation of different types of limb girdle syndrome, as well as, in determining the exact pattern of muscle involvement.

Action Potentials↗
PubMed1997

[Isaacs syndrome: long-term improvement with intravenous polyvalent immunoglobulins].

A 35 year-old man developed a syndrome with muscle cramp, myokimia, generalized, fasciculations, excessive sweating, sleep disorders and severe impairment. It was a syndrome of continuous muscle fiber activity--or Isaacs syndrome--with central disorders (this may be called "Maladie de Morvan"). Previous reports have suggested that Isaac's syndrome might be an autoimmune disorder. Moreover, high doses intravenous immunoglobulins were given resulting in a substantial improvement six months after the onset of this treatment.

Adult↗
PubMed1977

Nervous system effects of cardiac arrest in monkeys. Preservation of vision.

Thirteen juvenile monkeys were taught two visual discrimination tasks. After 12 to 24 hours of food deprivation, ten underwent 14-minute episodes of cardiac arrest. Three served as controls. Five of the ten arrested animals survived and were tested in the discrimination box. All continued to perform color and pattern discrimination tasks with one to eight days' delay. All appeared neurologically intact, while brain pathologic examination after 11 to 64 days' survival showed either intact brains or injury restricted to nuclear structures in the brain stem, cerebellar Purkinje cells, and hippocampus. Five animals died 4 to 36 hours after they were resuscitated. Two required prolonged cardiac massage and, despite return of adequate cardiovascular function, died early. A third animal dislodged its arterial catheter and exsanguinated. The remaining two animals, who received infusions of glucose just prior to arrest, developed widespread fasciculations and myoclonic seizures. They became decerebrate and opisthotonic and were killed after 10 and 36 hours. Their brains showed mild edema and widespread necrosis of cortex and basal ganglia. Thus, food-deprived monkeys tolerate 14 minutes of circulatory arrest well and show minimal neurologic and pathologic changes, while administration of glucose just before arrest markedly augments the severity of brain injury and alters its distribution.

Animals↗
PubMed1979

Presenile dementia with motor neuron disease in Japan. A new entity?

A 61-year-old woman suffered the gradual onset of difficulty with memory, concentration, and cognition at age 58. Progressively more severe dementia was accompanied by muscle wasting and fasciculation prominent in hand and bulbar muscles. An electromyogram and a muscle biopsy specimen demonstrated denervation patterns, and a computerized tomographic scan showed considerable cerebral atrophy. This report reviews cases of presenile dementia with motor neuron disease reported in Japan and discusses the possibility of a new clinicopathologic entity.

Dementia↗
PubMed1986

Hypertrophia musculorum vera in familial ataxia.

A kindred with dominantly inherited ataxia demonstrated hypertrophia musculorum vera as a phenotypic feature of the disease. The proband had fasciculations, cramps, absent sensory nerve action potentials, an increased creatine kinase level, dramatic enlargement of calf muscles, and a muscle biopsy specimen showing denervation accompanied by true muscle fiber hypertrophy; ataxia and other clinical signs of spinocerebellar degeneration were also present. Other family members displayed progressive ataxia and calf muscle enlargement to varying degrees. Though peroneal atrophy is a more common feature of the familial ataxias, some kindreds may have muscle enlargement simulating the pseudohypertrophy of muscular dystrophy that is due instead to denervation-induced compensatory individual fiber hypertrophy.

Adult↗
PubMed1993

Lower motor neuron dysfunction associated with human immunodeficiency virus infection.

