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At least 649 records · Page 36Linked to original sources
PubMed1981

Atlanto-axial instability in children with Down syndrome.

One hundred fifty-eight patients with Down syndrome underwent roentgenologic examinations of the cervical spine in neutral position, in hyperflexion, and in hyperextension. Fifteen children with Down syndrome were found to have a distance between the joint surfaces of 5 mm and more. The majority of these children were asymptomatic, one child displayed hyperactive deep tendon reflexes and two children were symptomatic and underwent surgery.

Adolescent↗
PubMed1984

Gastric secretion and emptying in hypothyroidism.

Hypothyroidism is commonly thought to cause decreased gastric emptying and secretion, but these may be related to associated autoimmune disease or chronic changes. Therefore, we measured gastric emptying and secretion in 11 healthy controls and in nine patients (19-54 years old; five females and 4 males) rendered athyreotic by surgery and/or 131I for thyroid cancer. Replacement T4 was stopped 47-65 days and subsequent replacement T3 was stopped 33-40 days before the study. All patients were symptomatic with complaints including weight gain, lethargy, and constipation. Deep tendon reflexes had delayed relaxation phase. Serum cholesterol and creatine phosphokinase levels were elevated. Thyroid hormone levels were markedly decreased (means +/- SE; T4: 0.7 +/- 0.3 micrograms/dl; free T4: 0.2 +/- 0.1 ng/dl; T3: 28 +/- 6 ng/dl) and TSH was markedly increased (88 +/- 16 microU/ml). Gastric fractional emptying rate (%/min) and hydrogen ion (H+) output (meq/hr) were determined before and following two sequential stimulations: a 250-ml water load and an intravenous infusion of pentagastrin (6 micrograms/kg/hr). There were no significant differences between controls and athyreotic patients. Our data demonstrated that short-term, profound, thyroid hormone deficiency does not modify gastric emptying or acid output.

Adult↗
PubMed1991

Maternal vegan diet causing a serious infantile neurological disorder due to vitamin B12 deficiency.

We present a 9-month-old exclusively breast-fed baby of a strict vegetarian mother who had excluded all animal proteins from her diet. The patient's symptoms included dystrophy, weakness, muscular atrophy, loss of tendon reflexes, psychomotor regression and haematological abnormalities. Biochemical investigations revealed severe methylmalonic aciduria and homocystinuria in the patient, slight methylmalonic aciduria in the mother and low concentrations of serum vitamin B12 in both patient and mother.

Adult↗
PubMed1983

Toxic polyneuropathy of shoe workers in Italy. A clinical, neurophysiological and follow-up study.

Cases of polyneuropathy due to exposure to industrial solvents have been studied at several shoe factories in the province of Siena. After the screening of 654 employees 98 verified cases were detected. Of these, 16 were rated as moderate to severe, 45 as mild, and 37 were minimally involved but with characteristic electrodiagnostic abnormalities. Follow-up study in 53 patients showed that neurological signs and symptoms as well as electrodiagnostic abnormalities continued for years in several patients. In addition, after a year's observation, some patients showed signs of central nervous system dysfunction such as spasticity of the lower limbs and increased deep tendon reflexes. High percentages of commercial n-hexane were found in all the samples of glues and solvents collected from home-workers and from factories where cases of polyneuropathy occurred.

Adhesives↗
PubMed1992

Neurological examination in patients recovering from general anesthesia.

We performed serial neurological evaluations on 86 patients who underwent surgery for herniated lumbar disk during the first 3 hours after the end of anesthesia (isoflurane-N20-02 for 25 patients, halothane-N20-02 for 38, fentanyl-droperidol-N20-02 for 23). At time 0, the idiomuscular response to percussion of the extensor carpi muscle was present in every patient, while tendon reflexes were always absent. Hyperreflexia was as frequent as shivering, but it took place earlier; we could determine no correlation between these two phenomena. There was no correlation between shivering and rectal temperature of the patients. Although almost all the patients were cooperative and could correctly calculate 100-7, the post-hyperventilation-apnea test was positive in 35 patients at time 120 minutes: this suggests that many patients considered "awake" still exhibit neurological abnormalities, such as inadequate respiratory drive.

