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PubMed2003

Neurological examination in the healthy term newborn.

We carried out a cross-sectional study with a sample of 106 normal full-term newborns examined within 24 to 72 hours of birth. The following findings were evaluated: head and chest measurements, muscle strength, tone, tendon reflexes, superficial reflexes, primitive reflexes, and cranial nerves. All 106 newborns were considered neurologically normal. We found no differences in the neurological examination findings for newborns with different gestational ages. Primitive reflexes and appendicular tone in newborns examined at earlier postnatal ages tended to be less intense. We were able to determine the prevalence of certain neurological examination findings for the normal newborn and to discuss some differences between our results and those of other studies. Prevalence estimations for the different findings in our study may be valid for different populations as long as the same methodology is adopted.

Anthropometry↗
PubMedDate not supplied

Friedreich's ataxia: clinical and molecular study of 25 Brazilian cases.

INTRODUCTION: Friedreich's ataxia is a neurodegenerative disorder whose clinical diagnostic criteria for typical cases basically include: a) early age of onset (< 20 or 25 years), b) autosomal recessive inheritance, c) progressive ataxia of limbs and gait, and d) absence of lower limb tendon reflexes. METHODS: We studied the frequency and the size of expanded GAA and their influence on neurologic findings, age at onset, and disease progression in 25 Brazilian patients with clinical diagnosis of Friedreich's ataxia - 19 typical and 6 atypical - using a long-range PCR test. RESULTS: Abnormalities in cerebellar signs, in electrocardiography, and pes cavus occurred more frequently in typical cases; however, plantar response and speech were more frequently normal in this group when the both typical and atypical cases were compared. Homozygous GAA expansion repeats were detected in 17 cases (68%) - all typical cases. In 8 patients (32%) (6 atypical and 2 typical), no expansion was observed, ruling out the diagnosis of Friedreich's ataxia. In cases with GAA expansions, foot deformity, cardiac abnormalities, and some neurologic findings occurred more frequently; however, abnormalities in cranial nerves and in tomographic findings were detected less frequently than in patients without GAA expansions. DISCUSSION: Molecular analysis was imperative for the diagnosis of Friedreich's ataxia, not only for typical cases but also for atypical ones. There was no genotype-phenotype correlation. Diagnosis based only on clinical findings is limited; however, it aids in better screening for suspected cases that should be tested. Evaluation for vitamin E deficiency is recommended, especially in cases without GAA expansion.

Age of Onset↗
PubMed1983

Epidemiological and clinical studies on blindness due to diabetic retinopathy.

To clarify the epidemiological and clinical features of blindness due to diabetic retinopathy, 21 patients were studied. Blind diabetics seen at our clinic numbered 2, 3, 4 and 12 in 1965-1969, 1970-1974, 1975-1979 and 1980-1981 respectively. The ratio of males to females was about 4:3. Patients whose onset was in the 10-19, 20-29, 30-39, 40-49 and 50-59 year age group numbered 4, 5, 6, 3 and 3 respectively. No difference was seen in the duration of the disease between patients whose onset was below and patients whose onset was above 40 years of age. Most of the blind diabetics (81.8%) were treated with insulin and hypoglycemic symptoms had occurred on several occasions in 14 cases. Hypertension was a complication in 10 (45%) and orthostatic hypotension in 7 cases (31.8%). Patellar tendon reflex disappeared in 15 cases (68.2%). Proteinuria was strongly positive in 11 cases (52.4%). It was therefore concluded that the number of blind diabetics has been increasing in our clinic since 1975. Insulin therapy and the association of hypoglycemia were thought to be important precipitating factors of blindness in diabetics. The levels of plasma fibrinogen and soluble fibrin monomer complexes in blind diabetics were higher than those in diabetics without retinopathy.

Adolescent↗
PubMed1983

The prevalence of neuropathic symptoms in diabetic patients newly referred to our hospital.

100 diabetic patients who were newly referred to our hospital and 50 normal controls received 1) physical and chemical examinations, 2) a self-rating depression score (SDS), and 3) an examination consisting of 18 questions to detect subjective neuropathic symptoms. Many more neuropathic symptoms were found in diabetics than in normal controls; these symptoms included pain and numbness of the extremities, cold sensation, dizziness, constipation, impotence and sweating disorders. Few patients with neuropathic symptoms, however, exhibited neurological findings (tendon reflex, sensory disturbance or dorsal pulsation) or blood glucose control measured by HbA1. The likelihood of symptoms in the feet or in the urogenital tract increased with the duration of diabetes mellitus. Diabetics were significantly more depressive than normal controls shown by the result of SDS.

