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At least 847 records · Page 47Linked to original sources
PubMed1993

Accelerated neuropathy of renal failure.

OBJECTIVE: To describe a syndrome of rapidly evolving polyneuropathy in patients with severe renal failure. DESIGN: Retrospective case series of four patients. SETTING: In-hospital evaluations and personal examinations of patients. PATIENTS: Four patients with severe or end-stage renal failure who were receiving peritoneal dialysis. RESULTS: These patients had an acute or subacute syndrome characterized by generalized limb weakness over days or weeks, severe imbalance, diminished reflexes, and numbness. Spinal fluid protein levels were elevated and some demyelinating features were noted on electrophysiological testing. Improvement occurred with more frequent peritoneal dialysis in one patient and transplantation in another, but the neuropathy progressed in the other two in whom diabetes may have played a role. CONCLUSION: This partly reversible acute uremic neuropathy, which is probably caused by the metabolic disturbances of end-stage renal failure, simulates Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy and may be complicated by diabetic neuropathy.

Adult↗
PubMed1993

Wallenberg's lateral medullary syndrome. Clinical-magnetic resonance imaging correlations.

OBJECTIVE: To correlate clinical and radiologic findings in patients with lateral medullary infarction. DESIGN: Case series with "blinded" evaluation of brain imaging. SETTING: Hospitalized and ambulatory patients at the Neurological Institute of New York (NY). PATIENTS: Thirty-three consecutive patients with lateral medullary syndrome were evaluated by the Stroke Center between 1983 and 1989. RESULTS: Ataxia (70%), numbness either of the ipsilateral face or of the contralateral body (64%), vertigo (51%), and dysphagia (51%) were the most frequent symptoms at onset. Eleven patients had ocular symptoms (diplopia or blurred vision). Horner's syndrome was found in 91%, ipsilateral ataxia in 85%, and contralateral hypalgesia in 85%. Nystagmus (61%) and facial weakness (42%) were less frequent. Head computed tomography was abnormal only when a cerebellar infarction was present (three cases). Magnetic resonance imaging, obtained in 22 cases, was normal in two; a lateral medullary infarction alone was present in 12, and a lesion extending beyond the lateral medulla was found in eight. No correlation was noted between facial weakness or ocular symptoms and infarction extending beyond the lateral medullary region. Vertebral artery disease was confirmed by vascular imaging or insonation studies in 73% of patients. CONCLUSIONS: The triad of Horner's syndrome, ipsilateral ataxia, and contralateral hypalgesia will clinically identify patients with lateral medullary infarction. Facial weakness and ocular symptoms are frequent and do not necessarily imply that the infarction extends beyond the lateral medulla. Cerebellar infarcts only infrequently accompany lateral medullary syndrome, suggesting that most of the posterior inferior cerebellar artery territory is spared, despite the high frequency of vertebral artery occlusion.

Adult↗
PubMed2001

Complications of gamma knife surgery for Parkinson disease.

BACKGROUND: Many medical centers throughout the world offer radiosurgery with the gamma knife (GK) for pallidotomy and thalamotomy as a safe and effective alternative to radiofrequency ablative surgery and deep brain stimulation for Parkinson disease (PD). The reported incidence of significant complications varies considerably, and the long-term complication rate remains unknown. DESIGN: We describe 8 patients seen during an 8-month period referred for complications of GK surgery for PD. RESULTS: Of the 8 patients, 1 died as a result of complications, including dysphagia and aspiration pneumonia. Other complications included hemiplegia, homonymous visual field deficit, hand weakness, dysarthria, hypophonia, aphasia, arm and face numbness, and pseudobulbar laughter. In all patients, lesions were significantly off target. CONCLUSIONS: The 8 patients with PD seen in referral at our center for complications of GK surgery highlight a spectrum of potential problems associated with this procedure. These include lesion accuracy and size and the delayed development of neurological complications secondary to radiation necrosis. Gamma knife surgery may have a higher complication rate than has been previously appreciated due to delayed onset and underreporting. We believe that the risk-benefit ratio of the GK will require further scrutiny when considering pallidotomy or thalamotomy in patients with PD. Physicians using this technique should carefully follow up patients postoperatively for delayed complications, and fully inform patients of these potential risks.

