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PubMed1979

Clinical and electrophysiological studies of diphtheritic neuritis in Jordan.

Eleven patients with acute diphtheritic neuritis were studied clinically and electrophysiologically. Decreased conduction velocity and increased distal motor latency were present in most patients within two weeks of the onset of neurological symptoms. Velocities later fell to approximately 45% of mean normal values. Wasting with electrophysiological evidence of denervation was present in 3 patients. Nerve conduction studies had returned to normal within 3 months in 8 patients. There was a striking dissociation between the time course of the clinical and the neurophysiological abnormalities: early in the illness, peripheral nerve conduction was normal in some patients despite the presence of severe weakness, and later, the maximum electrophysiological abnormalities were sometimes found after clinical recovery had commenced. The nature and sequence of the clinical and electrophysiological changes are in accord with the known pattern and distribution of the pathological changes in the disease.

Adolescent↗

PubMed1981

Fisher syndrome in childhood.

Three children, age 2, 7, and 12, developed Fisher syndrome: inability to walk because of ataxia, complete areflexia, and ophthalmoplegia. Ptosis was prominent in all the children, but the pupillary response to light was affected only in one child. Limb weakness was never present, and sensation normal. One patient was obtunded for several days. Two had prodromal upper respiratory tract illnesses, and the third patient was bitten by an insect 2 days before her symptoms began. Cerebrospinal fluid (CSF) protein content was moderately increased in all patients, but only one child's CSF had a pleocytosis. CSF gamma globulin levels were normal. In the acute phase, F waves and H responses were absent in two cases but returned to normal with clinical recovery. All three patients had marked electroencephalographic (EEG) abnormalities that later improved. The EEG pattern suggested a brainstem disorder. These findings plus the obtundation, gaze paralysis, and ataxia indicate that in Fisher syndrome there is parenchymal involvement of the central nervous system with or without nerve root involvement.

Ataxia↗

PubMed2000

Miller Fisher syndrome: axonal, demyelinating or both?

Controversy exists concerning whether Miller Fisher syndrome (MFS) is the result of a predominantly axonal or demyelinating polyneuropathy and whether the Guillain-Barré syndrome variant of acute ataxia and areflexia without ophthalmoplegia, ataxic Guillain-Barré syndrome (atxGBS), has a distinct pathophysiology. We explored these issues by reviewing the electrophysiologic features of 6 patients with MFS and 2 patients with atxGBS. EMG laboratory records were reviewed and electrophysiologic findings were categorized as axonal or demyelinating neuropathy using previously defined criteria. Of the 6 patients with MFS, 5 had electrophysiologic evidence suggestive of an axonal, predominantly sensory polyneuropathy; only 1 patient met criteria for demyelinating polyneuropathy. Both patients with atxGBS had demyelinating sensorimotor polyneuropathy. Electrophysiologic abnormalities in MFS typically suggest a predominantly axonal, sensory polyneuropathy, though demyelinating forms occur and may be under-diagnosed using current criteria. AtxGBS, in our experience, is a predominantly demyelinating polyneuropathy.

Adult↗

PubMed1990

Micturitional disturbance in cervical spondylotic myelopathy.

Neurourological studies were performed on 37 patients with cervical spondylotic myelopathy. Micturitional history revealed that 30 patients (81%) had voiding symptoms: obstructive in 14, irritative in 5, and both symptoms in 11. Urodynamic studies revealed that 9 of 20 had abnormal uroflowmetrogram, 17 of 37 had residual urine, 14 of 37 had detrusor hyperreflexia, and 6 of 37 had detrusor-sphincter dyssynergia. There were no statistical differences in lower extremity neurological signs in patients with or without residual urine.

Adult↗

PubMed1994

Hepatic encephalopathy with increased cerebral blood flow in SPECT and MRI abnormalities in the basal ganglia.

A case of hepatic encephalopathy with increased cerebral blood flow in single-photon emission computed tomography (SPECT) and abnormalities upon magnetic resonance imaging (MRI) is reported. SPECT showed increased blood flow exceeding 70 ml/100 g/min in the cerebellum, basal ganglia and cerebral cortex, with hyperammonemia and abnormal electroencephalographic signs showing severe generalized slowing. SPECT data and MRI might reflect abnormal metabolism and neuropathological changes in hepatic encephalopathy.

Antiparkinson Agents↗

PubMed1997

Comparison of neurologic deficits with atlanto-dens intervals in patients with Down syndrome.