Blood Glucose↗
PubMed1984

Arousal response induced by postural changes in patients with subacute myelo-optico-neuropathy, "SMON".

In reaction time (RT) experiments, shifts of arousal level induced by postural changes were examined in 10 normal subjects and 10 patients with SMON. Vocal RTs were recorded at neutral position (N) and facilitating position of the left arm (FA) or the left leg (FL). RT-ratios between two positions, FA/N and FL/N, were used as variables to estimate the arousing effect of facilitating positions. Also the correlations between these variables and several clinical signs such as patellar tendon reflex (PTR), sensory disturbance and dysesthesia were calculated. The grade of dysesthesia was strongly related to FL/N. It is assumed that proprioceptive input from the lower extremities causes abnormally high arousal response in patients with dysesthesia.

Adult↗
PubMed1989

Relationship between serum free thyroid hormone concentrations and target organ responsiveness in thyroid disease patients before and after treatment.

Relationship between serum free thyroid hormone concentrations and several markers of peripheral tissue response to thyroid status was studied in 24 patients with hyperthyroidism, 17 with euthyroidism with goiter and 10 with primary hypothyroidism before and after the initiation of standard forms of treatment. Before treatment, serum free T4 correlated significantly, either positively or negatively, with the basal metabolic rate (BMR), duration of the achilles tendon reflex (ATR), resting pulse rate (RPR), serum total cholesterol (T-cholesterol), ratio of the pre-ejection period to the left ventricular ejection time (PEP/LVET), serum high density lipoprotein (HDL) and creatine phosphokinase (CPK) in the descending order. Serum free T3 also correlated significantly all the these parameters. After treatment, changes in both serum free T4 and free T3 correlated well with those of the BMR and ATR in the patients with hyperthyroidism, and with those of the ATR, serum CPK and T-cholesterol in the patients with hypothyroidism. These results indicate that these peripheral parameters correlated well with serum free thyroid hormone concentrations in thyroid disease patients. Therefore, these parameters can be used to assess thyroid functions in patients with thyroid hormone resistance.

Adult↗
PubMed1990

A case of bulbospinal muscular atrophy with chief complaint of sensory disorder in the lower extremities.

A 56-year-old man was admitted to our department with a chief complaint of lower extremity dysesthesia. He described a dull numbness below the ankle and a dull pain in the nates for the past two years. Although the numbness extended to the thigh, he did not notice any muscular weakness or atrophy. Neurological examination revealed weakness and atrophy in the face, tongue and the proximal portions of all four extremities. Deep tendon reflexes were decreased. A moderate loss of vibratory sensation was noted below the knees. Electromyography showed neurogenic changes. Muscle biopsy revealed both myogenic and neurogenic changes. Sural nerve biopsy revealed a mild reduction of myelinated fibers, particularly the large-diameter fibers. Based on these findings, a diagnosis of bulbospinal muscular atrophy (BSMA) was made. In recent years, there have been some case reports of BSMA with sensory disturbances, or merely with subclinical manifestations of a sensory disturbance. This case is included in the same category as those reports, but it is interesting to note that the sensory disturbance in the lower extremities occurred as the chief complaint of the disease.

Humans↗
PubMed1993

Carbamazepine overdose. Features of 33 cases.

Review of the medical records of 2 major adult teaching hospitals for a 4-year period revealed 33 instances of carbamazepine overdose. These patients had a mean age of 30 years and 58% were known epileptics. They ingested a mean of 12g carbamazepine (range 1.6 to 45g), with 51% of cases involving other drugs, particularly alcohol. The clinical manifestations of toxicity formed a recognisable clinical picture of diminished conscious state (100% of patients), mydriasis (42%), abnormal muscle tone and tendon reflexes (55%) and ataxia, nystagmus or ophthalmoplegia (48%). 24% of cases were complicated by seizures. The incidence of hyperglycaemia and hypokalaemia were related to higher drug concentrations. 12% showed hyponatraemia and 50% had transient evidence of hepatic dysfunction. The pharmacokinetic properties of carbamazepine play a role in determining management strategies. Management is largely supportive through avoidance of drug interactions, large doses of activated charcoal, careful airway management and correction of electrolyte disturbances.

Adolescent↗
PubMed1994

Vinorelbine. A review of its pharmacological properties and clinical use in cancer chemotherapy.

