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Subclinical effects of chronic increased lead absorption--a prospective study. II. Results of baseline neurologic testing.

This report summarizes the results of baseline neurologic testing in a group of apparently healthy workers from a secondary lead smelter and a group of controls from nearby aluminum processing plants. The test battery included a standard neurologic examination nerve conduction measurements, quantitative oculomotor function tests and detailed audiologic studies. Lead workers and controls were intermixed so that the examiners were unaware of the status of any individual being tested. Although the lead workers reported significantly more neurologic symptoms than the controls, relatively few differences were found on quantitative neurologic testing. Decreased deep tendon reflexes occured more frequently in the lead workers than in the controls (22% vs. 11%) but the difference was of borderline significance (p=0.06) and other signs of peripheral neuropathy occurred with equal frequency in both groups. The mean motor conduction velocity and sensory latency measurements were not significantly different in the lead workers and in the controls and, of the six oculomotor function measurements, only the mean accuracy of saccadic eye movements was significantly (p less than 0.01) different in the two groups. High frequency hearing loss occurred with equal frequency and severity in both groups, consistent with the level of noise exposure in the lead and control plants.

Absorption↗

Postpartum hypokalemic paralysis.

An attack of hypokalemic paralysis occurring in a 23-year-old woman during the immediate postpartum period of her fourth pregnancy is described. A flaccid paralysis of her extremities and markedly diminished deep tendon reflexes developed 3 hours after delivery, at which time her serum potassium level was found to be 1.7 mEq/liter. Hypokalemic paralysis has not been previously reported as an immediate postpartum complication. The pathophysiology and management of this condition are discussed, as are the treatment and follow-up of this specific patient.

Adult↗

Hyperthyroidism with periodic paralysis.

Hyperthyroidism may be associated with hypokalemic periodic paralysis. Two cases are presented demonstrating intermittent attacks of flaccid paralysis associated with clinical symptoms, signs and laboratory findings of hyperthyroidism. During an attack, one patient had a serum potassium of 2.1 mEq. per litre.Various factors such as trauma, exposure to cold, excessive carbohydrate ingestion and certain medications have been stated to precipitate an episode of paralysis. Attacks may range from mild weakness to generalized flaccid paralysis with loss of deep tendon reflexes. Several reported patients have died owing to cardiac arrest or respiratory paralysis.During attacks, the serum potassium is usually in the range of 2.2 to 3.2 mEq. per litre. It is postulated that a metabolic abnormality affecting the muscle-cell membrane can occur in the hyperthyroid state resulting in a shift of potassium to the intracellular position, thus producing a situation of hyperpolarization of the muscle-cell membrane which in turn alters the muscle contractibility.The importance of recognizing the unusual association of hypokalemic periodic paralysis with hyperthyroidism is stressed because, with successful treatment of the hyperthyroidism, the episodes of paralysis disappear.

Adult↗

[Juvenile, non-progressive muscular atrophy localized in the hand and forearm. Report of 16 cases (author's transl)].

Sixteen cases of juvenile, non-progressive muscular atrophy localized in the hand and forearm were seen at our neurology out-patient clinic for the past 8 years. The analyses of these 16 cases disclosed characteristic features as follows: 1) juvenile onset 2) male preponderance 3) unique distribution of muscular atrophy and weakness in the hand and forearm. 4) insidious onset, initial progressive period and subsequent non-progressive course 5) tendon reflexes of the arms are hypoactive in half of the cases 6) no pathological reflexes 7) cold paresis 8) no definite sensory disturbance 9) no cranial nerve involvement 10) neurogenic patterns on EMG According to these features, this clinical entity carrying good prognosis must be differentiated from several diseases associated with similar muscular atrophy of extremities, especially amyotrophic lateral sclerosis which is notorious as a fetal disease.

Adolescent↗

Myasthenia gravis and the myasthenic syndrome.

Two disorders of neuromuscular transmission producing muscle weakness and easy fatigability which may confront the physician are myasthenia gravis and the myasthenic syndrome. The former has early symptoms and signs of oculobulbar and then extremity weakness with rapid decline of action potential and contractile strength with repetitive use and nerve-muscle stimulation. Anticholinesterases improve strength. The myasthenic syndrome has early symptoms and signs of pelvic girdle, pectoral girdle and proximal limb muscle weakness. This is worst when first starting to use or carry out nerve muscle stimulation in the rested muscles. It improves significantly for a time with use or on rapid stimulation, and then declines with continued activation. Deep tendon reflexes are sluggish or absent. Small cell carcinoma of the lung is often associated. Guanidine improves the strength. Other features and possible underlying mechanisms of the two disorders help to differentiate and treat them.

