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Acute cauda equina syndrome secondary to lumbar disc herniation mimicking pure conus medullaris syndrome--case report.

A 49-year-old male presented with a rare case of acute cauda equina syndrome secondary to a sequestrated lumbar disc mimicking pure conus medullaris syndrome. He consulted the emergency room of Otsu Municipal Hospital because saddle anesthesia and complete urinary retention which had started 2 days before admission. Additionally, he complained of constipation and impotence. Abnormality of the deep tendon reflexes, sciatica, and leg weakness were not organized. Emergent myelography and subsequent computed tomography revealed a sequestrated disc in the sacrum compressing the lower cauda equina, but not the conus medullaris. Emergent surgical decompression was performed about 60 hours after the onset. The patient almost fully recovered from sphincter dysfunction, impotence, and saddle anesthesia 3 months after the operation. When a syndrome like conus medullaris compression is encountered, the lower cauda equina should be examined as well as the conus level. Such cases require urgent diagnosis and treatment.

Cauda Equina↗

[Successful treatment of intravenous cyclophosphamide pulse therapy for lupus myelitis with urinary disturbance and acute confusional state].

A 18-year-old female had low grade fever, butterfly rush, proteinuria, leukocytopenia and hypocomplimentemia in 1988, and she was diagnosed as systemic lupus erythematosus (SLE) with lupus nephritis (WHOIIb). Treatments with prednisolone and mizoribine resulted in the remission for three years. In May 2001, she presented neurosis and polakisuria despite of the increase of prednisolone to 20 mg/day. Finally, she admitted in our hospital because of manic and repressive state and disorientaion. A brain MRI revealed high intensity lesions in bilateral basal ganglia in T2 weighted images, and cerebrospinal fluid showed elevated protein and IFN-alpha (421 IU/ml). In addition, she manifested neurogenic bladder, muscle weakness and hyperactive deep tendon reflex of bilateral lower limbs due to both supranuclear disorder and hypesthesia under the Th 10 level. Spinal MRI revealed marked atrophy and high intensity signals at the middle to lower thoracic spinal cord in T2 weighted images, indicating complication of lupus myelitis as well as cerebral involvement. Although the symptoms of CNS lupus did not respond to prednisolone, twelve monthly cyclophosphamide pulse therapy (IV-CY) has resolved urinary disturbance, muscle weakness and sensory loss, along with the improvement of both cerebral and spinal MRI images. Lupus myelitis and neurogenic bladder are the rare, but very refractory manifestation among CNS involvement of SLE. We here propose IV-CY as an invaluable choice for the treatment of not only active lupus myelitis but also neurogenic bladder resisted for steroid.

Adolescent↗

Spastic paraplegia with amyotrophy of the legs: a rare case of motor and sensory neuropathy.

A 36-year-old man who suffers from gait disturbance is reported. He noticed deformity of his feet at the age of 15. When he was 32 years old, he complained of heaviness in his lower extremities. Since then his legs have been always stiff. He had no previous illness or familial neuromuscular diseases. Neurological examination revealed no impairment of mental function or cranial nerves. Marked weakness and wasting of the feet were noted. The legs showed an inverted champagne bottle shape and pes cavus deformity was evident. Deep tendon reflexes were normal in the arms but abnormally brisk in the legs. Bilateral Babinski sign and ankle clonus were elicited. The patient tended to walk on his toes and the legs scissored. The motor nerve velocities were less than normal. Sural nerve biopsy showed reduced myelinated fiber density and increased endoneurial connective tissue. Electron microscopy showed axonal swellings filled with neurofilaments. Distal wasting and weakness involving the legs more than the arms resembled that of Charcot-Marie-Tooth disease. According to the classification by Dyck, this disorder could be referred to as hereditary motor and sensory neuropathy type V. Spastic paraplegia with amyotrophy is rare, but should be identified as a distinct disorder. Recognition of this disorder would imply the clinical and genetic heterogeneity of Charcot-Marie-Tooth disease.