Adolescent↗
PubMed1997

Orthostatic hypotension in a case with multiple sclerosis.

A 37-year-old woman with a 5-year history of multiple sclerosis is reported. She began having recurrent syncope even in the sitting position; other neurological features included hiccup, faciooro-lingual flushing and clumsiness of the hands. She had alternating Horner's syndrome, mild hypoalgesia of the right face, exaggerated deep tendon reflexes of the upper extremities, decreased deep sensation and ataxia of the upper extremities, and incomplete transverse myelopathy with a T4 sensory level. Head-up tilt testing confirmed orthostatic hypotension with relative preservation of the heart rate increase. Magnetic resonance imaging indicated abnormal intensities in the paramedian tegmentum and base of the medulla, which may have been additionally responsible for orthostatic hypotension. Steroid pulse therapy and L-threo-3,4-dihydroxyphenylserine caused regression of brainstem signs and reduced syncopal attacks.

Adult↗
PubMed2005

Benign acute childhood myositis.

OBJECTIVE: To describe the clinical and laboratory features of benign acute childhood myositis. METHODS: 40 children of BACM were seen during October 2001 to February 2002, 22 (52%) were male with mean age of 5.3 years. Duration of illness was 3.97 days. Preceding symptoms included fever, leg pain, vomiting and inability to walk. A provisional diagnosis of viral myositis was made in 26 (66%). Guillian Barre Syndrome was the most common referral diagnosis. RESULTS: 11 (27.5%) children had leucopenia with lymphocytic response and 16 (40%) had thrombocytopenia. CRP was negative in 32 (80%). CPK was markedly elevated (more than 1000 IU/l) in 18 (45%) and more than 500 IU/l in 11 (27.5%) remaining between 200 to 500 IU/l. Associated features were hepatitis (elevated SGOT & SGPT) in 28 (70%) and shock in 5 (12.5%). Serological test were indicative of dengue virus (Elisa PAN BIO) in 20 (50%) of which 8 (25%) were primary dengue and 12 (30%) were secondary dengue. The outcome of therapy mainly supportive were excellent. CONCLUSION: Benign acute myositis occurs often in association with viral infection. In the present study, Dengue virus was positive in 20 (50%) children. Benign acute myositis can be differentiated from more serious causes of walking difficulty by presence of calf and thigh muscle tenderness on stretching, normal power and deep tendon reflex and elevated CPK.

Acute Disease↗
PubMedDate not supplied

Hypomelanosis of Ito.

A two-year-old male child presented with bizarre hypopigmented skin lesions, severe mental retardation and generalized tonic-clonic seizures. Examination showed hypopigmented patterned whorls and irregular patches over the trunk and linear streaks over the flexor aspects of upper and lower limbs. He also had generalized hypertonia and brisk tendon reflexes. Other systems were normal.

Child, Preschool↗
PubMed1990

Neurological phenomena during emergence from enflurane or isoflurane anaesthesia.

During emergence from anaesthesia, transient neurological signs that would usually be considered pathological may appear. The objective of this randomized, patient (n = 30) and observer-blinded study was to compare prospectively the incidence and duration of post-anaesthetic neurological abnormalities in healthy patients undergoing minor elective procedures following thiopentone and succinylcholine induction, and enflurane-N2O or isoflurane-N2O anaesthesia. Patients were studied for 60 min after anaesthesia. Arousal state, muscle tone, deep tendon reflexes, plantar reflex, sustained clonus, shivering, intense muscular spasticity and temperature were assessed. Results of neurological examination were correlated with the patient's state of arousal. Transient emergent neurological abnormalities occurred more frequently following enflurane-N2O anaesthesia than isoflurane N2O anaesthesia. This was statistically significant (P less than 0.05) for quadriceps hyperreflexia, upgoing toes (positive Babinski reflex) and intense muscular spasticity. Neurological abnormalities occurred most commonly 5-20 min after anaesthesia and all abnormalities resolved within 60 min. Following enflurane anaesthesia, as patients became more alert the incidence of abnormalities declined, while the arousal state did not affect the incidence of abnormalities after isoflurane. There was no significant difference between axillary temperatures of those patients who shivered and those who did not. In conclusion, temporary emergent neurological abnormalities occurred more often following enflurane-N2O than after isoflurane-N2O anaesthesia.