Vinorelbine is a semisynthetic vinca alkaloid with a broad spectrum of antitumour activity. The drug is effective as a single agent in inoperable/advanced non-small cell lung cancer (NSCLC), producing objective response rates of about 15 to 30%, and as first-line or later chemotherapy for metastatic spread in advanced breast cancer. Combining vinorelbine with standard chemotherapeutic regimens improves response rates in these indications compared with vinorelbine monotherapy: in NSCLC response rates increase to 30 to 50% when vinorelbine is administered with cisplatin. Importantly, survival was prolonged by a further 9 weeks with this combination in a trial in > 600 patients with NSCLC. Comparative trials evaluating vinorelbine in women with advanced breast cancer are few at present. However, results suggest greater efficacy for vinorelbine than for melphalan as second-line chemotherapy, and similar efficacy for vinorelbine plus doxorubicin compared with doxorubicin plus 2 other drugs as first-line chemotherapy. Vinorelbine has tended to yield superior response rates when compared with vindesine, and appears to have greater haematological toxicity (i.e. granulocytopenia) but less neurological toxicity (peripheral neuropathy, constipation, loss of deep tendon reflexes) than this agent. Myelosuppression is the most frequent cause of vinorelbine treatment delay or dose reduction. Other consequences of vinorelbine therapy are those typically seen with antineoplastic agents, such as diarrhoea, nausea and vomiting, and alopecia. However, these are rarely severe. Early clinical investigations indicate that the antitumour effects of vinorelbine in other malignancies including ovarian carcinoma, lymphoma and head and neck cancer warrant further exploration, as does the efficacy of the drug relative to standard approaches and its possible beneficial effects on quality of life of cancer patients. Clarification is also required of the feasibility of an oral dosage form, which in preliminary investigations has shown some efficacy in NSCLC, but a variable response rate and high incidence of gastrointestinal disturbances in women with breast cancer. Thus, vinorelbine as a single agent or combined palliative therapy is effective against advanced NSCLC, and as first- or second-line chemotherapy in advanced breast cancer. This semisynthetic vinca alkaloid has a manageable tolerability profile and potential for use in other malignancies and as an oral formulation. With these attributes, vinorelbine is a valuable option which extends the range of treatments available for these difficult-to-treat malignancies.

Absorption↗
PubMed1996

Spasticity after stroke. Epidemiology and optimal treatment.

Spasticity following stroke reflects a spectrum of clinical problems including increased muscle tone, abnormal limb posture, excessive contraction of antagonist muscles and hyperactive cutaneous and tendon reflexes. The prevalence of stroke-related disability in stroke survivors is high, and spasticity may be a significant component of this. Management strategies include a multidisciplinary team approach utilising a variety of rehabilitation techniques. Although some interventions are well tolerated and fairly standardised, older adults may be particularly sensitive to drug treatment-related adverse effects. This article reviews some of the commonly employed interventions, such as oral medications, and some of the newer techniques, such as intrathecal baclofen infusion and botulinum toxin injections. The optimal management of spasticity following stroke in older adults requires careful goal setting and skilful combination of treatment modalities in order to produce the best outcome.

Cerebrovascular Disorders↗
PubMed1987

Clinical experience with cyclandelate in insulin-dependent diabetic patients with neuropathy.

Previous trials have demonstrated a clinical and electrophysiological improvement of diabetic peripheral polyneuropathy in diabetic patients treated with cyclandelate at a dosage of 1600 mg/day. Hence, a double-blind randomised trial was started in 16 insulin-dependent diabetic patients presenting with symptoms of neuropathy, an increased vibration perception threshold (VPT), disturbed tendon reflexes at lower limbs and an EMG showing a significantly decreased motor nerve conduction velocity (MCV) of the peroneal nerves. The placebo-treated group and the cyclandelate-treated group were not significantly different regarding age, duration of diabetes and level of metabolic control (measured as total HbA1), which remained unchanged during the year of observation. In the cyclandelate-treated group, pathological sensation improved significantly in 7 of 8 patients. MCV, measured under standardised conditions, increased significantly during the first 6 months of treatment, while mean VPT did not change. In the placebo group 3 of 8 patients showed an improvement of sensation, 3 did not feel any change and 2 worsened. Neither mean MCV nor VPT changed significantly. No severe side effects were observed during the study period.

Adult↗
PubMed2004

Poisoning due to chlorophenoxy herbicides.