Adult↗

Epidemiological correlates of diabetic neuropathy. Report from Pittsburgh Epidemiology of Diabetes Complications Study.

The natural history of diabetic neuropathy and its risk factors are not well understood, apart from the recognition that prevalence increases with duration and, in many studies, degree of glycemia. The role of potential risk factors was therefore evaluated in a cross-sectional analysis from the baseline examination of the Pittsburgh Epidemiology of Diabetes Complications Study. We present results from the first 400 subjects seen at baseline examination. Neuropathy was determined by a trained internist with a standardized examination and was defined as the presence of at least two of three criteria: abnormal sensory or motor signs, symptoms consistent with neuropathy, and decreased tendon reflexes. The prevalence of neuropathy in this cohort was 34% (18%, 18-29 yr old, 58% greater than or equal to 30 yr old) with no difference by sex. By focusing on subjects greater than or equal to 18 yr old, all significant univariate variables (e.g., duration, glycosylated hemoglobin [HbA1]) were analyzed in 3 multiple logistic regression models: all subjects greater than or equal to 18 yr old and separating the same subjects into two groups based on age (18-29 and greater than or equal to 30 yr). Duration, HbA1, smoking status, and high-density lipoprotein cholesterol were found to be associated with neuropathy in the models for the greater than or equal to 18-yr-old group and the greater than or equal to 30-yr-old group. In the 18- to 29-yr-old group, duration, HbA1, and hypertension status were found to be significantly associated with neuropathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Proliferative retinopathy in NIDDM. Incidence and risk factors in Pima Indians.

The incidence of proliferative diabetic retinopathy was determined in the Pima Indians of the Gila River Indian Community in Arizona. Over 4 yr, this complication developed in 25 of 953 subjects greater than or equal to 9 yr of age with non-insulin-dependent diabetes. No cases were diagnosed in less than 35-yr-old subjects, and the incidence was strongly related to the duration of diabetes. The cumulative incidence of proliferative retinopathy after 20 yr duration was 14%. All cases of proliferative retinopathy occurred in subjects with background retinopathy. Younger age at diagnosis of diabetes was associated with a higher incidence of proliferation when subjects with diabetes of similar duration were compared. A higher incidence of proliferative retinopathy, after controlling for age, sex, and diabetes duration, was associated with hypertension, proteinuria, renal insufficiency, absence of Achilles tendon reflex, elevated total serum cholesterol concentration, and insulin therapy.

Adolescent↗

Measuring vibration sensations with graduated tuning fork. Simple and reliable means to detect diabetic patients at risk of neuropathic foot ulceration.

To find a simple and reliable means to measure vibration sensations, 189 diabetic patients and 88 control subjects were tested at different sites with a graduated tuning fork. Within-test variation at big toes reached 8.4% in diabetic patients vs. 2.2% in control subjects. Mean contralateral variation was 7.5% in diabetic patients vs. 2.5% in control subjects. Tuning-fork sensations were inversely correlated with duration of diabetes, whereas no correlation was found with HBA1c levels or the severity of retinopathy. Ninety-nine (52%) patients had vibratory sensation at big toes of less than 99th percentile of normal values for age. In addition, 51% of the patients with clinical symptoms at extremities (n = 67), 70% of the patients without tendon reflexes (n = 50), and 75% of the patients with abnormal nerve conduction velocities (n = 60) also had low vibration sensations. All patients with lower-limb injuries (n = 7) had values at big toes of less than 2. Altogether, the graduated tuning fork represents a simple and reliable alternative to quantitate vibration sensations. Long-term follow-up of asymptomatic patients will indicate whether these abnormalities reflect underlying neuropathy. Patients with abnormal values at screening will necessitate additional investigations and special foot-care education programs.