Eighty-four patients with Down syndrome had flexion-extension lateral roentgenograms of the C1-C2 articulation for the purpose of dividing the group into subluxators (> or = 4 mm atlanto-dens interval and 2 mm translation) and nonsubluxators (those who did not meet these criteria). Neurologic examinations and chart review were carried out on all patients to ascertain those with a positive neurologic finding or history. Seventeen (20%) were defined as subluxators and 67 (80%) as nonsubluxators. Five (29%) of the subluxators were found to have positive neurologic findings and 18 (27%) of the nonsubluxators had similar types of positive neurologic findings. These percentages are not significantly different. This led us to conclude that positive neurologic findings and an abnormal atlanto-dens interval are not alone adequate criteria to judge need for surgical stabilization of the C1-C2 articulation in patients with Down syndrome.

Adolescent↗

PubMed2002

Functional Neuromuscular Stimulation for articular angle control with an Inverse Dynamics Model tuned by a neural network.

A Functional Neuromuscular Stimulation (FNS) system was developed to restore the motor function of people with central nervous system disorders. An FNS articular angle control system with an Inverse Dynamics Model (IDM) tuned by a neural network was proposed. The system was designed to control the elbow joint angles by learning the characteristics of the neuromuscular system of controlled limbs. Three kinds of control schemes using the IDM were proposed. In a simulation experiment, the system was able to learn the abnormal characteristics of a limb and any changes in these characteristics, and then allow for better control of the limb. The results of experiments using human participants showed that the system had ability to control both normal and paralysed limbs.

Biophysical Phenomena↗

PubMed1994

Abnormal classical eye-blink conditioning in autism.

Cerebellar and limbic system pathologies have been reported in persons with autism. Because these brain areas are involved centrally in the acquisition and performance in classical eye-blink conditioning, this study evaluated conditioning in 11 persons with autism. Compared to matched controls, persons with autism learned the task faster but performed short-latency, high-amplitude conditioned responses. In addition, differences in learning the extinction rates systematically varied with age thus suggesting a developmental conditioning abnormality in autism. The observed pattern of eye-blink conditioning may indicate that persons with autism have the ability to rapidly associate paired stimuli but, depending on processing of certain contextual information, have impairments in modulating the timing and topography of the learned responses. This abnormality may relate to deviant cerebellar-hippocampal interactions. The classical eye-blink conditioning paradigm may provide a useful model for understanding the biological and behavioral bases of autism.

Adolescent↗

PubMed1991

Manometric and radiologic investigations and biofeedback treatment of chronic idiopathic anal pain.

In 12 patients suffering from chronic idiopathic anal pain, the rectosphincteric function was studied using manometric and x-ray techniques. The results of manometric investigations were compared with those obtained in 12 healthy volunteers. In all patients, the resting pressure in the anal canal was significantly higher than in control subjects. In 10 patients, defecography revealed abnormalities of the pelvic muscles. We treated the patients by using biofeedback techniques, consisting of voluntary modifications of the state of contraction of the external sphincter. In all cases, pain disappeared after a mean of eight biofeedback training sessions. When noxious manifestations had disappeared, manometry showed a significant decrease in the anal canal resting pressure. Our results indicate 1) that chronic idiopathic anal pain is associated with abnormal anorectal manometric profiles, probably resulting from a dysfunctioning of the striated external anal sphincter, and 2) that biofeedback training is an effective treatment for chronic idiopathic anal pain.

Adult↗

PubMed1990

Micturitional disturbance in ossification of the posterior longitudinal ligament in the cervical spine.

Neurourological studies were performed on 39 patients with cervical myelopathy due to ossification of the posterior longitudinal ligament. Micturitional history revealed that 30 patients (77%) had voiding symptoms: obstructive in 8, irritative in 11, and both obstructive and irritative symptoms in 11. Ten patients had urge incontinence and 4 had urinary retention. Urodynamic studies revealed that 7 of 19 had abnormal uroflowmetrogram, 16 of 35 had residual urine, 18 of 39 had detrusor hyperreflexia, and 10 of 39 had detrusor-sphincter dyssynergia. There were no statistical differences in lower extremity neurological signs in patients with incontinence, and patients with residual urine or urinary retention.

Calcinosis↗

PubMed1988

The usefulness of head-up tilt testing and hemodynamic investigations in the workup of syncope of unknown origin.