Adult↗

Neuromyopathy in malignant hyperthermia.

A 31-year old male developed malignant hyperthermia (MH) during the initial minutes of Halothane induction. CPK rose to 6120 U/ml and decreased to normal levels as the patient became afebrile over a 10 day period of cooling measures and metabolic management. Muscle weakness, predominantly proximal and depressed deep tendon reflexes were found upon examination during convalescence. Muscle biopsy showed neurogenic changes characterized by fiber type grouping and targetoid fibers. CPK was elevated in one of the patient's children. This case supports the view of underlying hereditary neuromyopathy in MH.

Adult↗

Ketazolam treatment for spasticity: double-blind study of a new drug.

A minor tranquilizer, ketazolam, was tested in a double-blind, randomized, crossover study of 50 patients for its effects in neurologic spasticity. The drug was compared with diazepam (widely accepted as an effective antispasticity agent) and a placebo. The patients with spasticity were almost all cases of multiple sclerosis (24) or stroke (24). Thirty-nine patients completed the study. There was not statistically significant superiority of either diazepam or ketazolam, but both relieved symptoms significantly better than the placebo, as measured clinically and by electromyographic recording of deep tendon reflexes. Ketazolam is a relatively safe and clinically effective antispasticity agent (especially for patients with multiple sclerosis). The well-known "big 3"--dantrolene sodium, baclofen, and diazepam--produce large and small problems in many individual cases; hence, ketazolam now offers a safe and clinically useful alternative.

Adolescent↗

Orthopedic aspects of congenital insensitivity to pain.

In an 11-year-old girl with congenital insensitivity to pain, diagnosis depended on three diagnostic features: pain sensation absent from birth; entire body affected; all other sensory modalities and deep tendon reflexes present. The cause of this disease is unknown. Other diseases to be considered when insensitivity to pain is present are diabetes, lues, and syringomyelia. Less common neurologic diseases are congenital sensory neuropathy with or without anhidrosis, familial dysautonomia (Riley-Day syndrome), and sensory radicular neuropathy. The three orthopedic manifestations of congenital insensitivity to pain are recurrent fractures, neuropathic (Charcot's) joints, and osteomyelitis. Management is based on proper appreciation of the disease. Prevention of complications is important. Treatment of fractures and osteomyelitis is straightforward. However, the treatment of neuropathic joints demands caution and is done best nonsurgically.

Arthropathy, Neurogenic↗

[A case of brain stem encephalitis with complete recovery (Bickerstaff's encephalitis)].

A 17 year old high school boy experienced fever and diarrhea, which subsided within 4 days by appropriate medications. Six days later, however, he developed unsteadiness and limb spasm. On the morning of admission, he was found to have drowsiness, dysarthria, gait disturbance and involuntary jerks. When he was brought to the hospital, he was lethargic but could follow simple verbal commands. Frequent involuntary movements manifested by facial grimacings, limb spasms and twitchings with dystonic features were seen. Decorticate posturing was readily elicited by painful stimuli. There was no meningeal irritation sign or gross sensory impairment. The deep tendon reflexes were symmetrically exaggerated with bilateral Babinski signs. Bilateral lateral rectus muscle weakness was found together with mild ptosis and upward gaze limitation. Nystagmus was not present and the funduscopic examination was normal. Immediately he was placed on anticonvulsants, steroid hormone, gamma-globulin and antibiotics as well. A brain CT scan and a CSF examination revealed no abnormality. Meanwhile he continued to show a progressive deterioration associated with fever and status epilepticus, and within 24 hours he lapsed into coma in decorticate posture. An EEG obtained at the 3rd hospital day was compatible with spindle coma. In spite of aggressive treatment he remained febrile and comatous. Therefore, vidarabine (adenine arabinoside) was initiated from the 3rd hospital day for 5 days. Then he began to groan and show frequent choreic movements. For the subsequent 2 weeks he made a slow recovery.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Function of the neuromotor apparatus in diabetes mellitus depending on metabolic compensation].

Electromyography was used to show that metabolism decompensation in diabetes mellitus induces reversible muscular changes, manifesting in the decreased tendon reflex and motor response amplitudes. These indices of neuromotor apparatus function return to normal in diabetes compensation. Meanwhile, diabetes compensation does not lead to rapid recovery of the affected nervous conduction and spinal motoneuronal excitability.