Adolescent↗

Treatment of diabetic neuropathy with gamma-linolenic acid. The gamma-Linolenic Acid Multicenter Trial Group.

OBJECTIVE: To compare the effects of placebo and GLA on the course of mild diabetic neuropathy over 1 yr. RESEARCH DESIGN AND METHODS: We entered 111 patients with mild diabetic neuropathy from seven centers into a randomized, double-blind, placebo-controlled parallel study of GLA at a dose of 480 mg/day. MNCV, SNAP, CMAP, hot and cold thresholds, sensation, tendon reflexes, and muscle strength were assessed by standard tests in upper and lower limbs. RESULTS: For all 16 parameters, the change over 1 yr in response to GLA was more favorable than the change with placebo, and for 13 parameters, the difference was statistically significant. Sex, age, and type of diabetes did not influence the result, but treatment was more effective in relatively well-controlled than in poorly-controlled diabetic patients. CONCLUSIONS: GLA had a beneficial effect on the course of diabetic neuropathy.

Analysis of Variance↗

Neurologic sequela of hydrogen sulfide poisoning.

A 25-year-old man, a field operator in a petroleum refinery was found unconscious. He was exposed to hydrogen sulfide and presented with Glasgow Coma Score of 5, severe hypoxemia on arterial blood gas analysis, normal chest radiography, and normal blood pressure. On hospital day 7, his mental state became clear, and neurologic examination showed quadriparesis, profound spasticity, increased tendon reflexes, abnormal Babinski response, and bradykinesia. He was also found to have decreased memory, attention deficits and blunted affect, which suggested general cognitive dysfunction, but which improved soon. MRI scan showed abnormal signals in both basal ganglia and motor cortex, compatible with clinical findings of motor dysfunction.

Adult↗

Clinical and electrophysiological studies in a patient with keratitis, ichthyosis and deafness (KID) syndrome.

Neurological studies were performed in a young boy with keratitis, ichthyosis and deafness syndrome. Skin biopsy showed features of ichthyosis. Clinically and electrophysiologically, he had normal motor and sensory systems, but there was an acoustic nerve lesion and absence of tendon reflexes. Audiometry and brainstem auditory evoked potentials showed bilateral neurosensory deafness. Poor vision may not be due to an optic nerve lesion as evidenced on visual evoked potential findings, but is probably due to pronounced vascularizing keratitis of the cornea. Computer tomography scan showed mild hypoplasia of the inferior vermis and left side of the cerebellum.

Blinking↗

Phenol block of the tibial nerve for spasticity: a long-term follow-up study.

Ninety-two tibial nerve blocks with phenol were performed in 59 patients for treatment of severe spasticity of the foot. The Achilles tendon reflex was abolished, ankle clonus was eliminated and resistance to passive stretch was reduced substantially following the procedure in all patients. Significant functional gains were observed as a result of decrease in spasticity with long-term follow-up averaging 28.7 months (range 14-60). The simplicity of the procedure, the functional results observed with long-lasting effects, and the lack of serious complications, would suggest the more widespread use of this procedure in the treatment of the spastic foot.

Adolescent↗

Severe peripheral neuropathy after exposure to monosodium methyl arsonate.

A case of severe peripheral neuropathy after several days of exposure to a pesticide spray containing monosodium methyl arsonate (MSMA) is reported. The clinical manifestations of symmetrical peripheral neuropathy with stocking-glove sensory deficit, decreased position sense, decreased and absent deep tendon reflexes, and muscle wasting are consistent with those described in other cases of arsenic intoxication. The anemia, leukopenia, and bone marrow changes of dyserythropoiesis in this case are also similar to those previously described with arsenic intoxication. The authors discuss the possible contribution of toxicity from exposure to other pesticides.

Adult↗

Isopropanol ingestion: interpretation of blood concentrations and clinical findings.