Adult↗
PubMed1998

Lumbosacral plexopathy from iliopsoas haematoma after combined general-epidural anaesthesia for abdominal aneurysmectomy.

PURPOSE: To report a case of iliopsoas haematoma after resection of an abdominal aortic aneurysm which resulted in a lumbosacral plexopathy. CLINICAL FEATURES: An 81-yr-old man presented with an abdominal aortic aneurysm for aneurysmectomy and tube grafting. An epidural catheter was placed at the L1-2 spinal level and combined epidural-general anaesthesia was provided for surgery. The surgery was complex and a suprarenal clamp was necessary to obtain proximal control. A continuous infusion of demerol through the epidural catheter was prescribed for postoperative analgesia. On the first postoperative day, examination revealed a paretic, pulseless right leg and he was returned to the operating room for femoral-femoral bypass. By the following day, the motor and sensory impairment had progressed to complete paralysis with loss of all deep tendon reflexes and absent sensation below L1, despite palpable pulses in the leg. A CT of the abdomen demonstrated a right iliopsoas haematoma. There was no evidence of either disc herniation or an epidural haematoma. A diagnosis of lumbosacral plexopathy secondary to a iliopsoas haematoma was made. CONCLUSION: Iliopsoas haematoma is a rare cause of postoperative neurological deficit following aortic vascular surgery. The haematoma results in compression of the lumbosacral neural elements and typically presents as a femoral neuropathy. The diagnosis is clinical and can be readily validated with computed tomography.

Aged↗
PubMed1999

[Hypokalemic paralysis in furosemide therapy and simultaneous laxative abuse].

BACKGROUND: Clinical signs of hypokalemia are not directly related to the extent of the electrolyte imbalance, and therefore monosymptomatic cases may be observed. CASE REPORT: Following an acute gastroenteritis with considerable diarrhea, a 47-year-old male patient was admitted to hospital for progressive painful paraparesis. Upon admission, the patient complained of painful paresthesias in both legs, and a moderate flaccid paraparesis with widespread fasciculations and loss of leg tendon reflexes was found. Serum potassium level on admission was 1.7 mmol/l. Other signs of hypokalemia were absent, and the ECGs showed a slow sinus rhythm without disturbances of de- or repolarisation or cardiac arrhythmias. Hypokalemic paralysis was diagnosed and was considered to be primarily drug-induced, as the patient had a history of laxative abuse and was on a continuous medication with furosemide (80 mg/d) without regular assessment of serum electrolytes. The additional electrolyte loss following the gastroenteritis precipitated the development of clinical signs of hypokalemia. In parallel to the rise in serum potassium levels, both painful paresthesias and muscle weakness disappeared, and electromyography documented the amelioration of the myopathic syndrome. CONCLUSION: The prominent clinical symptom of hypokalemia was a dyskalemic paralysis in the absence of other sequelae of electrolyte imbalance, such as cardiac arrhythmias or vegetative disturbances.

Cathartics↗
PubMedDate not supplied

Vincristine neurotoxicity. Pathophysiology and management.