OBJECTIVE: The association of human immunodeficiency virus with a clinical picture of motor neuron disease is uncommon, with three cases reported to date. This case represents an additional case of a human immunodeficiency virus-infected patient with apparent motor neuron disease. DESIGN: Single patient case report. SETTING: Large urban public hospital. PATIENT: A 45-year-old human immunodeficiency virus-positive Hispanic man who presented with muscle wasting, fasciculations, areflexia, cranial nerve deficits, and weakness progressing to a complete quadriplegia. RESULTS: Electrophysiologic data showed evidence of diffuse denervation with normal sensory and motor nerve conductions and no evidence of demyelination. Electromyography showed diffuse sharp waves and fibrillation. CONCLUSIONS: This case demonstrates a progressive motor neuron dysfunction in a patient positive for the human immunodeficiency virus and provides additional evidence that infection with the human immunodeficiency virus should be considered in the differential diagnosis of apparent motor neuron disease.

HIV Infections↗
PubMed1997

Amyotrophic lateral sclerosis. Insights from genetics.

Amyotrophic lateral sclerosis (ALS) is among the most dire neurological diseases. The essential clinical feature of the disorder is relentless, lethal paralysis, usually beginning in midadult years. The disease is caused by a slow, progressive loss of motor neurons in the brain and spinal cord. It usually begins focally and then spreads. In most cases, there is concurrent involvement of corticospinal (upper) and spinal (lower) motor neurons, although in some instances the spinal motor neuron features predominate. Involvement of the spinal motor neurons produces muscle denervation of the affected muscles and fasciculations, followed by muscle atrophy. When corticospinal motor neurons degenerate, the weakness is accompanied by spasticity. The mean age at onset of ALS is 55 years; the mean duration is about 4 years. The incidence of new cases is approximately 1 per 100,000 population. The total number of cases is about 5 per 100,000 population. In the United States, it is estimated that there are 20,000 to 30,000 cases. About 10% of cases are inherited as an autosomal dominant trait; familial and sporadic ALS are clinically indistinguishable.

Amyotrophic Lateral Sclerosis↗
PubMed1999

Mother with amyotrophic lateral sclerosis and daughter with Creutzfeldt-Jakob disease.

OBJECTIVE: To describe a mother who had autopsy-proved amyotrophic lateral sclerosis and her daughter who had clinically diagnosed Creutzfeldt-Jakob disease. DESIGN: Case reports with molecular genetic analyses. SETTING: A tertiary care center. PATIENTS: The mother had progressive upper and lower motor neuron symptoms and signs starting at the age of 54 years. Electrophysiological testing supported the diagnosis of amyotrophic lateral sclerosis. Autopsy results confirmed the diagnosis. Her daughter had received injections of human growth hormone prepared from pooled human pituitary glands as a child. At the age of 31 years, she experienced the onset of gait ataxia and dysarthria. Cerebrospinal fluid showed the 14-3-3 protein. Cognitive difficulties ensued. She progressed to a nearly akinetic and mute state. She had overt visible fasciculations and muscle atrophy in the legs. MAIN OUTCOME MEASURES AND RESULTS: Neither patient carried a mutation in the prion protein gene. Both were homozygous for methionine at the polymorphic codon 129. Neither patient carried a deletion of the 5 exons of the superoxide dismutase 1 gene. CONCLUSIONS: It is uncertain whether the 2 cases occurred in the same family by chance or whether the patients shared genetic risk factors for the 2 diseases. The possibility that homozygosity at codon 129 is a risk factor for amyotrophic lateral sclerosis is being tested in a case-control study.

Adult↗
PubMed2001

Convulsive-like movements in brainstem stroke.

BACKGROUND: Involuntary convulsive-like movements sometimes occur in patients with brainstem strokes. These movements vary in nature, frequency, and trigger, including fasciculation-like, shivering, jerky, tonic-clonic, and intermittent shaking movements. Some are interpreted as decerebrate postures or seizures. It is important to recognize this type of motor phenomenon since it may be a diagnostic clue for early diagnosis and treatment of brainstem strokes. DESIGN: Case report and review of the literature OBSERVATION: A 72-year-old-man presented with impaired consciousness and jerks of the upper limbs mimicking seizures. These episodes consisted of brief clonic contractions of the proximal and distal upper extremities. They were observed in paroxysms lasting for 3 to 5 seconds. Magnetic resonance imaging showed large midpontine infarction. Magnetic resonance angiography revealed the absence of basilar artery blood flow. No seizure discharges were observed in the electroencephalogram. Anticoagulation with intravenous heparin was started. Two days after admission, the patient had a cardiac arrest and died. We review the frequency and nature of convulsive-like movements in brainstem stroke in the literature. CONCLUSIONS: Movements associated with brainstem lesions are not easily differentiated from convulsions. Unexpected onset and inexperience of the observers limit the characterization of this phenomenon. Convulsive-like movements in brainstem stroke may occur more frequently than reported. Early detection of this motor phenomenon may have practical implications.

