Síndrome de Miller Fischer: apresentaçäo de quatro casos e revisäo da posiçäo nosológica / Miller Fischer syndrome: 4 cases and the nosological position of this disorder

Neste trabalho säo descritos quatro casos tipicos, recentes, da síndrome de Miller Fisher, acrescidos de revisäo da literatura sobre sua posiçäo nosológica. Na falta de maiores esclarecimentos anatomopatológicos, näo foi possível responder com certeza a questäo da origem da Síndrome. Tanto pela literatura quanto nos casos ora descritos, parece ocorrer envolvimento combinado do sistema nervoso central e do sistema nervoso periférico

Bladder hypocompliance in the spinal cord injury population.

To determine the incidence and effects of low bladder compliance on the upper urinary tracts in spinal cord injury patients, we evaluated the last 254 patients during a 3-year period who underwent a complete urodynamic study, along with an excretory urogram and/or renal ultrasound. Compliance was determined at 100 cc volume. A compliance number of 20 or less was considered low. Of the 254 patients 43 (17 per cent) had low compliant bladders by our definition. Hydronephrosis was present in 56 of the 84 renal units (64 per cent) and reflux was present in 39 (46 per cent). These results are in sharp contrast to the normal compliant group in which 21 per cent of 418 renal units had hydronephrosis and only 6 per cent had reflux. Of the 254 patients over-all 44 had lower motor neuron lesions as determined by no bulbocavernosus reflex as well as a denervated external sphincter; 22 of these 44 patients (50 per cent) had low compliant bladders. Conversely, among the 210 patients with suprasacral lesions only 21 (10 per cent) had low compliant bladders. In conclusion, the development of a low compliant bladder in spinal cord injury patients is not an uncommon event and places the upper urinary tracts at great risk. All attempts must be directed toward maintaining low detrusor pressures, and these patients must have adequate and frequent followup evaluation.

Atlantoaxial instability and neurologic indicators in rheumatoid arthritis.

Atlantoaxial subluxation in rheumatoid arthritis is characterized by pain, disability, and occasionally death. Surgical fusion of the offending vertebrae is the appropriate surgical remedy, but it is a procedure with a high failure and complication rate. Because cord compression and myelopathy are the main complications of this condition, it would be logical to use evidence of early neurologic deficit as an indicator for surgery. A group of 250 patients with rheumatoid arthritis were investigated to evaluate the neurologic status with respect to the degree of instability in the proximal cervical spine. Neurologic isolated signs such as hyperreflexia could not be correlated with the onset of cervical myelopathy or quadraparesis or to the radiographically measured degree of atlantoaxial instability. These signs should not be used as an indication for surgery. The positive indications are intractable pain, cervical myelopathy, and severe atlantoaxial instability.

[Clinico-neurologic long-term results following surgery of intervertebral disk displacement]. / Klinisch-neurologische Langzeitergebnisse nach Operationen lumbaler Bandscheibenvorfälle.

Of the 1732 patients undergoing disk surgery in the period 1971-1980, a sample of 245 was selected, and data gathered prior to their operations compared with data gathered two to twelve years after the operation. Analyses was made of the specific character of pain, the state of the reflexes, motor and sensory failure, Lasègue's sign, finger-ground-distance Disorders of bladder and Rectum, and the patients' subjective judgement of their complaints. The possibility of improvement in the various findings is discussed.

Urologic aspects of tethered cord.

Tethered cord syndrome, a form of spinal dysrhaphism, may involve vesical neurologic dysfunction. We present herein 60 cases of tethered cord syndrome, including 24 patients who underwent preoperative urodynamics studies. Preoperative cystometrography revealed areflexic bladders in 71 percent of cases and hyperreflexic bladders in 29 percent. Eight patients had serial pre- and postoperative urodynamics testing. Slight improvement was noted in postoperative urodynamics studies performed on 4 of 6 areflexic bladders and in the only hyperreflexic bladder studied. In addition, of the 3 patients in the serial urodynamics groups who had urinary symptoms preoperatively, 2 noted clinical improvement postoperatively. The urologist's role is important in the early detection, evaluation, and treatment of tethered cord syndrome and the neuropathic bladders that may result.

[Unusual pyramidal pathology syndrome]. / O svoeobraznom sindrome piramidnoi patologii.

A syndrome is described comprising: 1, impossibility to close only one eye (right or left) at a time, or each eye alternatively with intact capability of closing both eyes; 2, unvoluntary screwing up of an eye during voluntary closing of another (palpebro-palpebral synkinesia); 3, head turning to the side of an eye closed (palpebrocervical synkinesia); 4, with the hands clenched, left thumb is over the right, as is the left arm over the right when folded. The syndrome is supposed to be related with consealed lefthandedness which is presumably indicative of lateralization of the dominant speech center. If this is correct, the syndrome may have a distinct topical diagnostic value.

Risk factors for developing voiding dysfunction after abdominoperineal resection for adenocarcinoma of the rectum.

Voiding dysfunction is a common sequel of abdominoperineal resection of the rectum. Twenty patients symptomatic after abdominoperineal resection, 14 with a preoperative normal urodynamic study and six with evidence of obstruction, were studied postoperatively. The importance of the following factors is analyzed: sex, stage, grade, size, distance of the tumor from the anal verge, metastatic lymph-node involvement, and extent of lymphadenectomy. Male gender, tumors situated between 4 and 8 cm from the anal verge, and lymphadenectomy that includes more than ten nodes may be considered risk factors for neurologic damage and postoperative voiding dysfunction.