Vincristine is an antineoplastic drug with a broad spectrum of activity against haematological malignancies and childhood sarcomas. Besides useful activity, it lacks the usual emetic and myelotoxicity of anticancer drugs, but its use is limited by neurotoxicity. The neurotoxicity commonly manifests as reduced motility of the intestines resulting in constipation, and peripheral neuropathy which is predominantly sensory in nature. The early symptoms include numbness and tingling of hands and feet which is accompanied by loss of deep tendon reflexes. In its more severe form, muscle weakness develops which is more marked in distal muscles of the hands and feet. Other manifestations of neurotoxicity include ocular palsies, hoarseness of voice, and autonomic neuropathy in the form of postural hypotension and atony of the urinary bladder. The neurotoxicity is dose related and cumulative with repeated dosage such that the drug therapy has to be stopped after a cumulative dose of 30 to 50 mg. The neurotoxicity is usually reversible on interruption of the therapy, but the recovery is slow and takes several months. There are no specific antidotes which have established usefulness against neurotoxicity.

Humans↗
PubMed2002

[Hypo-osmolar hyponatremia as the chief symptom in hypothyroidism].

CASE HISTORY AND DIAGNOSIS: A 75-year-old male patient presented with a history of anorexia, muscle weakness, and increasing memory loss. He had mild pedal edema and decreased deep tendon reflexes. As the laboratory tests showed hypoosmolar hyponatremia and urinary sodium within the normal range, a syndrome of inappropriate ADH secretion (SIADH) was presumed. While neither the medical history nor any of the diagnostic procedures revealed any underlying pathology explaining the SIADH, laboratory tests showed significant hypothyroidism. Hypothyroid states are associated with significant changes in renal function, one of which is hypoosmolar hyponatremia. TREATMENT AND COURSE: Treatment included fluid restriction and hormone substitution and resulted in a quick correction of the hyponatremia and a clear improvement of the patient's cognitive function. CONCLUSION: It is concluded that the diagnosis of SIADH should only be made after thorough investigation of the adrenal and thyroid hormone status.

Aged↗
PubMed2005

[Hypokalemic thyrotoxic periodic paralysis (HTPP). Rare differential diagnosis in case of acute tetraparesis in Europe].

BACKGROUND: Differential diagnosis of acute tetraparesis includes paraplegia caused by trauma, ischemia, inflammation or tumor, Guillain-Barré syndrome, periodic paralysis, myasthenia gravis, and dissociative paralysis. CASE REPORT: The case of a 45-year-old man is reported who noticed weakness of both legs after heavy labor. In the evening he was unable to climb stairs, at night he noticed additional weakness in both arms and dyspnea. After transfer to the authors' department profound tetraparesis, tachycardia and slight dyspnea were found on examination. Cranial nerve examination was unremarkable. Deep tendon reflexes, however, were only slightly diminished. Laboratory work-up revealed hypokalemia (potassium 1.9 mmol/l) and hyperthyreosis. Elevated TRAK and TPO antibodies as well as sonography and scintigraphy of the thyroid gland gave evidence of Graves' disease. After intravenous administration of potassium tetraparesis resolved completely. CONCLUSION: Hypokalemic thyrotoxic periodic paralysis (HTPP), a subtype of hypokalemic paralysis, is unusual in Caucasians. In Asian men the disease is more common. The underlying mechanism is an extra-intracellular shift of potassium in the voluntary muscle cells. In contrast to hereditary autosomal dominant paralyses, a genetic defect is not known. Usually, symptoms of hyperthyreosis in HTPP are mild which can hamper the correct diagnosis.

Acute Disease↗
PubMed2006

[Medically induced myopathia].