Aged↗
PubMed2002

Creutzfeldt-Jakob disease with amyotrophy and demyelinating polyneuropathy.

OBJECTIVE: To report the clinical and neuropathological features in a patient with Creutzfeldt-Jakob disease with amyotrophy and demyelinating polyneuropathy. DESIGN: Case report. PATIENT AND RESULTS: A 62-year-old man had progressive numbness of the left foot, unsteady gait, diminished deep reflexes, fasciculations, and tactile hypesthesia on the feet. Cerebrospinal fluid, electroneurography, and electromyography were suggestive of chronic inflammatory demyelinating polyneuropathy. He was treated with plasmapheresis, corticosteroids, and immunglobulins, with minimal improvement. After 2 months, severe amyotrophy, polyneuropathy, cerebellar signs, and dementia developed, and he died 8 months after onset of the disease. Autopsy and prion protein immunohistochemistry proved typical Creutzfeldt-Jakob disease. No mutation was found in the prion protein gene, and the codon 129 polymorphism was methionine-valine. In the ventral horn, the loss of the motoneurons was accompanied by prion protein immunoreactivity. The peripheral nerves were segmentally demyelinated but free of prion protein deposition. CONCLUSIONS: The view that peripheral neuropathy and amyotrophy may occasionally be an integral part of Creutzfeldt-Jakob disease is supported by our case, which showed these abnormalities simultaneously. These symptoms, when prominent, may cause problems in differential diagnosis.

Brachial Plexus Neuritis↗
PubMed2003

Polymyositis masquerading as motor neuron disease.

BACKGROUND: Several conditions have been reported to mimic motor neuron disease (MND), and misdiagnosis remains a common clinical problem. OBJECTIVE: To report a case of bulbar-onset polymyositis where the initial clinical presentation was suggestive of MND. CASE DESCRIPTION: A 73-year-old woman was admitted for investigation of acute-onset dysphagia without dysarthria. Examination revealed nasal dysphonia and severe oropharyngeal weakness. Subtle upper-limb weakness, brisk tendon reflexes, and fasciculations in the right deltoid muscle were also demonstrated. A clinical diagnosis of MND was entertained. The serum creatine kinase value was within the reference range. Findings from electromyographic studies, however, were suggestive of a myopathic rather than a neurodegenerative process, and a muscle biopsy specimen was diagnostic of polymyositis. The dysphagia rapidly resolved upon treatment with corticosteroids and azathioprine. CONCLUSIONS: Bulbar-onset polymyositis may mimic MND, particularly in the absence of inflammatory markers or elevated muscle enzyme levels. Caution should be exercised in the clinical diagnosis of bulbar dysfunction, and further investigations such as electromyography and muscle biopsy are indicated to confirm the diagnosis.

Adrenal Cortex Hormones↗
PubMed2005

A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease.