Micturitional dysfunction after anterior resection for rectal cancer. Rehabilitative treatment.

A clinical study was made of 21 patients (13 men and eight women) who had undergone anterior resection of the rectum for cancer at the National Tumour Institute of Milan between April 1984 and April 1985. After surgery, 13 patients (including three men with benign prostatic hypertrophy) showed voiding dysfunctions (hesitancy, dysuria, and weak stream) and bladder areflexia. Two of them also had positive Lapides' tests. An early rehabilitative treatment was started after surgery and the entire group was thoroughly reexamined one year later. Only the two patients with positive Lapides' tests still had bladder areflexia with residual urine greater than 100 ml. One of them also had a urinary tract infection. None of them showed decreased renal function.

Increased current delivery and sperm collection using nifedipine during electroejaculation in men with high spinal cord injuries.

Elevated blood pressure associated with autonomic hyperreflexia during electroejaculation in persons with high spinal cord injuries often prevents successful sperm retrieval. The ability of the calcium channel blocker nifedipine to reduce the effects of autonomic hyperreflexia, to facilitate greater current delivery, and to increase sperm collection was evaluated in six persons with spinal cord injuries. Ten milligrams of nifedipine given sublingually ten to 15 minutes before electroejaculation attempts helped to moderate autonomic hyperreflexia and the associated blood pressure elevations. These effects of nifedipine allowed greater current delivery and ultimately increased the chances of successful sperm retrieval in the six men. No adverse drug effects were observed.

Perinatal correlates of major and minor neurological dysfunction at school age: a multivariate analysis.

A prospective study was carried out on 747 infants: 147 neurologically abnormal, 300 with mild neurological abnormalities and 300 normal infants. They were re-examined at nine years of age, with special attention being paid to minor neurological dysfunction (MND). Extensive data on obstetrical history, neonatal course and interval complications were collected. Neurological handicap was not found on follow-up in the group of normal newborns. Other than neonatal neurological abnormality, risk factors contributing significantly to later handicap were low one-minute Apgar scores, a disturbed neonatal course, low social-class and interval complications; obstetrical events were conspicuous by their absence. Two aetiologically and clinically distinct kinds of MND were distinguished on the basis of a neurological cluster profile: MND-1 (one or two abnormal clusters) was only associated with a birthweight below the 2.3 centile and male gender, and MND-2 (more than two abnormal clusters) was associated with neonatal neurological findings, social class, obstetrical optimality score and gender.

Autonomic hyperreflexia, cesarean section and anesthesia. A case report.

Autonomic hyperreflexia, a potentially life-threatening condition, can appear during labor, delivery or cesarean section in a woman with a spinal cord injury. A case of autonomic hyperreflexia in a parturient was managed with regional neural blockade.

[Polyneuropathies following administration of tetanus toxoid]. / Polyneuropathien nach Gabe von Tetanustoxoid.

Two cases are reported: one with an asymmetric polyneuropathy involving the left lower extremity, the other with a generalised chronic relapsing-remitting demyelinating polyneuropathy, hitherto not described in this context. The findings are discussed with regard to the relevant literature.

Carotid sinus syndrome associated with an occult primary nasopharyngeal carcinoma.

A patient presented with syncope, and a diagnosis of carotid sinus syndrome was established. Further evaluation revealed a large nasopharyngeal carcinoma invading the left petrous bone. There were no cervical metastases. The tumor partially regressed with radiation therapy, and there was complete resolution of the carotid sinus syndrome, but the patient died three months later of metastatic disease. Autopsy confirmed petrous bone involvement by the mass but no cervical metastatic disease. This is the first reported case (to our knowledge) of malignancy-associated carotid sinus syndrome attributable to a primary nasopharyngeal carcinoma. The literature on malignancy-associated carotid sinus syndrome is reviewed, with emphasis on pathophysiology and treatment.

Pseudotumor cerebri, spinal and radicular pain, and hyporeflexia: a clinical variant of the Guillain-Barré syndrome?

A 5-year-old patient developed pseudotumor cerebri, spinal and radicular pain, and hyporeflexia. Ataxia and multiple cranial nerve involvement also were evident. With the exception of residual hyporeflexia and the abnormalities present in the electrophysiologic studies, the patient's prompt recovery suggested the diagnosis of Guillain-Barré polyradiculoneuritis.

The localization of the lesion in patients with acute ophthalmoplegia, ataxia and areflexia (Miller Fisher syndrome). A serial multimodal neurophysiological study.

Results of comprehensive serial neurophysiological tests from onset to full recovery in 3 patients with the Miller Fisher syndrome (acute ophthalmoplegia, ataxia and areflexia) are presented. These included EMG and nerve conduction, late response (H and F wave) and direct facial motor and blink reflex studies, computerized motor unit number estimation, automated quantitative sensory threshold measurements, quantitative pupillometric and pupillopharmacological studies and multimodality evoked potential (VEP, SEP and BAEP) and EEG recordings. The results provided unequivocal evidence of peripheral nerve dysfunction. Improvement of the peripheral neurophysiological parameters accompanied or followed clinical recovery in all 3 patients. No abnormality in the CNS pathways investigated by these tests was found. The findings support the conclusion that this syndrome is to be included within the spectrum of acute inflammatory polyneuropathy. The value of serial measurements in detecting milder peripheral nerve lesions is emphasized.