Aged↗
PubMed2002

Creutzfeldt-Jakob disease with amyotrophy and demyelinating polyneuropathy.

OBJECTIVE: To report the clinical and neuropathological features in a patient with Creutzfeldt-Jakob disease with amyotrophy and demyelinating polyneuropathy. DESIGN: Case report. PATIENT AND RESULTS: A 62-year-old man had progressive numbness of the left foot, unsteady gait, diminished deep reflexes, fasciculations, and tactile hypesthesia on the feet. Cerebrospinal fluid, electroneurography, and electromyography were suggestive of chronic inflammatory demyelinating polyneuropathy. He was treated with plasmapheresis, corticosteroids, and immunglobulins, with minimal improvement. After 2 months, severe amyotrophy, polyneuropathy, cerebellar signs, and dementia developed, and he died 8 months after onset of the disease. Autopsy and prion protein immunohistochemistry proved typical Creutzfeldt-Jakob disease. No mutation was found in the prion protein gene, and the codon 129 polymorphism was methionine-valine. In the ventral horn, the loss of the motoneurons was accompanied by prion protein immunoreactivity. The peripheral nerves were segmentally demyelinated but free of prion protein deposition. CONCLUSIONS: The view that peripheral neuropathy and amyotrophy may occasionally be an integral part of Creutzfeldt-Jakob disease is supported by our case, which showed these abnormalities simultaneously. These symptoms, when prominent, may cause problems in differential diagnosis.

Brachial Plexus Neuritis↗
PubMed2002

Efficacy of intravenous immunoglobulin in patients with IgG monoclonal gammopathy and polyneuropathy.

CONTEXT: The optimal treatment of patients with neuropathy associated with IgG monoclonal gammopathy of undetermined significance is unknown. Plasma exchange has been shown to be effective but alternative therapies have not been systematically evaluated. OBJECTIVE: To report our experience with intravenous immunoglobulin (IVIG) in patients with IgG monoclonal gammopathy of undetermined significance polyneuropathy. DESIGN: Retrospective review of clinical and electrodiagnostic features of 20 consecutive patients treated with IVIG over an 8-year period. SETTING: Academic medical center. MAIN OUTCOME MEASURES: Medical Research Council strength (maximum, 40 points) and sensory (maximum, 26 points) scores, modified Rankin Disability Scale score. RESULTS: There were 14 men and 6 women (mean age, 65 years; age range, 36-82 years). The mean strength score was 35.6 points and the mean sensory score was 15.8 points prior to therapy. After IVIG therapy, the mean strength score increased by 1.1 points (P =.22) and the sensory score increased by 1.7 points (P =.11). Eight patients (40%) improved by 2 points or more in their motor or sensory score and 1 point or more in the modified Rankin Disability Scale score and were considered IVIG therapy responders. They had a shorter duration of symptoms (P =.03), numb hands (P =.02), and falling episodes (P =.02), and had greater proximal leg weakness (P =.02) compared with nonresponders. In IVIG therapy responders, the ulnar motor conduction velocity was slower, ulnar and peroneal distal motor latencies were prolonged, and the frequency of conduction block was higher (13 of 36 motor nerves in responders vs 6 of 53 in nonresponders, P =.008). CONCLUSIONS: Intravenous immunoglobulin therapy was beneficial in 8 (40%) of our 20 patients with polyneuropathy and IgG monoclonal gammopathy of undetermined significance. Proximal leg weakness, short duration of symptoms, and demyelinating features on electrodiagnostic studies were associated with a response to IVIG therapy.

Adult↗
PubMed2003

Cerebral cavernous malformations with dynamic and progressive course: correlation study with vascular endothelial growth factor.