Muscular side effects of various anesthetics, analgetics, antibiotics, antihistaminic drugs, antiretrovirals, cardiotropics, immunosuppressants, lipid-lowering drugs, psychotropic drugs, anticancer drugs, and other substances are more frequent than assumed and are easily overlooked. Clinically, muscular side effects manifest as fatigue, myalgias, persistent or transient weakness, stiffness, intolerance to exercise, psychomotor slowing, muscle cramps, wasting, dyspnea, dysphagia, fasciculations, reduced tendon reflexes, impaired consciousness, myoglobinuria, renal failure, or hyperthermia. Diagnosis of these drug-induced myopathies is based on history, clinical neurologic examination, blood work, urine analysis, repetitive stimulation, electromyography, and muscle biopsy. A drug which induces muscular side effects should never be given again. Particularly in patients suffering from primary myopathy, myotoxic drugs should be applied with caution. The drugs which most frequently induce muscular side effects are steroids, statins, fibrates, antiretrovirals, immunosuppressants, colchicine, amiodarone, and anticancer drugs. Many drugs exhibit their myotoxic potential only in combination with other drugs or premorbid pathologic myogenic conditions.

Drug-Related Side Effects and Adverse Reactions↗
PubMed1999

[Hereditary motor-sensory neuropathies (Charcot-Marie-Tooth syndrome) and related neuropathies. Current classification and genotype-phenotype correlation].

Charcot-Marie-Tooth (CMT) disease is the most common inherited disorder of the peripheral nervous system with an incidence of 40:100,000. Clinically, it is characterized by distal muscle weakness and wasting, primarily of the legs and later of the arms, foot deformity, diminished or absent tendon reflexes, and mild-to-moderate sensory loss. Molecular genetic studies over the past 2 decades have revealed the genetic heterogeneity of this disorder and the identification of different genes or gene loci, respectively. Therefore, a current CMT classification though constantly changing due to ongoing detection of further genetic defects must take into consideration both phenotypic and genotypic criteria. Since certain clinical features appear to be associated with specific genetic subtypes, we provide a detailed description of characteristic phenotypic variants to facilitate differential diagnosis and allow more precise referral to subsequent genetic investigations.

Charcot-Marie-Tooth Disease↗
PubMed2006

Tethered cord syndrome in a 24-year-old woman presenting with urinary retention.

We discuss a case of newly diagnosed tethered cord syndrome (TCS) in a 24-year-old woman who presented with dysuria, hematuria, and urinary retention. Physical exam revealed hyperreflexic lower extremity deep tendon reflexes. MRI showed filum terminale terminating in a lipoma, consistent with TCS. We discuss the role of MRI in the workup of adults with bladder dysfunction.

Adult↗
PubMed2000

Loss of large-diameter spindle afferent fibres is not detrimental to the control of body sway during upright stance: evidence from neuropathy.

Fifteen patients with Charcot-Marie-Tooth type 1A (CMT1A) disease and 46 normal controls were studied. In the patients, leg muscle strength, touch-pressure, vibration and joint position sense were reduced; lower limb tendon reflexes were absent in 12 or markedly decreased. Motor and sensory conduction velocity (CV) of leg nerves was either reduced or not measurable. The Neurological Disability Score and the Neuropathy Score were obtained from clinical and electrophysiological examination, respectively. Tilt of a supporting platform elicited short- (SLR) and medium-latency (MLR) responses to stretch in the foot muscle flexor digitorum brevis (FDB) in controls. In the patients, the former response was absent and the latter delayed. These findings are in keeping with the known loss of large-diameter myelinated fibres, with relative sparing of the smaller fibres. The MLR delay was fully accounted for by the slowed CV of the motor fibres. The MLR afferent time was similar to that in normal subjects. Body sway area (SA) during quiet stance was recorded with eyes open or closed, and with feet apart or together. Under all postural and visual conditions, SA was within normal range in the less severely affected patients, but was moderately increased in the patients with a more severe neuropathy score. Across all patients, no correlation was found between SA and muscle force, motor CV, touch pressure, vibration and joint position sense, considered either separately or as an aggregate. We suggest that: (1) functional integrity of the largest afferent fibres is not necessary for appropriate equilibrium control during quiet stance and (2) any unsteadiness is related to additional functional alterations in smaller fibres, most likely group II spindle afferent fibres.

Adolescent↗