Autonomic neuropathy: clinical and instrumental findings

A good staging system should be able to accurately reflect the natural history of a malignant disease, to express the extent of the disease at the time of diagnosis, and stratify patients in prognostically distinctive groups. The staging system for prostate cancer, as it is today, fails to fulfill these requirements. Approximately one third of the patients who undergo surgery for complete excision of prostate cancer in fact do not have a localize disease. The incidence of tumor at the inked margin may reach 30% for T1 stage and up to 60% for clinical T2b prostate cancer according to comparison with pathologic examination of resected specimen. Several concepts have been recently proposed as a means of improving the accuracy of the available staging system. In this paper, we review current aspects of clinical and pathological staging of prostate cancer, and the importance of these new concepts on the early stages of prostate cancer.     

Orthostatic tremor: clinical and electrophysiologic characteristics

Orthostatic tremor, sometimes known as "shaky legs syndrome," is a disorder of middle-aged or elderly people characterized by feelings of unsteadiness in the legs and a fear of falling when standing. Patients stand on a wide base but walk normally. These symptoms are due to high-frequency (13-18 Hz) burst firing in weight-bearing muscles. They are attenuated by walking and are abolished immediately by sitting. Some authors believe that the disorder is a variant of essential tremor. This study reports the clinical and electrophysiologic features of orthostatic tremor in 30 patients. The findings indicate that orthostatic tremor is distinct from essential tremor, both clinically and electrophysiologically. The major differences are the frequency of electromyographic burst firing, the invariable involvement of lower limb and paraspinal muscles, and the task-specific nature of the tremor in orthostatic tremor. The study shows that the diagnosis can be established rapidly with surface electromyographic recordings.     

Hereditary neuralgic amyotrophy and hereditary neuropathy with liability to pressure palsies: two distinct clinical, electrophysiologic, and genetic entities

Fistulas secondary to Crohn's disease occur in about 33% of patients. A colobronchial fistula complicating Crohn's disease is very rare, having been reported only twice previously. We present an unusual fistula secondary to Crohn's colitis that originated from the splenic flexure and crossed the diaphragm to involve the bronchial tree.     

Diabetic neuropathy: clinical aspects

The importance of diabetic neuropathy derives from its remarkable frequency and its clinical impact. In view of the varying underlying pathogenetic mechanisms and the resulting diversity of clinical representations, it becomes apparent that there are diabetic neuropathies, rather than a single entity of diabetic neuropathy. The scope of involvement is widespread with virtually every system at risk. Although peripheral neuropathy is by far the most common expression, visceral neuropathy is also highly significant. It may affect every part of the gastrointestinal tract, the genitourinary tract, and sexual function, as well as direct autonomic nerve pathology. Clearly, neuropathy in diabetes offers a specific and important diagnostic challenge to the clinician and plays a definitive role in differential diagnosis. The problem is heightened by the fact that any and all of the diabetic neuropathic syndromes may be the initial clinical manifestation of diabetes in the absence of covert manifestations of carbohydrate metabolic disorder. It is to be stressed that the diagnosis is more than an academic exercise, since each diabetic neuropathic syndrome carries with it some beneficial therapeutic modality to aid the patient.     

Electrophysiologic findings in multifocal motor neuropathy

We performed detailed electrophysiologic studies on 16 patients with clinically defined multifocal motor neuropathy and found a wide spectrum of demyelinating features. Only five patients (31%) had conduction block in one or more nerves. However, in 15 patients (94%) at least one nerve showed other features of demyelination. We also noted a significant degree of superimposed axonal degeneration in 15 patients. Eight patients (50%) had individual nerves with pure axonal injury, despite the presence of demyelinating features in other nerves. Antiganglioside antibodies were elevated in four of five patients with conduction block and five of 11 patients without conduction block. We conclude that multifocal motor neuropathy is characterized electrophysiologically by a wide spectrum of axonal and demyelinating features. Diagnostic criteria requiring conduction block may lead to underdiagnosis of this potentially treatable neuropathy.     

