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PubMed1983

Cytoplasmic body myopathy. Report on a family and review of the literature.

A 15-year-old girl who was seen for scoliosis presented with cardiorespiratory failure associated with a respiratory infection. She was found to have weakness predominant in the face, sternomastoid, proximal limb, respiratory, spinal and cardiac muscles. The serum creatine kinase level was slightly elevated and the electrocardiogram was abnormal. The electromyograph was consistent with a myopathy. The course was malignant. Her 14-year-old brother had similar findings and succumbed at the age of 14 and one-half years from cardiorespiratory failure. The mother had minimal weakness of proximal limb muscles since early life. The tendon reflexes were normal as was the serum creatine kinase level. The course was benign. On light microscopy the muscle biopsy in the girl showed fibre diameter variation, centrally placed nuclei, necrosis, fibrosis and cytoplasmic bodies. The muscle biopsy in the brother and mother had similar findings except that the inclusion bodies were not seen in the mother. On electron microscopy, the girl showed typical cytoplasmic bodies, involving predominantly type 1 fibres. The mother also had these structures. The literature is reviewed and the origin, pathogenesis and aetiology of the cytoplasmic body are discussed.

Adolescent↗
PubMed1985

Multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a case and review of the literature.

A 31/2-year-old girl presented with frequent falls. She had an unsteady gait, delayed behavioural development absent tendon reflexes and in the legs decreased strength tone and equivocal plantar responses. She then developed ataxia, nystagmus, choreoathetosis, cranial nerve palsies, diminished strength and tone in the arms, sensory deficit in the limbs and autonomic nervous system dysfunction. She became progressively less responsive and succumbed at the age of 63/4 years. Examination of the central, peripheral and autonomic nervous system showed ubiquitous neuronal intranuclear hyaline inclusions and neuronal loss in several sites.

Amino Acids↗
PubMed1989

Quantitative measures of neurological function in chronic neuromuscular diseases and ataxia.

Interexaminer and intraexaminer reliability were determined for 30 quantitative measures of neurological function, including sensory threshold, tendon reflexes, maximum isometric strength, and timed tests and coded ratings of functional ability, in patients with Charcot-Marie-Tooth disease (n = 30), idiopathic polyneuropathy (n = 16) and spinocerebellar degeneration (n = 30). Five of 6 sensory and reflex measures had interexaminer reliability greater than 0.80 when the neurologists' examinations were 1 h apart; 2 of 7 achieved this level when the examinations were separated by 1 month. Interexaminer reliability between physical therapists was greater than 0.80 for 19 of 20 measures of strength and functional ability. Intraexaminer reliability coefficients greater than 0.80 were found for 13 of 24 sensory and reflex measures, 28 of 30 isometric strength measures, 24 of 30 timed tests and 5 of 6 coded ratings of functional ability. Electro-oculography, oral diadochokinetic syllable rates, hand and foot accelerometry, and tracings of sine and square waves were examined as quantitative indices of ataxia for patients with spinocerebellar degeneration. Of 11 measures of ataxia, only the oral diadochokinetic syllable rate test had acceptable reliability.

Activities of Daily Living↗
PubMed1990

Bickerstaff brainstem encephalitis. A grave non-demyelinating disease with benign prognosis.

We describe 6 patients with ophthalmoplegia, ataxia and normal or exaggerated deep tendon reflexes. All had been preceded by a febrile illness and had a full recovery without sequelae. The brainstem auditory evoked potentials showed a localised lesion in the upper brainstem while the pattern shift visual evoked potentials were normal and did not show any additional silent lesions. CSF IgG oligoclonal bands were not detected in any of the patients. MRI in 2 patients showed a confluent high intensity lesion in the upper mesencephalon and thalamus involving white and gray matter. Follow-up ranged from 6 to 24 months and showed no relapse.

Adult↗
PubMed1990

Acute or subacute alcoholic neuropathy mimicking Guillain-Barré syndrome.

Over the last 10 years we have encountered 8 patients with chronic alcoholism who presented with severe symmetrical polyneuropathy, primarily proximal in 6, which evolved over a period of 24 h to 3 weeks. In 3 cases, artificial ventilation was required. Sensory symptoms were in all instances intense, and tendon reflexes absent. CSF protein levels were normal. The course was one of gradual improvement, often incomplete with residual motor and distal sensory deficits. Three patients died within 2 months to 2 years with multiple and severe pathologies attributable to chronic alcoholism. There was no evidence for disorders other than the alcoholism and malnutrition. Electrophysiological findings were consistent with predominantly axonal lesions and nerve biopsy specimens confirmed acute and severe axonal lesions. Several of these patients had been referred to us with a possible diagnosis of Guillain-Barré syndrome due to the severity of the neuropathy and the rapidity of its onset (Landry syndrome). Acute alcoholic neuropathy is distinguishable, however, on clinical, electrophysiological and morphological grounds.