BACKGROUND: Cerebral cavernous malformations (CCMs) are reported to exhibit a wide range of dynamic patterns including growth, regression, and de novo formation, which generally show slow and steady courses. Although the pathogenesis of CCMs is not well known, vascular endothelial growth factor (VEGF) has been suggested as a possible mediating factor. OBJECTIVES: To report CCMs showing rapid progression over a short period and to investigate these biological characteristics. DESIGN: Experimental study. SETTING: Tertiary referral center, neurology department. Patient A 40-year-old man was admitted because of a left-sided numbness, vertigo, and ataxia, which were attributed to a pontine hemorrhage. He had experienced a left-sided weakness 6 months before admission, and thereafter had complained of intermittent headache. Serial brain magnetic resonance images showed multiple intracerebral microhemorrhages throughout the cerebral hemispheres. A biopsy of the lesion confirmed the diagnosis of CCM. MAIN OUTCOME MEASURES: We investigated the expression of VEGF by immunohistochemistry of the biopsy specimen. Dynamic patterns of CCMs, obtained with spin-echo magnetic resonance images with gradient-echo sequences, were compared with serial serum VEGF concentrations, determined by enzyme-linked immunosorbent assay. RESULTS: Immunohistochemistry of the specimen displayed increased VEGF expression. Serial magnetic resonance images during 7 months showed dynamic signal changes of the preexisting lesions and 15 de novo formations in many cortices. The VEGF level in serum increased during this dynamic period and became normal during the steady and resolving stages. CONCLUSIONS: Cerebral cavernous malformations can be progressively deteriorating. The endothelial proliferation induced by VEGF is likely to be an important aspect of the pathogenetic mechanisms of CCMs.

Adult↗
PubMed2004

Transverse myelitis in systemic sclerosis.

BACKGROUND: Neurological involvement occurs rarely with systemic sclerosis (SSc). Only a few cases of transverse myelopathy have been reported in the setting of SSc. OBJECTIVE: To describe a patient with SSc who developed transverse myelitis that improved during a course of immunosuppression. RESULTS: A 30-year-old woman with SSc presented with subacute onset of bilateral lower extremity weakness and numbness. Results of magnetic resonance imaging and cerebrospinal fluid studies supported a diagnosis of transverse myelitis. The patient responded favorably to a course of corticosteroids and cyclophosphamide. No overlapping autoimmune disorders were evident. Clinical follow-up showed significant recovery, with resolution of radiological abnormalities. CONCLUSION: Transverse myelitis can occur as a rare manifestation of SSc and may respond favorably to immunosuppressive therapy.

Adrenal Cortex Hormones↗
PubMed2004

HIV-associated distal sensory polyneuropathy in the era of highly active antiretroviral therapy: the Manhattan HIV Brain Bank.

OBJECTIVES: To examine distal sensory polyneuropathy (DSP) in a highly active antiretroviral therapy era, human immunodeficiency virus (HIV)-infected cohort, to determine whether clinical manifestations are affected by demographic or other clinical variables. PATIENTS: One hundred eighty-seven patients with HIV infection enrolled in the Manhattan HIV Brain Bank underwent baseline neurologic evaluations between January 29, 1999, and June 17, 2002. Distal sensory polyneuropathy was diagnosed if patients displayed abnormalities in 2 or more of the following: ankle reflexes or vibratory or pinprick perception. Patients were classified as symptomatic if they described pain, paresthesia, or numbness. Nonneurologic information was obtained by interview, laboratory testing, and medical chart review. Psychiatric and substance use disorders were elucidated by semistructured interview. In 36 patients, morphometric analysis was performed on autopsy-derived sural nerves. RESULTS: Of 187 patients, 99 (53%) had DSP. Patients with neuropathy were older than those without (mean +/- SD age, 45.3 +/- 0.7 vs 41.2 +/- 0.8 years, P <.001), and DSP was significantly more common in men (58% [83/99]) than in women (37% [16/99]) (P =.02). The presence of neuropathy was not correlated with plasma viral load, decreased CD4 cell counts, or neurotoxic antiretroviral therapy. Twenty-six of 99 patients with DSP were asymptomatic. Asymptomatic neuropathy was correlated with histories of opiate and sedative abuse and dependence. Symptomatic DSP correlated with ethanol and hallucinogen syndromes, but not neurotoxic therapy. Sural nerve morphometric findings did not distinguish between patients with substance use syndromes and those without. CONCLUSIONS: In contrast to populations before the era of highly active antiretroviral therapy, DSP in the Manhattan HIV Brain Bank cohort is not associated with increased viral load or decreased CD4 cell counts in this cross-sectional analysis. Symptoms in DSP are associated with substance use disorders, but no difference in morphologic structure is seen in nerves of patients with HIV infection with and without substance use histories. Previously reported virologic and immunologic underpinnings of DSP may be affected by highly active antiretroviral therapy. Furthermore, symptoms of DSP in substance users may be altered by central mechanisms of increased or decreased tolerance to sensory disturbance.