BACKGROUND: Hirayama disease (HD) is a segmental nonprogressive spinal muscular atrophy found in male patients. OBJECTIVE: To report the results of a comprehensive evaluation of clinical, magnetic resonance imaging (MRI), electromyography (EMG), and survival motor neuron (SMN) gene analysis of HD. DESIGN: Clinical, MRI, and SMN gene deletion study. SETTING: Tertiary care teaching hospital. PATIENTS: Patients with HD diagnosed according to defined criteria were included in the study. INTERVENTIONS: Patients underwent a neurologic evaluation and pedigree charting. Concentric needle EMG was performed on a number of muscles. Motor nerve conduction study of the median, ulnar, and peroneal nerves and sensory conduction study of the median, ulnar, and sural nerves were also performed. Spinal MRI of the cervical region was performed with the 2-T scanner operating at 1.5 T. Gene deletion study of SMN1 and SMN2 was performed in all patients. MAIN OUTCOME MEASURES: History of trauma, occupation, exercise, associated medical disease, and cold paresis and muscle wasting, power, reflex changes, and tone. RESULTS: Fifteen male patients with HD from 14 families participated in the study (mean age at the onset of disease, 18 years; range, 15-23 years). Muscle weakness and wasting were noted in the right upper limb in 12 and the left upper limb in 3, which became bilateral in 8 patients. Cold paresis was present in 6 patients and polyminimyoclonus in all patients. The EMG revealed fibrillations in 10, fasciculations in 15, and neurogenic motor unit potentials in C7, C8, and T1 myotomes in all patients. The EMG abnormalities were unilateral in 5, bilateral in 10, and subclinical in 2 patients. Spinal MRI revealed cord atrophy in 3 of 11 patients. Although family history was present in 1 brother only, the results of both SMN1 and SMN2 gene deletion studies were negative in all patients. CONCLUSIONS: The SMN gene deletion is not found in HD. Exclusive occurrence in male patients and the presence of this disease in 2 brothers suggest a possible role of the X chromosome, which needs further evaluation.

Adolescent↗
PubMed1977

Corneal exposure during sleep (nocturnal lagophthalmos).

In our referral practice at the University of Florida, corneal exposure during sleep appears to be a relatively common cause of previously undiagnosed chronic keratitis. The spectrum of the disease ranges from minimal epithelial changes that may heal later in the day to severe exposure ulcers with marked loss of stromal substance. Keratitis sicca and previous lid abnormalities are predisposing factors, but not necessary concomitants of the disease. The diagnosis can usually be made by asking the patient to gently (not forcibly) close his eyes, and usually within a minute or two, a small crack can be seen between the lids by shining light on the intrapalpebral areas-during this time there is usually some fasciculation of the lids. In our experience the most effective treatment, when ointments at bedtime are not adequate, is closing the eye by using a piece of paper tape to pull up the cheek, and then attaching the tape to the forehead, with no pad or pressure on the eye. After the initial healing, ointments at bedtime may be adequate to maintain a symptom-free state in this apparently common cause of treatment-resistant chronic keratitis.

Corneal Ulcer↗
PubMed1999

Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis.

The two main peripheral nerve sheath tumors found in patients with neurofibromatosis, type 1 (NF1), are neurofibroma, a benign tumor, and malignant peripheral nerve sheath tumor (MPNST). The tumors are related in that most MPNSTs are thought to arise by malignant transformation of neurofibromas. Such an event occurs in about 2% of NF1 patients. There are five forms of neurofibroma; three of them-localized cutaneous neurofibroma when multiple, plexiform neurofibroma, and massive soft-tissue neurofibroma-are highly specific for NF1. Only two forms of neurofibroma, plexiform and localized intraneural neurofibroma, are significant precursors of MPNST. Massive soft-tissue neurofibromas are worrisome in that they may mask MPNST arising from one of the mentioned neurofibromas. The vast majority of MPNSTs are high-grade malignant tumors with a high rate of distant metastasis. The overall 5-year survival rate for patients with MPNSTs ranges from 34% to 52%. MPNSTs generally are solitary, deep-seated globoid or fusiform tumors. They are firm, fleshy, tan, and often focally to extensively necrotic, and they invade surrounding soft tissue. On histological examination, MPNSTs are most often hypercellular, hyperchromatic, fasciculated, and mitotically active tumors. Low-grade tumors account for only about 10-15% of cases. Twenty percent of MPNSTs have unusual and potentially misleading histological features, such as epithelioid cells and divergent mesenchymal or glandular differentiation. Am. J. Med. Genet. (Semin. Med. Genet.) 89:23-30, 1999.