Painful trigeminal neuropathy: clinical and pharmacological observations

A 74-year-old woman had a 5-year history of constant burning pain and numbness of the central face of subacute onset. The central region of the face, oral cavity, and nose lacked all sensation. Corneal reflexes and the jaw jerk were absent. Blood tests, rectal biopsy, neurodiagnostic studies, and surgical exploration of the trigeminal nerve were normal. Blink reflexes were absent. Facial nerve motor latencies and EMG of the facial and masseter muscles were normal. Responses to the thermoregulatory sweat test, intradermal histamine, and simulated diving were present. Oral administration of 500 mg L-dopa aggravated her pain and produced transient hypalgesia in the C2 through C6 dermatomes. Infraorbital nerve biopsy demonstrated loss of large myelinated fibers. In conclusion: (1) Only the central region of the face is exclusively supplied by the trigeminal nerves. (2) Somato-autonomic reflexes coupled with electrophysiological studies localized the lesion to the large fibers. (3) Large fiber loss and central brain stem reorganization may explain the burning pain. (4) Dopamine may modulate trigeminal nociception.     

Hemifacial spasm: clinical findings and treatment

Hemifacial spasm (HFS) is a peripherally induced movement disorder characterized by involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve. We reviewed the clinical features and response to different treatments in 158 patients (61% women) with HFS evaluated at our Movement Disorders Clinic. The mean age at onset was 48.5+/-14.1 years (range: 15-87) and the mean duration of symptoms was 11.4+/-8.5 (range: 0.5-53) years. The left side was affected in 56% instances; 5 patients had bilateral HFS. The lower lid was the most common site of the initial involvement followed by cheek and perioral region. Involuntary eye closure which interfered with vision and social embarrassment were the most common complaints. HFS was associated with trigeminal neuralgia in 5.1% of the cases and 5.7% had prior history of Bell's palsy. Although vascular abnormalities, facial nerve injury, and intracranial tumor were responsible for symptoms in some patients, most patients had no apparent etiology. Botulinum toxin type A (BTX-A) injections, used in 110 patients, provided marked to moderate improvement in 95% of patients. Seven of the 25 (28%) patients who had microvascular decompression reported permanent complications and the HFS recurred in 5 (20%). Although occasionally troublesome, HFS is generally a benign disorder that can be treated effectively with either BTX-A or microvascular decompression.     

Unverricht-Lündborg disease: clinical and electrophysiologic study of 19 Maghreb families

We describe clinical, electrophysiological and genetic features in 44 patients with Unverricht-Lündborg disease from 19 families living in North African countries (Tunisia, Algeria and Morocco). The mean age of patients was 25.3 years; mean age was at onset 11.3 years. The disease began more frequently with seizures (91 per cent) or myoclonus (80 p. 100) than ataxia (16 p. 100). Subsequently myoclonus and generalized seizures were present in all patients, cerebellar signs were absent in four cases. EEG findings included normal background activity (90 p. 100), spontaneous fast generalized spikes (93 p. 100) and photosensitivity (70 p. 100). Antiepileptic polytherapy (clonazepam and/or phenobarbital and/or valporic acid) was used in 84 per cent of cases. Antiepileptic drugs were more effective in controlling epileptic seizures (less than one seizure/month in 60 p. 100) than myocloni which persisted daily in 64 p. 100 of cases. Mean duration of the disease was 13.5 years. One patient died of status epilepticus. Consanguinity was noted in 17 families (first degree in 15 families). Linkage to chromosome 21q 22.3 was confirmed in 11 families. We noted an inter and intrafamilial variability of clinical signs and disease course.     