Acute Disease↗
PubMed1994

Infantile onset spinocerebellar ataxia with sensory neuropathy: a new inherited disease.

We report the clinical findings in 19 Finnish patients, including six pairs of siblings, with a new, early onset spinocerebellar ataxia. The slowly progressive clinical symptoms manifested between one and two years of age in previously healthy infants. The first manifestation of children at that age was clumsiness and loss of ability to walk. Ataxia, athetosis and muscle hypotonia with loss of deep tendon reflexes were discovered on clinical examination. By school age ophthalmoplegia and hearing loss were diagnosed, while sensory neuropathy developed by adolescence. In addition, an acute crisis with status epilepticus was a late manifestation. We found a marked decrease in sensory nerve condition velocities, a progressive loss of myelinated fibers in sural nerve specimen, and abnormal background activity in EEG with advancing age. The main finding in neuroradiological investigations was cerebellar atrophy. The occurrence of the disease in siblings and lack of manifestations in parents indicate recessive inheritance.

Adolescent↗
PubMed1996

Multi-system signs and symptoms in X-linked ataxia carriers.

Neurological, auditory, vestibular and ocular motor examinations were performed on 3 definite and 3 possible heterozygous carriers of a previously described X-linked multi-system disorder with early childhood onset, rapid progression and a fatal outcome (Arts et al., 1993). The symptoms, i.e., delayed motor development, ataxia, hearing loss and subnormal intelligence, were so evident in 2 of the possible carriers that they could be redesignated as probable carriers. Other symptoms in the definite and probable carriers were clubfeet, dysarthria, intention tremor and abnormal gait, while their signs included dysdiadochokinesia, ataxic paraplegia, abnormal muscle tendon reflexes and extensor plantar responses. All the symptomatic carriers developed moderate-to-severe sensorineural hearing loss with normal stapedial reflexes and brain stem auditory evoked potentials (BAEPs) in those in whom this could be evaluated. Speech discrimination was disproportionally poor unilaterally in one case from whom no BAEPs could be obtained because of her degree of hearing loss. Various combinations were found of high gain of the vestibulo-ocular reflex, spontaneous nystagmus and directional preponderance of vestibularly evoked nystagmus, slowing, hypometria or multi-stepping of saccades, saccadic intrusions of eye movements (macro square wave jerks, double saccadic pulses), impairment of smooth pursuit eye movements and optokinetic nystagmus, and failure of visual fixation suppression of vestibularly evoked nystagmus. Such findings indicate major involvement of the (vestibulo)cerebellum and the vermis. MRI in one carrier showed mild cerebellar atrophy.

Adult↗
PubMed1992

Dental and oral findings in patients with familial dysautonomia.

Familial dysautonomia is an inherited autosomal recessive disease found almost exclusively in Ashkenazi Jews. It is characterized by selective damage to the sensory, motor, and autonomic peripheral nervous system. The main clinical features include decreased pain sensation, impaired temperature and blood pressure regulation, lack of tearing, absent tendon reflexes, and fungiform papillae on the tongue. The purpose of this study was to explore in depth and to verify the oral and dental status in familial dysautonomia. Twenty-two patients and 44 match-paired healthy persons of Ashkenazi descent were examined. Patients and parents had only little concern for their oral condition. Caries prevalence was lower than normal and plaque accumulation increased in all patients. Dental trauma was found in 59% of the patients, and 32% showed orodental self-mutilation. Dental age was within normal range, and dental arch measurements implied proportionally small jaws and little crowding. The low caries rate may be related to the known "hypersalivation" in familial dysautonomia and/or a possible change in the salivary composition and content, caused by chronic autonomic denervation.

Adolescent↗
PubMed1988

Antibodies against acetylcholinesterase and low levels of cholinesterases in a patient with an atypical neuromuscular disorder.