Adult↗
PubMed1998

Parry-Romberg syndrome associated with intracranial vascular malformations.

We describe a 23-year-old woman with iridocyclitis, enophthalmos, facial hemiatrophy, and transient numbness of her contralateral upper and lower extremities. The patient was found to have white matter densities in the right hemisphere in magnetic resonance T2-weighted images and vascular malformations involving right vertebral, right carotid, and right anterior cerebral arteries. Histopathologic evaluation of a biopsy specimen of anterior orbital fat and lacrimal gland revealed fibrosis and chronic inflammation. These findings were consistent with the diagnosis of progressive facial hemiatrophy (Parry-Romberg syndrome) in association with iridocyclitis and intracranial vascular malformations.

Adipose Tissue↗
PubMed2001

Fibrous dysplasia involving the skull base and temporal bone.

OBJECTIVE: To gain a broader appreciation of the clinical presentation, operative treatment, and outcome of patients with fibrous dysplasia involving the skull base. DESIGN: Retrospective review of a clinical case series. SETTING: A single tertiary academic medical center. PATIENTS: Twenty-one patients with histopathologically confirmed fibrous dysplasia involving the skull base cared for over a 15-year-period (1983-1998). MAIN OUTCOME MEASURES: Clinical and radiographic location of the fibrous dysplasia lesions within the skull base, clinical presentation, surgical intervention, and clinical outcome were tabulated for each patient. RESULTS: The ethmoids were most commonly involved (71%), followed by the sphenoid (43%), frontal (33%), maxilla (29%), temporal (24%), parietal (14%), and occipital (5%) bones. The most common presenting features included atypical facial pain and headache, complaints referable to the sinuses, proptosis and diplopia, hearing loss, and facial numbness. Surgical treatment, guided by clinical presentation, ranged from simple biopsy with conservative follow-up to craniofacial resection. CONCLUSIONS: Fibrous dysplasia can present in myriad ways within the skull base. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward. Surgery, particularly in such a challenging region as the skull base, should be reserved for patients with functional impairment or a cosmetic deformity. Because of the benign nature of the condition, the surgery itself should be relatively conservative, with the primary goal being preservation of existing function.

Adolescent↗
PubMed2001

Younger patients have a higher rate of recovery of taste function after middle ear surgery.