We evaluated blood concentrations and clinical findings in 17 cases of isopropanol (IPROH) ingestion seen over a 8.5 year period at our institution. Eight ingestions involved IPROH alone ("pure") while the remainder involved at least ethanol in addition to IPROH ("mixed"). Fourteen patients had a history of alcoholism. Admission blood IPROH concentrations ranged from 5 to 70 mg/dL while the concentration of the acetone metabolite ranged from nondetectable to 220 mg/dL. The mean acetone concentration was significantly higher for "pure" ingestions than for "mixed" ingestions (p less than 0.05); however, the mean IPROH concentrations showed no significant difference. The mean anion gap was significantly higher for "mixed" ingestions than for "pure" ones (p less than 0.01). Fifteen patients were either alert or lethargic while two, who had ingested no compounds other than IPROH, were comatose. The most common other physical findings were tachycardia (10 cases), decreased deep tendon reflexes (5), dysarthria (4), and ataxia, hypotension, fever, and mydriasis (3 cases each). None of the findings including level of consciousness showed statistically significant correlation with the IPROH concentrations. Twelve patients were hospitalized; eleven of these recovered with supportive care alone. One patient expired from trauma suffered in a motor vehicle accident.

1-Propanol↗

Neurotoxicological evaluation of hospital sterilizer workers exposed to ethylene oxide.

Ethylene oxide is now frequently used to chemically sterilize heat-sensitive materials in the hospital setting. Previous reports of neurotoxic effects of ethylene oxide have been described in animals and humans. Recent reports suggest that cognitive deficits may be associated with chronic low-level ethylene oxide exposure. We undertook this study of hospital workers with chronic ethylene oxide exposure and compared them with a non-exposed control group in an attempt to detect neurological and neuropsychological abnormalities. Ethylene oxide breathing zone levels of up to 250 ppm in exposed subjects were reported. All evaluations were done without examiners' knowledge of exposure status of the subjects. The exposed group was found to have a statistically significant lower P300 amplitude, bilaterally hypoactive distal deep tendon reflexes and poorer performance on neuropsychological tests involving psychomotor speed. Exposed subjects acknowledged more symptoms and higher levels of depression and anxiety. Nerve conduction velocities and EEG spectral analysis were similar in both exposed and control groups as were scores on most psychological tests. Based upon this information and prior reports, ethylene oxide should be considered in a differential diagnosis of neuropsychological, peripheral and central nervous system dysfunction in workplace settings associated with ethylene oxide exposure.

Adult↗

Prolonged toxicity with intermediate syndrome after combined parathion and methyl parathion poisoning.

A prolonged type of organophosphate toxicity, previously characterized as the Intermediate Syndrome, has been recognized in 6 out of 7 prospectively studied patients poisoned by insecticide containing parathion and methyl parathion in equal proportions. The clinical characteristics included respiratory paresis, weakness in the territories of several motor cranial nerves, neck flexors and proximal limb muscles, and depressed tendon reflexes, all lasting for several days or weeks. Electromyography in the early stages disclosed diverse types of impaired neuromuscular transmission. EMG normalization preceded clinical recovery. Severe plasma butyrylcholinesterase and erythrocyte acetylcholinesterase inhibition persisted along with the occurrence of Intermediate Syndrome-related symptoms. We conclude that combined parathion and methyl parathion poisoning is more likely to induce Intermediate Syndrome than parathion poisoning alone. The mechanisms underlying this difference remain obscure. The Intermediate Syndrome shows clinical and electromyographic hallmarks of combined postsynaptic impairment of neuromuscular transmission.

Adult↗

Severe rhabdomyolysis mimicking transverse myelitis in a heroin addict.