Humans↗
PubMed1997

Molecular characterization of E587 antigen: an axonal recognition molecule expressed in the goldfish central nervous system.

The E587 antigen (Ag) is a 200-Kd membrane glycoprotein originally identified by a monoclonal antibody on new and regenerating retinal ganglion cell axons in the adult goldfish. We report the isolation of cDNAs encoding the E587 Ag and identify it as a member of the L1 family of cell adhesion molecules (CAMs). The predicted amino acid sequence of E587 Ag shows an approximately equal identity (40%) to mouse L1, chick neuron-glia CAM, and chick neuron-glia-related CAM. Although the overall similarity is low, there is a high conservation of structural domains and specific sequence motifs. Wholemount in situ hybridizations were performed on goldfish between 34 hours and 3 days postfertilization (pf). A dramatic increase in E587 Ag mRNA was observed between 34 and 48 hours pf. The expression of E587 Ag mRNA in neurons shortly precedes axonogenesis. A marked decrease in expression occurs by 3 days pf, when the axonal scaffold has already been established. Wholemount immunohistochemistry on embryos demonstrates expression of E587 Ag on all major tracts. E587 Ag is absent from mature retinal ganglion cell axons, but its expression is induced by optic nerve transection. A corresponding induction of E587 Ag mRNA in retinal ganglion cells is shown by in situ hybridization. Furthermore, E587 Ag mRNA was detected in the optic nerve, which suggests that nonneuronal cells also express this molecule. E587 Ag was previously shown to promote retinal axon fasciculation and outgrowth in young fish and to mediate axon-glial interactions in vitro. The expression pattern and developmental regulation of E587 Ag in the central nervous system, its reexpression in retinal ganglion cells following optic nerve transection, and its relation to the L1 family indicate that E587 Ag functions as a cell recognition molecule important during axonal growth and regeneration.

Amino Acid Sequence↗
PubMed1997

Expression of the cell adhesion proteins BEN/SC1/DM-GRASP and TAG-1 defines early steps of axonogenesis in the human spinal cord.

We have studied the expression pattern of two cell adhesion proteins of the immunoglobin (Ig) superfamily, BEN/SC1/DM-GRASP (BEN) and the transient axonal glycoprotein TAG-1, during the development of the human nervous system. This study was performed by immunocytochemistry on sections of human embryos ranging from 4 to 13 weeks postconception. The overall distribution of the two proteins during development is very similar to that reported in other vertebrate species, but several important differences have been observed. Both proteins exhibit a transient expression on selected neuronal populations, which include the motor and the sensory neurons. In addition, BEN was also detected on virtually all neurons derived from the neural crest as well as in nonneuronal tissues. A major difference of expression with the chick embryo is that, in the motor neurons, BEN expression was not observed at early stages of development, thus arguing against a role of this molecule in pathfinding and fasciculation. BEN was observed to be restricted to subsets of motor neurons, such as the medial column at the upper limb level. Expression was also detected in a laterodorsal population of the ventral horn cells, which are likely to correspond to migrating preganglionic neurons that originate from the motor pool at the thoracic level. TAG-1 was found on commissural neurons and weakly on the sympathetic neurons; it was also detected on restricted nonneuronal populations. In addition, we observed TAG-1 expression in fibers that could correspond either to subsets of dorsal root ganglia (DRGs) central afferences (including the Ia fibers) or to the axons of association interneurons and in scattered motoneurons likely to correspond either to preganglionic neurons, to gamma-motoneurons, or to late-born motoneurons. Therefore, our results indicate that the molecular strategies used to establish the axonal scaffolding of the nervous system in humans are extremely conserved among the different vertebrates.

Axons↗