Antibodies against acetylcholinesterase were found in the serum of a patient presenting dyspnea, generalized muscle paresis, diminished tendon reflexes, and fasciculations. Electrodiagnostic studies showed a decremental response, an incomplete interference pattern, and reduced motor nerve conduction velocity. Edrophonium administration resulted in extreme cholinergic crisis. Biopsies displayed muscle atrophy and nervous tissue degeneration. Recurrent acute respiratory failure ended in death. The patient's serum pseudocholinesterase and red blood cells acetylcholinesterase levels were generally very low, with periodical fluctuations. Minute quantities of the patient's serum inhibited the activity of cholinesterases from normal human serum and from various fetal tissues. Enzyme inhibition was abolished following preadsorption of the serum immunoglobulins with goat antihuman Fab, and radioiodinated acetylcholinesterase from human erythrocytes was precipitated by the patient's serum, confirming that anticholinesterase antibodies were present. Acetylcholinesterase extracted from fetal striated muscle with detergent and salt was inhibited to a larger extent than the enzymes similarly prepared from other fetal tissues and more efficiently than buffer-soluble muscle enzyme. These findings suggest that the patient's serum contained antibodies which interacted preferentially with the membrane-associated forms of muscle acetylcholinesterase and indicate that autoantibodies against acetylcholinesterase could play a role in the pathogenesis of the disease.

Acetylcholinesterase↗
PubMed1994

Spinal canal stenosis at the level of the atlas: case report.

We describe a rare case of cervical canal stenosis at the level of the atlas, presenting with cervical myelopathy as an initial symptom. A 55-year-old man was admitted to our hospital with a 4-month history of gait disturbance and clumsiness in both hands. He had no history of trauma, and showed left-sided hemihyperesthesia and mild quadriplegia with exaggerated deep tendon reflexes. Plain cervical tomography revealed marked spinal canal stenosis, with the diameter of 8.0 mm at the level of the atlas. Magnetic resonance imaging demonstrated severe compression of the spinal cord at the atlas, with an extensive high intensity area indicating edema on T2-weighted images. The patient showed an excellent recovery after laminectomy of the atlas combined with decompression of the lower posterior fossa of the foramen magnum and dural plasty.

Cervical Atlas↗
PubMed1995

Functional prognosis after treatment of spinal radiculomeningeal arteriovenous malformations.

BACKGROUND: We performed surgical treatment successfully in six patients with spinal radiculomeningeal arteriovenous malformation (AVM); however, only four patients showed improvement of gait function postoperatively. METHODS: These experiences prompted us to review the clinical findings and their possible association with the functional outcome in 33 reported cases of radiculomeningeal AVM together with those in our six patients. RESULTS: Statistical analysis revealed that the duration from onset of symptoms until diagnosis, the age at the time of treatment, the condition of the deep tendon reflexes (DTR) in the lower extremities, as well as the severity of both gait and urinary disturbance before treatment were significantly correlated with the functional outcome. CONCLUSIONS: A patient under 70 years old, who is treated within 2 years 6 months after the onset, whose gait or urinary disturbance is slight or moderate, and without absence of DTR in the lower extremities, is expected to have a good functional prognosis after treatment.

Aged↗
PubMed1991

Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China.

Acute peripheral nervous system diseases leading to paralysis in children are rare in Europe and the USA, whereas epidemics of a Guillain-Barré-like syndrome occur annually among children in rural parts of northern China. To clarify the features of this disorder 36 patients, aged 15 months to 37 years (median 7) with this syndrome were investigated; 91% were from rural areas. In 47%, a prodromal illness was reported in the preceding 4 weeks. Leg weakness and resistance to neck flexion were the earliest symptoms. The weakness ascended rapidly and symmetrically to affect the arms and respiratory muscles, with maximum weakness occurring a mean of 6 days after onset of weakness. Bulbar weakness occurred in 61% of patients, but only 1 had extraocular paresis. Respiratory assistance was needed by 31% of patients. Tendon reflexes were lost as weakness developed. 42% of patients had raised concentrations of protein in the cerebrospinal fluid, and the mean cell count was 3 cells/microliters (range 0-12/microliters). Electrodiagnostic studies in 22 patients showed severe reductions in motor evoked amplitudes from distal stimulation. Sensory action potentials were normal. Electromyography revealed denervation potentials in limb muscles. The distinctive epidemiological, clinical, and neurophysiological characteristics of this illness suggest that the disorder is different from both Guillain-Barré syndrome and poliomyelitis. The neurophysiological findings support the hypothesis that the disorder is a reversible distal motor nerve terminal or anterior horn cell lesion.

Acute Disease↗
PubMed1975

A forme fruste of the pterygium syndrome?

A case of a girl with popliteal webbing associated with limited extension of the knee, flexion contractures of the third, fourth, and fifth digits, hypothrophia of leg musculature, and absent tendon reflexes in the legs is reported. Between 19 months and 6 years of age, the digital contractures, popliteal webbing, and limitation of knee extension progressively improved. This is the first reported instance of this combination of signs and the first report of regression of some of these physical findings.

Abnormalities, Multiple↗
PubMed1993

Heterogeneous findings in four cases of cerebellar ataxia associated with hypogonadism (Holmes' type ataxia).