Early detection of diabetic visceral neuropathy. An electrophysiologic study of bladder and urethral innervation

The segmental and supraspinal innervation of the detrusor muscle and periurethral striated musculature was studied in 27 patients with diabetes mellitus by gas cystometry, integrated sphincter electromyography, and spinal evoked-response latency measurements. Slowing of neural conduction velocities was a consistent finding in all the patients, even when cystometry did not show abnormalities. Thus, neuropathy in the segmental innervation of the bladder and urethra was documented.     

Primary muscle lymphoma: clinical and imaging findings

PURPOSE: To assess the clinical and imaging findings in primary muscle lymphoma. MATERIALS AND METHODS: Seven patients with biopsy-proved primary muscle lymphoma without evidence of systemic disease underwent imaging with plain radiography or computed tomography (CT) and magnetic resonance (MR) imaging. Four underwent bone scintigraphy, and two underwent gallium scintigraphy and fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) before and after therapy. RESULTS: Plain radiographs at initial examination (n = 5) showed no bone abnormalities. Soft-tissue masses and bone marrow involvement showed isoattenuation at CT (n = 3), but at MR imaging (n = 7), all masses demonstrated increased signal intensity on T2-weighted images that involved multiple muscle compartments and typically spanned a long segment of the extremity. Adjacent bone disease was less extensive than muscle disease, and, in most cases, subcutaneous stranding or extension was observed adjacent to the masses. Good size correlation was observed between findings at MR imaging, gallium scintigraphy, and FDG PET. Two patients developed recurrent multifocal muscle lymphoma several years after initial examination. CONCLUSION: The presence of an extensive soft-tissue mass with infiltration of adjacent subcutaneous fat and minimal or no extension into the bone marrow cavity at MR imaging and normal plain radiographic findings may suggest primary muscle lymphoma.     

MRI findings in patients with acute autonomic and sensory neuropathy

Acute autonomic and sensory neuropathy (AASN), characterized by acute onset of extensive autonomic dysfunction and severe sensory deficits, was first described by Colan et al. (1978). We present two female patients with AASN in whom magnetic resonance imaging (MRI) confirmed such findings in the posterior column of the spinal cord. One patient was a 44-year-old woman who developed an upper respiratory tract infection followed in 2 weeks by numbness of the limbs and gait disturbance. There was orthostatic hypotension with syncope, paretic ileus, anhidrosis and urinary retention. There was a loss of sensation over the entire body, including the face, and deep tendon reflexes were generally absent. Neurophysiologic studies showed that sensory nerve action potentials and SSEPs were not evoked in the nerves examined. Sural nerve biopsy demonstrated severe axonal degeneration of the myelinated and unmyelinated fibers. Our second patient, a 27-year-old woman, exhibited similar clinical and laboratory features. The autonomic dysfunction in both patients improved gradually without drug treatment, but the sensory deficits--predominantly a loss of deep sensation--persisted for several years. In both patients, MRI revealed the T2*-weighted high intensity area in the fasciculus gracilis of the posterior column of the spinal cord. Such high intensity areas were present in all spinal segments. The severe and persistent sensory disturbance in these patients may have been caused by a lesion of the posterior column of the spinal cord following the involvement of the dorsal root ganglion cells, or ganglioneuronopathy, as demonstrated by MRI.     

Spontaneous bacterial peritonitis: clinical study, microbiological findings and clinical course

One hundred and forty-four episodes of spontaneous bacterial peritonitis (SBP) treated in our service between July 1988 and September 1995 were studied retrospectively to assess the clinical presentation, microbiological findings, possible pathogens, treatment and course. Ascites, abdominal pain and fever were the most common symptoms. Only 3.5% of cases were asymptomatic. The outcome was fatal in 12 (8.33%). Among the factors analyzed, only a prothrombin time of less than 35% correlated significantly with a higher mortality rate (60% and 8.33%, respectively; p < 0.01). Ascitic fluid culture was positive in 43.05% of cases; significant differences existed between these patients and those with negative ascitic fluid culture with respect to clinical findings or course. Gram-negative microorganisms were those most frequently isolated (48.38%). Treatment was initiated within 12 hours in 77.7% of the patients, between 12 and 72 hours in 11.8% and later in 10.41%. Intravenous cefotaxime was administered in 86.1% of cases and other drugs or drug combinations in only 13.9%; the mortality rate was much lower with cefotaxime (2.4% vs 45%; p < 0.01).     