BACKGROUND: Although the chorda tympani nerve (CTN) is frequently damaged during tympanoplasty, little attention has been given to the patients' symptoms and taste function. OBJECTIVE: To investigate patients' symptoms and the functional recovery of taste after surgery using electrogustometry (EGM). DESIGN: Prospective study. PATIENTS: Analysis of 163 ears of 156 patients who underwent middle ear surgery from April 1997 through December 1999. There were 18 ears with noninflammatory diseases, 80 with chronic otitis media, and 65 with cholesteatoma. The patients' taste functions were examined 2 days before surgery and 2 weeks and 6 months after surgery. MAIN OUTCOME MEASURES: The taste disturbance before and after middle ear surgery and the relationship between age and the recovery rate of CTN function. RESULTS: Two weeks after surgery, the mean EGM threshold was elevated in all groups regardless of preservation or section of the CTN. Numbness in the tongue and taste disturbance were more frequently found in patients with preservation of CTN than in those with section of the CTN (P =.008 and P =.001, respectively). In patients with preservation of the CTN, 6 months after surgery, the recovery rate of EGM threshold was 83% in those aged 20 years or younger (P =.008 compared with the 2 older groups), 53% in the those aged 21 to 40 years, and 44% in those aged 41 to 60 years. CONCLUSION: Age is an important factor for recovery of taste function after middle ear surgery, which is useful information when explaining complications to patients.

Adolescent↗
PubMed2003

Endoscopic transnasal approach to the pterygopalatine fossa.

OBJECTIVE: To describe an endoscopic transnasal approach to the pterygopalatine fossa (PPF). DESIGN: Case series of 3 patients. SETTING: An academic medical center. Patients One patient presented with an asymptomatic PPF schwannoma. The second patient presented after a sudden onset of complete unilateral vision loss with a complete ipsilateral sphenoid sinus opacification and radiographic signal abnormality in the PPF and inferior orbital fissure. The third patient had a history of adenoid cystic carcinoma of the lacrimal gland, and was found to have new-onset facial numbness. Intervention One patient had a complete excision of a schwannoma by means of an endoscopic transnasal approach. The other 2 patients had wide exposure and biopsies of the PPF. One patient had a revision procedure through the same approach with further lateral exposure to the area of the inferior orbital fissure. RESULTS: All patients had successful endoscopic approaches for tumor removal (case 1) and biopsy (cases 2 and 3) of the PPF. The second patient had a repeat endoscopic biopsy 1 week later to obtain more tissue for diagnostic purposes. None of the patients had any major vascular complications. At follow-up, 2 of 3 patients had persistent sensory deficits. CONCLUSIONS: The endoscopic transnasal approach to the PPF is a safe and effective method for biopsy and removal of PPF masses. The endoscopic approach improves access and visualization, and has the potential to reduce complications compared with open approaches. Image guidance is helpful in these cases.

Adult↗
PubMed1976

Occult tumors of the infratemporal fossa: report of seven cases appearing as preauricular facial pain.

Seven patients had occult malignant neoplasms either originating in or extending to the infratemporal fossa. Common symptoms were facial pain centered over the temporomandibular joint and facial numbness. These symptoms were sometimes associated with middle ear effusion and trismus. Multiple biopsies are often necessary to establish the diagnosis. The average delay in diagnosis from first symptom was 14 months. Infratemporal fossa neoplasia belongs in the differential diagnosis of facial pain.

Aged↗
PubMed1985

Brain-stem auditory-evoked potentials during lidocaine infusion in humans.

Auditory brain-stem responses (ABR) were recorded in six healthy male volunteers during intravenous infusion of lidocaine that achieved systemic blood levels similar to those seen with conduction anesthesia and antiarrhythmic therapy. Following an initial loading dose of lidocaine (1 mg/kg), subjects noted prominent tinnitus, perioral numbness, and drowsiness. All of these symptoms except drowsiness abated during continued infusion as blood concentrations reached equilibrium. All subjects noted that the click stimuli used to elicit ABR varied markedly in intensity and character throughout the lidocaine infusion. Although waves I and III were unaffected by lidocaine, wave V exhibited significant decreases in amplitude and increases in latency. Therefore, the more central components of the auditory system seem to be the prominent site of lidocaine's central nervous system effects.

Acoustic Stimulation↗
PubMed1987

Audiologic and other clinical findings in a case of basilar artery aneurysm.