Heroin addiction is known to cause various medical and neurological complications. We report here a case of rhabdomyolysis following heroin abuse, in which a neurological lesion mimicking transverse myelitis was also noted. A 29-year-old man was found comatose in a kneeling position one day after a heroin overdose. On admission, he was awake, yet with total paralysis of his lower legs. Physical examination revealed marked swelling and tenderness of the four limbs, especially the lower extremities. Deep tendon reflexes and positional sense were absent in both legs; however, pin-prick sense was preserved. Transverse myelitis or spinal cord vasculitis was the initial working diagnosis. Laboratory tests disclosed significantly elevated creatinine kinase of 146289 U/L. Though suffering transient acute renal failure, his neurological abnormalities gradually improved over four weeks and a left foot drop was the only residual lesion at discharge. Rhabdomyolysis, a well defined complication following heroin use, may also cause concomitant neurological symptoms, for which careful differential diagnosis is warranted. With the increasing number of heroin addicts in Taiwan, more cases with rhabdomyolysis-induced neurological symptoms may be observed in the future.

Adult↗

Hypermagnesemia following an acute ingestion of Epsom salt in a patient with normal renal function.

CASE REPORT: Hypermagnesemia is a rare condition that commonly follows excessive therapeutic administration of magnesium sulfate to treat eclampsia of pregnancy. Signs and symptoms of this condition include extreme muscle weakness, loss of deep tendon reflexes, mental status depression, and cardiac dysrhythmias. Clinically significant hypermagnesemia following oral or rectal administration of magnesium containing products in patients with normal renal function is rare. We report a case of hypermagnesemia following massive Epsom salt ingestion that resulted in extreme musculoskeletal weakness and altered mentation.

Central Nervous System Diseases↗

Malnutrition-induced hypokalemic myopathy in chronic alcoholism.

CASE REPORT: A 42-year-old man with a history of Billroth II-gastrectomy, chronic alcoholism, and malnutrition developed acute tetraparesis, two days before admission. He presented with bilateral, proximal upper and lower limb weakness, limb girdle wasting, bilaterally reduced Achilles tendon reflexes, and bilateral stocking-type sensory disturbances. Laboratory data revealed hypokalemia (2.2 mmol/L), elevated creatine kinase (7282 U/L), metabolic alkalosis and reduced urine potassium, albumin, and total protein. Muscle biopsy showed atrophic, necrotic, and regenerating fibers, endomysial macrophages, and vacuolar degeneration, interpreted as hypokalemic myopathy. With the correction of the serum potassium, tetraparesis rapidly resolved. With other causes excluded, malnutrition and gastrectomy were considered responsible for hypokalemia in this patient with acute tetraparesis and chronic alcoholism.

Acute Disease↗

Effect of British-made videotapes on clinical performance of medical students in Pakistan.

The efficacy of videotapes, recorded in Cardiff, in improving the clinical performance of final-year medical students in Abbottabad (Pakistan) was tested, by carrying out a structured, stepwise, clinical assessment before and after video teaching in 32 students. All students examined eight systems/subsystems at eight stations and spent 5 min with each patient, during which their performance was checked against structured check-lists by eight examiners. These students had not received any clinical instruction from a specialist rheumatologist, neurologist or endocrinologist during their clinical apprenticeship. Before the video teaching they performed poorly when examining the knee joint, motor system, hands and thyroid status, but when tested again 2 days after video teaching, there was a transformation in their clinical behaviour and their mean (s.d.) score improved from 40 (6.6 per cent) to 57.6 (9.4 per cent; P = 0.001). In contrast to their pre-video performance, they interacted well with the patients and examiners providing a running commentary of their findings, as demonstrated in the videotapes. Among the residual problems were a poor technique of testing tendon reflexes and percussion. All the examiners and 21 of 32 students thought that the structured examination was fairer than the conventional examination. Of the 32 students, 20 thought that video teaching was less effective than personal bedside teaching, while 12 students thought that video was structured better than bedside instruction. All students would welcome video teaching to supplement their existing teaching and would like the structured examination introduced to their curriculum. This study suggests that videotaped demonstrations can be used effectively in transmitting clinical skills to students not exposed to clinical teaching by specialists in various subjects.

Clinical Competence↗