We report four sporadic cases of cerebellar ataxia associated with hypogonadism. All patients were female. The neurological symptoms appeared in the first three decades. Apart from ataxia, the most frequent features were nystagmus, dysarthria, mental impairment, brisk tendon reflexes, skeletal deformities, peripheral neuropathy, and tremor. Neuroimaging studies showed constant cerebellar atrophy, in some instances associated with involvement of either grey or white cerebral matter. Neurophysiological studies demonstrated an axonal neuropathy. Endocrine evaluation showed heterogeneity of the hypogonadism, which was hypogonadotrophic in one patient and hypergonadotrophic in the other three. One patient had partial deficiency of muscle cytochrome c oxidase. The syndrome appears to be a heterogeneous multisystem disorder and in some cases a mitochondrial metabolism deficiency could be suspected.

Adolescent↗
PubMedDate not supplied

Dominantly inherited early-onset non-progressive cerebellar ataxia syndrome.

A mother and daughter with suspected dominantly inherited, early-onset, non-progressive cerebellar ataxia syndrome have been reported. A review of the literature and the clinical features of the present cases revealed the nosologic features of this rare disorder, possibly dominant inheritance, floppiness and delayed milestones preceding early-onset mild cerebellar ataxia, non-progressive clinical course, retained or even brisk tendon reflexes without pyramidal tract involvement, normal or slightly delayed intelligence, and occasional nystagmus. Neuroimaging reveals selective involvement of the cerebellum, which is prominent in the vermis and the anterior part of the hemispheres.

Adult↗
PubMedDate not supplied

Jittery babies: a short-term follow-up.

Thirty-nine full-term babies, appropriate for gestational age, and otherwise healthy, were followed-up in our neurology clinic because of jitteriness, until complete resolution of symptoms and neurological findings. The babies were examined at 3 month intervals, and were classified according to the severity of their associated neurological findings, hypertonicity and increased tendon reflexes, into two groups: 'mild' (n = 24), and 'moderate-to-severe' (n = 15). The mean follow-up period was 13.5 months. In 81% of the study population, jitteriness and neurological findings disappeared before the age of 9 months. In only 11% did they persist beyond the age of 1 year. The mean time until disappearance of associated neurological findings was significantly shorter (5.5 months) in the mild group, compared to the moderate-to-severe group (9.5 months). Only one infant displayed motor delay and required physiotherapy.

Child, Preschool↗
PubMed1989

Magnesium toxicity secondary to catharsis during management of theophylline poisoning.

Multiple doses of oral activated charcoal are used increasingly to promote elimination of toxins that have already reached the bloodstream; this is often referred to as gastrointestinal dialysis. Cathartics usually are used in conjunction to hasten transit of the charcoal-adsorbed toxin. In the present case, a regimen of activated charcoal and magnesium citrate was used to treat a patient with theophylline poisoning. It was effective in lowering the patient's serum theophylline concentration but produced an elevated magnesium level associated with decreased responsiveness, confusion, and diminished deep tendon reflexes. Magnesium citrate may not be the optimal cathartic for use in gastrointestinal dialysis, at least in selected patients. Sorbitol has been shown to produce a more rapid catharsis without disturbing magnesium serum concentrations. Therefore, the use of sorbitol in place of magnesium citrate, at least in selected patients, may be preferred.

Aged↗
PubMedDate not supplied

Spinal cord ischemia after cardiac arrest.

Subsequent to cardiac arrest, a 58-year-old man with intractable dysrhythmia and severe arteriosclerosis developed flaccid paraplegia, depressed deep tendon reflexes, and showed no pain or temperature sensation caudal to Th-7 in spite of completely intact proprioception and vibration sensation. An echocardiogram showed no clots or vegetation on the prosthetic valve and no thrombus in the left atrium or left ventricle. The patient's paraplegia was permanent, at least through a follow-up period of 2 years. These findings suggest that the etiology was spinal cord ischemia due to blood supply in the area of the anterior spinal artery (ASA); however, magnetic resonance T2-weighted imaging demonstrated signal abnormalities throughout the gray matter and in the adjacent center white matter. Somatosensory-evoked potentials (SEP) measure neural transmission in the afferent spinal cord pathway, which is located in the lateral and posterior columns of the white matter; these showed a delay in latency between Th-6 and Th-7. The spinal cord is as vulnerable to transient ischemia as the brain. Spinal cord ischemia after cardiac arrest results from principal damage in the anterior horn of the gray matter, the so-called ASA syndrome; however, the pathways of SEP and pathogenesis of the spinal cord ischemia need further investigation.

Evoked Potentials, Somatosensory↗