Chlorophenoxy herbicides are used widely for the control of broad-leaved weeds. They exhibit a variety of mechanisms of toxicity including dose-dependent cell membrane damage, uncoupling of oxidative phosphorylation and disruption of acetylcoenzyme A metabolism. Following ingestion, vomiting, abdominal pain, diarrhoea and, occasionally, gastrointestinal haemorrhage are early effects. Hypotension, which is common, is due predominantly to intravascular volume loss, although vasodilation and direct myocardial toxicity may also contribute. Coma, hypertonia, hyperreflexia, ataxia, nystagmus, miosis, hallucinations, convulsions, fasciculation and paralysis may then ensue. Hypoventilation is commonly secondary to CNS depression, but respiratory muscle weakness is a factor in the development of respiratory failure in some patients. Myopathic symptoms including limb muscle weakness, loss of tendon reflexes, myotonia and increased creatine kinase activity have been observed. Metabolic acidosis, rhabdomyolysis, renal failure, increased aminotransferase activities, pyrexia and hyperventilation have been reported. Substantial dermal exposure to 2,4-dichlorophenoxy acetic acid (2,4-D) has led occasionally to systemic features including mild gastrointestinal irritation and progressive mixed sensorimotor peripheral neuropathy. Mild, transient gastrointestinal and peripheral neuromuscular symptoms have occurred after occupational inhalation exposure. In addition to supportive care, urine alkalinization with high-flow urine output will enhance herbicide elimination and should be considered in all seriously poisoned patients. Haemodialysis produces similar herbicide clearances to urine alkalinization without the need for urine pH manipulation and the administration of substantial amounts of intravenous fluid in an already compromised patient.

2,4,5-Trichlorophenoxyacetic Acid↗
PubMed2005

Organophosphate-induced delayed polyneuropathy.

Organophosphate-induced delayed polyneuropathy (OPIDP) is a rare toxicity resulting from exposure to certain organophosphorus (OP) esters. It is characterised by distal degeneration of some axons of both the peripheral and central nervous systems occurring 1-4 weeks after single or short-term exposures. Cramping muscle pain in the lower limbs, distal numbness and paraesthesiae occur, followed by progressive weakness, depression of deep tendon reflexes in the lower limbs and, in severe cases, in the upper limbs. Signs include high-stepping gait associated with bilateral foot drop and, in severe cases, quadriplegia with foot and wrist drop as well as pyramidal signs. In time, there might be significant recovery of the peripheral nerve function but, depending on the degree of pyramidal involvement, spastic ataxia may be a permanent outcome of severe OPIDP. Human and experimental data indicate that recovery is usually complete in the young. At onset, the electrophysiological changes include reduced amplitude of the compound muscle potential, increased distal latencies and normal or slightly reduced nerve conduction velocities. The progression of the disease, usually over a few days, may lead to non-excitability of the nerve with electromyographical signs of denervation. Nerve biopsies have been performed in a few cases and showed axonal degeneration with secondary demyelination. Neuropathy target esterase (NTE) is thought to be the target of OPIDP initiation. The ratio of inhibitory powers for acetylcholinesterase and NTE represents the crucial guideline for the aetiological attribution of OP-induced peripheral neuropathy. In fact, pre-marketing toxicity testing in animals selects OP insecticides with cholinergic toxicity potential much higher than that to result in OPIDP. Therefore, OPIDP may develop only after very large exposures to insecticides, causing severe cholinergic toxicity. However, this was not the case with certain triaryl phosphates that were not used as insecticides but as hydraulic fluids, lubricants and plasticisers and do not result in cholinergic toxicity. Several thousand cases of OPIDP as a result of exposure to tri-ortho-cresyl phosphate have been reported, whereas the number of cases of OPIDP as a result of OP insecticide poisoning is much lower. In this article, we mainly discuss OP pesticide poisoning, particularly when caused by chlorpyrifos, dichlorvos, isofenphos, methamidophos, mipafox, trichlorfon, trichlornat, phosphamidon/mevinphos and by certain carbamates. We also discuss case reports where neuropathies were not convincingly attributed to fenthion, malathion, omethoate/dimethoate, parathion and merphos. Finally, several observational studies on long-term, low-level exposures to OPs that sometimes reported mild, inconsistent and unexplained changes of unclear significance in peripheral nerves are briefly discussed.

Animals↗
PubMed2000

Sensory ataxic dominant neuropathy associated with polyarteritis nodosa.