Diabetes Mellitus↗

[Protective effect of isaxonine against vincristine-induced neuropathy (author's transl)].

A double-blind survey versus a placebo was carried out with C.P.V. protocole on 25 patients treated for lymphosarcoma or reticulosarcoma; its aim was to appraise the preventive action of isaxonine on the appearance of neuropathy induced by vincristine. The frequency of such neuropathy was clearly lower in the isaxonine group (3/10) than in the placebo group (8/10) in a statistically significant manner. The protective effect of isaxonine was particularly striking on tendon reflexes and on the number of motor units recorded at musculus extensor digitirum pedis brevis; on the contrary, the patients treated with the placebo showed real denervation.

Adult↗

[Controlled trial of isaxonine in traumatic or ischaemic (compression) neuropathies (author's transl)].

Fifty patients showing one or more impairments to large nerve trunks due to trauma or ischaemia received, after drawing lots, either isaxonine or a placebo. The evaluation criteria of the recovery were both clinical (testing of muscles, sensory disorders, vegetative disorders, tendon reflexes, tonicity, amyotrophy) and electrophysiological (electromyogram, conduction velocity, distal latency time, H reflex and H/M index). This study shows two major elements: the action of isaxonine shortens healing time (45-90 days instead of 120-150 days) and isaxonine allows neurological recovery to resume at neurologic level whereas the situation was dating back more than three months.

Clinical Trials as Topic↗

[Hypertrophic neuropathies beginning in infancy: a study of 3 cases (author's transl)].

The genetical forms of hypertrophic neuropathies, inherited either as recessive or autosomal dominant trait, are classified, according to Dyck (1975), as HMSN type I, III, and IV. Sporadic cases are also reported. We studied three patients, one with autosomal recessive inheritance, and two without family history, who had the following common features: --onset of symptoms before the age to two years; --slowly progressive course; --peroneal muscular atrophy with absent tendon reflexes; --reduction of MCV and SCV; --decreased number of myelinated fibers; --schwannian cell hyperplasia, with onion bulb complexes formation; --absence of aspects of hypomyelination; --increased number of collagen pockets and denervated Schwann-Remak cells or processes. On light microscopy, multilamellated onion bulbs of large size were found in a very high percentage in case 1, while there were either simple in type or in a lower percentage in case 2. In the third, case, onion bulbs were recognized only on electron microscopy. It is known that in the various kinships affected with type I of HMSN, the pathological changes of peripheral nerves differ greatly. Therefore, despite early onset of symptoms and varying degree of severity of nerve changes, all three cases have been classified within the group of HMSN type I. The different severity of nerve damage may suggest the possibility of a genetical heterogeneity in this disorder.

Adolescent↗

Cervical radiculopathy: the diagnosis and treatment of pathomechanics in the cervical spine.

Lateral flexion anteroposterior roentgenograms of the cervical spine were obtained of patients suffering from cervical radiculopathy. Specific attention was given to the normal mechanical attitudes of the cervical motion segments at their end range. Deviations from the normal coupling mechanism in the cervical spine were directly related to the level of clinical radiculopathy. Aberrancies in the coupling mechanism of the cervical motion segments were reduced by manipulation, and post manipulative reduction examination revealed improvements in sensation, motor power, and deep tendon reflexes, as well as subjective patient improvement. Normal mechanical attitudes could be restored by manipulative reduction of the cervical motion segments.

Adolescent↗

[Human peripheral neurolymphomatosis].

Two further cases of human neurolymphomatosis are reported, and clinical, histopathologic and nosologic features of the affection reviewed with respect to findings in these 2 patients and in 7 other reported cases. Clinical manifestations are those of a flaccid ascending paralysis, associated in most cases with an asymmetrical abolition of tendon reflexes, violent muscular pains and sphincter disturbances, frequently preceded by a regressive cranial nerve palsy. Pathology shows infiltration of the peripheral nervous system (nerves, roots, and spinal ganglia) by lymphoid cells, sometimes associated with central nervous system (cord and brain) infiltrates in severe advanced cases. Involved nerves are increased in size. The disease could be a viral polyradiculoneuritis due to the virus of Marek's disease. Arguments in favor of this hypothesis include: the onset of the affection in subjects working in poultry farms under poor hygienic condition; the fact that clinical and histologic findings are similar to those in Marek's disease and the failure, after careful examination, to detect any malignant blood disorder or lymphoma in one of the cases studied.