Bronchopulmonary dysplasia: correlation of radiographic and clinical findings

BACKGROUND AND PURPOSE: Abnormalities of the chest wall have been described in bronchopulmonary dysplasia (BPD). Clinical, radiographic and pulmonary function variables were evaluated in 1-year-old children ventilated because of neonatal lung disease in order to quantify these thoracic changes and to evaluate the lung disease. METHODS: The pulmonary status of 51 infants with neonatal lung disease requiring artificial ventilation was reevaluated clinically and radiographically at the age of 1 year. Twenty-two of these infants had developed BPD. Thoracic depth and width were measured clinically and on chest X-ray. The Toce score evaluated the presence of cardiomegaly, hyperinflation, emphysema and interstitial lung disease. Lung function was measured after sedation using previously reported methods. In BPD patients, Toce score and lung function were determined and compared at 1 month and at 1 year of age. RESULTS: In BPD patients, chest depth was significantly smaller when measured clinically as well as on chest radiograph (P < 0.05; Mann-Whitney U-test). There was a statistically significant correlation between chest depth measured clinically and on chest X-ray. Toce score was significantly higher in BPD patients (P < 0.05). In BPD patients intersitial abnormalities and decreased lung compliance were more frequent at the age of 1 month than at the age of 1 year. At the age of 1 year, hyperinflation was more frequent and at that time increased airway resistance was still noted. Thus the type of X-ray abnormality reflects the type of lung function disturbance. CONCLUSION: The flatness of the chest is most likely a consequence of the long-standing lung function abnormalities.     

"Primary" orthostatic tremor. 10 clinical electrophysiologic observations

Distraction osteogenesis has been shown to be an effective method of lengthening and augmenting endochondral bone. It has also been applied effectively in the reconstruction of the membranous bones of the craniofacial skeleton. With the accumulation of clinical experience in mandibular distraction, the differences between endochondral and membranous bone distraction have become apparent, especially in the limitations of uniplanar distraction for the three-dimensional reconstruction of the deficient mandible. Distraction of the mandible in a single plane cannot satisfy fully the functional and structural requirements of the patient with malocclusion as well as deficiency of the skeletal and soft tissue. This study reports the development and clinical use of a multiplanar mandibular distraction device with the ability to achieve linear distraction (Z-plane or sagittal), angular distraction (Y-plane or vertical), and transverse distraction (X-plane or coronal). The device contains two independent gear arrangements attached to two arms that extend from the central unit. Therefore, the trajectory of the regenerated bone may be changed during the distraction process. The device also allows manipulation of the various planes of movement independent of each other. Furthermore, the rotational points for the multiplanar distraction devices are located at a single point; therefore only a single osteotomy and two pin sites are required. The multiplanar distraction device allows the surgeon to customize and contour the dimensions of the distraction process by controlling the trajectory of the translation of the regenerated bone.     

Right fetal cardiac axis: clinical significance and associated findings

A basic aspect of the neuronal control of bipedal stance and gait represents the antigravity function of leg extensors. Proprioceptive reflexes involved in the maintenance of body equilibrium depend on the presence of contact forces opposing gravity. Extensor load receptors are thought to signal changes of the projection of the body's centre of mass with respect to the feet. According to recent observations in the spinal cat, this afferent input probably arises from Golgi tendon organs and represents a newly discovered function of these receptors in the regulation of stance and gait. In humans, evidence for a significant contribution of the load receptor to leg muscle activation has come from immersion experiments. Compensatory leg muscle activation depends on the actual body weight. Furthermore, recent experiments in paraplegic patients showed that the beneficial effects of a locomotor training critically depends on the initial degree of body unloading and reloading during the course of the training period.