A 68-year-old man with sensory ataxic dominant neuropathy associated with polyarteritis nodosa (PAN) had deep sensory disturbance with unsteady gait and absence of the Achilles tendon reflex. Examination revealed weight loss, elevated CRP level, negative antineutrophil cytoplasm antibodies, decreased M-wave amplitude in the peroneal motor nerve and absence of action potentials in the sural sensory nerve. Sural nerve biopsy revealed a marked loss of myelinated fibers, myelin ovoid formation and necrotizing angiitis of large epineurial arterioles. Renal biopsy revealed global and/or segmental necrotizing angiitis in glomeruli, but not in the arcuate artery. These pathological findings were distinct from those of PAN, particularly microscopic polyangiitis (MPA). Treatment with a steroid improved the deep sensory disturbance, unsteady gait, and CRP level. This case is similar to ataxic neuropathy which can arise from various disorders. This is the first report of a case of sensory ataxic dominant neuropathy associated with MPA in PAN.

Action Potentials↗
PubMed2003

Motor neuron involvement in a patient with long-term corticosteroid administration.

An asthmatic patient with corticosteroid treatment for 45 years presented with slowly progressive limb muscle atrophy. His muscle symptoms were involved in four limbs and tongue, and deep tendon reflexes were exaggerated. Biopsied muscle pathology indicated the presence of neurogenic muscular atrophy in combination with corticosteroid myopathy. Furthermore, 8-hydroxy-deoxyguanosine (8-OH-dG) was prominently increased in mitochondrial and nuclear DNA. An aerobic exercise test demonstrated remarkable serum lactate elevation, which was attenuated by the administration of coenzyme Q10. These findings are consistent with the assumption that long-term corticosteroid administration potentially induces not only myopathy but also motor neuron involvement as in mitochondrial diseases.

Aged↗
PubMed2005

Exacerbation of chronic inflammatory demyelinating polyradiculoneuropathy during interferonbeta-1b therapy in a patient with childhood-onset multiple sclerosis.

Interferonbeta-1b (IFNbeta-1b) is commonly used for relapsing-remitting multiple sclerosis (MS). We report a 23-year-old woman with childhood onset relapsing-remitting MS treated with IFNbeta-1b who developed overt chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) immediately after therapy. A baseline conduction study before IFNbeta-1b therapy revealed decreased motor conduction velocities and prolonged F wave latencies in several nerves, but there was no neurological sign indicating neuropathy. The existence of subclinical demyelinating neuropathy before IFNbeta-1b treatment was suggested, although the clinical criteria for CIDP were unfulfilled. Following two months of IFNbeta-1b therapy, numbness of her right upper and lower limbs progressively worsened and all tendon reflexes were depressed. Electrophysiologically, F waves were not evoked in any limbs except for the left ulnar and tibial nerves, which showed marked prolongation of F wave latencies. Moreover, subclinical hyperthyroidism developed in association with high titers of anti-thyroglobulin and antithyroid peroxydase antibodies, which were negative before IFNbeta-1b therapy. These findings indicated that peripheral demyelination worsened at the nerve roots after IFNbeta-1b therapy. In addition to the development of autoimmune thyroid disease, the patient now fulfilled the criteria for probable CIDP. Along with the results of a previous report demonstrating IFNbeta-induced CIDP development in patients with childhood MS, this case underscores IFNbeta as a potential risk factor for CIDP in patients with childhood onset MS.

Adult↗
PubMed2006

Acute disseminated encephalomyelitis associated with oral polio vaccine.

A 27-year-old woman presented with acute paresis after taking an oral polio vaccine (OPV). Deep tendon reflexes were preserved, needle electromyography showed no neurogenic changes, and there were no lesions on spine magnetic resonance imaging (MRI), suggesting that motor neurons of the spinal cord were not affected. Brain MRI showed abnormal lesions in the tegmentum of the upper pons, left cerebral peduncles, truncus of the corpus callosum, and right parietal lobe. Cerebrospinal fluid revealed mild pleocytosis. The most probable diagnosis was acute disseminated encephalomyelitis associated with OPV.

Acute Disease↗
PubMed1996

Cervical ventral epithelial cyst treated by anterior corpectomy--case report.

A 15-year-old boy presented with a cervical ventral epithelial cyst manifesting as intractable cervical pain. Neurological examination revealed weakness of his left grasp and increased deep tendon reflexes. The anterior approach was selected because the lesion was located directly ventral to the spinal cord. He underwent C4-5 anterior corpectomy and subtotal resection of the cyst wall. He made a good recovery without additional deficits. Most previously reported cases have been treated using the posterior approach despite the ventral location of the lesion. The anterior approach offers a safer and more effective means of treating such lesions because it allows direct access to the lesion without manipulation of the spinal cord.

Adolescent↗