Adult↗

[A case of Forestier's disease causing dysphagia].

A case of Forestier's disease causing dysphagia is reported. A 52-year-old man was admitted to our hospital because of dysphagia, hoarseness and constant urge to clear the throat. On admission, physical examination revealed a hard mass between the larynx and left sternocleidomastoid muscle. Deep tendon reflexes were all hyperactive and Hoffmann's sign was elicited on the right side. Cervical roentogenogram showed extensive anterior excrescences from C-2 to C-7 vertebra with preservation of the relatively normal intervertebral disc space. CT scan of the cervical spine showed anterolateral displacement of the trachea by the anterior excrescences of the cervical vertebrae and narrow flat spinal canal. Esophagram showed marked right lateral displacement of the esophagus by the cervical osteophytes. Myelogram revealed a narrow canal and anterior filling defects by the posterior osteophytes at C3/4 and C6/7. Technetium 99m bone scintigram disclosed abnormal uptake in the cervical and thoracic spines. The patient underwent anterior cervical exploration for removal of the exostotic bone from C-3 to C-7 and decompression of the esophagus and trachea. Postoperative course was uneventful and all of his preoperative symptoms improved remarkably. Pertinent literature were reviewed and pathogenesis of Forestier's disease was discussed.

Deglutition Disorders↗

[A case of intracranial malignant lymphoma with pure akinesia and repeated regression on CT scans].

In a case of primary reticulum cell sarcoma in the brain, histologically verified by biopsy, the tumor regressed twice on a CT scan without radiotherapy. The systemic freezing phenomenon was seen as a main clinical symptom. The patient, a 44 year-old male, first complained of decreased livido and festinating speech. He also showed frozen gait, micrographia, a decrease in spontaneity and urinary incontinence. Four months after onset he was hospitalized. Neurological findings on admission revealed freezing of gait, writing, and speech, but there was no weakness of muscles with normal tendon reflexes, and normal muscular tone. In the CT scan on admission, there were high density areas mainly in the head of the right caudate nucleus, the medial deep portion of the right frontal lobe, the right side of the hypothalamus, the anterior thalamus, the globus pallidus. There were also nodular-type enhanced effects in the same areas. Regression of the tumor was seen on the CT scans after administration of betamethasone. The tumor which had again increased in size regressed spontaneously without the use of steroids after 3 months. Thereafter, the tumor gradually became larger and an open biopsy was performed. Histopathological findings showed a reticulum cell sarcoma. There were no findings of systemic malignant lymphoma. Such intracranial malignant lymphomas showing repeated regression including spontaneous one are very rare in the literature. The freezing phenomenon in this case started with festinating speech and spread to writing and gait. L-DOPA had no effect.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[A case of spongiform encephalopathy with ataxia and amyloid plaques].

CASE: A 63-year-old man was admitted to our hospital because of gait disturbance. His daughter died of the same disorder at age 29. In 1974, at age 59, the patient noticed mild recent memory disturbance and clumsiness in handwriting. In 1976, his gait was markedly unstable, but he could walk without assistance, and his speech became dysarthric. In spring, 1977, he was unable to walk without assistance. From Jan. 9, 1978, through Feb. 14, 1978, he was hospitalized in our hospital. Neurological examination revealed an ataxic gait and scanning speech. Deep tendon reflexes of the upper extremities and ankle jerks were normal, but knee jerks were absent. Pathological reflexes were not elicited. Both superficial and deep sensations were normal. He was alert and showed no overt dementia. Laboratory data including CSF, EEG and brain CT were normal except positive TPHA in serum. Thereafter, he was followed up with the diagnosis of spinocerebellar degeneration. In 1979, he developed a limb ataxia. In spring, 1980, he became very irritable and was easily excited. He gradually developed dementia and urinary incontinence. In 1981, his illness progressed to an akinetic mutism and died of pneumonia on July 19, 1981. Myoclonus and periodic synchronous discharges in EEG were not observed until his death. The brain was 1,075 g in weight after formalin fixation and the cerebrum was generally soft. The gyri showed no evidence of abnormalities or atrophy, while the cerebellum and brain stem, especially the pons, were atrophic. The arterial and venous systems showed no remarkable changes. The spinal cord was externally unremarkable. The ventricles were dilated.(ABSTRACT TRUNCATED AT 250 WORDS)

Akinetic Mutism↗