A patient with a large, right-sided basilar artery aneurysm was evaluated. Major symptoms included progressive hearing loss, facial numbness, occipital headaches, dizziness, and diplopia of less than a year's duration. Audiologic results indicated a low-frequency sensorineural hearing loss with marked discrepancies between ascending and descending pure-tone thresholds for the ear ipsilateral to the lesion. Auditory brain-stem response demonstrated bilateral abnormalities, but the early waves were normal. Contralateral acoustic reflexes were absent on the right and elevated on the left. Electronystagmography results showed bilaterally absent caloric responses as well as gaze nystagmus and abnormal pursuit movements. Additional audiologic results as well as radiologic and medical findings are also presented.

Aneurysm↗
PubMed1992

Spontaneous dissection of the cervical internal carotid artery. Presentation with lower cranial nerve palsies.

Spontaneous dissections of the internal carotid arteries are uncommon but are not rare. They constitute a fairly common cause of ischemic stroke in young patients (young in terms of the age at which strokes generally occur). The common presenting manifestations are (1) unilateral headaches followed after a period of delay by focal cerebral ischemic symptoms or (2) unilateral headaches and ipsilateral incomplete Horner's syndrome. These may or may not be associated with subjective or objective bruits. In rare instances, spontaneous dissections of the internal carotid arteries may present as lower cranial nerve palsies and cause dysphonia, dysarthria, dysphagia, and numbness of the throat. Affected patients may initially present to the otolaryngologist or be referred to one. This article describes eight patients with spontaneous dissections of the internal carotid arteries and lower cranial nerve palsies, and the pertinent literature is reviewed.

Adult↗
PubMed1993

A twin study of genetic and environmental contributions to liability for posttraumatic stress symptoms.

We studied 4042 Vietnam era veteran monozygotic and dizygotic male twin pairs to determine the effects of heredity, shared environment, and unique environment on the liability for 15 self-reported posttraumatic stress disorder symptoms included in the symptom categories of reexperiencing the trauma, avoidance of stimuli related to the trauma, and increased arousal. Quantitative genetic analysis reveals that inheritance has a substantial influence on liability for all symptoms. Symptoms in the reexperiencing cluster and one symptom in the avoidance and numbing cluster are strongly associated with combat exposure, and monozygotic pairs are more highly concordant for combat exposure than dizygotic pairs. By fitting a bivariate genetic model, we show that there are significant genetic influences on symptom liability, even after adjusting for differences in combat exposure; genetic factors account for 13% to 30% of the variance in liability for symptoms in the reexperiencing cluster, 30% to 34% for symptoms in the avoidance cluster, and 28% to 32% for symptoms in the arousal cluster. There is no evidence that shared environment contributes to the development of posttraumatic stress disorder symptoms.

Asia, Southeastern↗
PubMed1993

Abnormal noradrenergic function in posttraumatic stress disorder.

To evaluate possible abnormal noradrenergic neuronal regulation in patients with posttraumatic stress disorder (PTSD), the behavioral, biochemical, and cardiovascular effects of intravenous yohimbine hydrochloride (0.4 mg/kg) were determined in 18 healthy male subjects and 20 male patients with PTSD. A subgroup of patients with PTSD were observed to experience yohimbine-induced panic attacks (70% [14/20]) and flashbacks (40% [8/20]), and they had larger yohimbine-induced increases in plasma 3-methoxy-4-hydroxyphenylglycol levels, sitting systolic blood pressure, and heart rate than those in healthy subjects. In addition, in the patients with PTSD, yohimbine induced significant increases in core PTSD symptoms, such as intrusive traumatic thoughts, emotional numbing, and grief. These data were consistent with a large body of preclinical data that indicated that uncontrollable stress produces substantial increases in noradrenergic neuronal function. We discuss the implications of these abnormalities in noradrenergic functional regulation in relation to the long-term neurobiological sequelae of severe uncontrollable stress and the pathophysiological relationship between PTSD and other anxiety disorders, such as panic disorder.

Adult↗