To enhance the clinical evaluation of patients suffering from recurrent syncope of unknown origin, the integrity of mechanisms controlling blood pressure was examined in 151 patients utilizing a screening tilt test. Ninety-eight patients had an abnormal blood pressure and/or heart rate response to tilt testing, including provoked syncopal attacks in 63 patients. Whenever indicated, the screening tilt test was followed by blood volume and hemodynamic determinations, as well as autonomic nervous system testing to identify contributing pathophysiological abnormalities (hypovolemia, venous pooling, autonomic dysfunction). Detailed analysis of this battery of tests allowed us to conclude that: (1) The tilt test is commonly a provocative tool in the workup of patients with recurrent syncope due to vasovagal - vasodepressor reactions and other abnormalities of blood pressure regulation; (2) Its usefulness is augmented by associated hemodynamic and blood volume evaluations; (3) The identification of contributory pathophysiological mechanisms of blood pressure control facilitates specific therapeutic interventions.

Autonomic Nervous System Diseases↗

PubMed1989

The Auto-Tilt Test in patients with positional vertigo and Menière's disease.

As a new method of testing the righting reflex, which has been considered to be closely related to the otolith function, we used the Auto-Tilt Test (ATT) on 24 patients with positional vertigo, on 5 patients with Meniere's disease before and after cochlearendolymphatic shunt, and on 10 normal subjects. The subject is required to stand on an electrically driven tilt board with an autoregulating switch in hand, and to perform bilateral tilt swings of 5 degrees and 10 degrees wearing a helmet with a Head Inclinometer or tilt sensor. The result of this test in patients with positional vertigo revealed the patients had an abnormal labyrinthine reflex and significantly excessive tilt in the return swing from the side of the lesion. In addition, we consistently observed an inaccurate righting reflex, coupled with a diminished sensation of tilt, on the side of the lesion. The results in Meniere's disease patients, followed up before and after surgery, showed that the depressed righting reflex toward the affected side and the tendency to list to the healthy side before surgery, improved after surgery.

Adult↗

PubMed1989

An analysis of factors predisposing to neurological injury in patients undergoing coronary bypass operations.

In a prospective study of 312 patients undergoing elective coronary bypass surgery we evaluated 50 preoperative, intraoperative and postoperative factors with the aim of identifying predisposing causes for perioperative neurological morbidity. Factors which showed a significant association with the development of neurological complications included the duration and severity of heart disease before surgery; the presence of extracoronary vascular disease; history of cardiac failure; history of diabetes; difficulty in terminating bypass; intraoperative mean arterial pressure levels of less than 40 mmHg; a large drop in haemoglobin level during surgery; prolonged stay in the intensive therapy unit after operation; and abnormalities of blood pressure control in the postoperative period. The significance of these findings is discussed and a comparison made with data available from previous studies.

Adult↗

PubMed1997

Developments in our understanding of detrusor instability.

It is important to recognize that our understanding of detrusor instability is far from complete. Although the significance of some of the abnormalities reported is unclear, it is likely that several factors will eventually be found to contribute to the overall clinical picture. Nevertheless, there is increasing evidence that the development of post-junctional supersensitivity of the detrusor muscle is implicated in each of the clinical subgroups of instability. Partial denervation of the detrusor appears to be the mechanism responsible for this in obstructive detrusor instability, but other abnormalities causing a chronic decrease in the excitatory motor innervation to the detrusor (e.g. decentralization, pre-synaptic inhibition) could produce similar effects and may therefore be involved. This evidence of a specific abnormality at the cellular level offers scope for therapy for detrusor instability with a new range of drugs, using agents that hyperpolarize the detrusor muscle membrane or modify calcium activation. However, bladder specificity remains a fundamental problem in the development of effective therapeutic agents for detrusor instability and poses the great challenge for the future.

Animals↗

PubMed2006

Anorectal functions in patients with lumbosacral spinal cord injury.

OBJECTIVE: To investigate the anorectal status in patients with lumbosacral spinal cord injury (SCI). METHODS: Twenty six patients (23 males, 3 females) with lumbosacral SCI and 13 normal volunteers were enrolled into this study as controls. The median age was 43.7 years (ranging 17-68 years) and the median time of patients since injury was 59.1 months (ranging 8 months-15 years). They were diagnosed as complete lumbosacral SCI (n =2, American Spinal Injury Association (ASIA) score A), or incomplete lumbosacral SCI (n=24, ASIA score B-D) with mixed symptoms of constipation and/or fecal incontinence, and were studied by anorectal manometry. None of the patients had any medical treatments for neurogenic bowel prior to this study. RESULTS: The maximum anal resting pressure in lumbosacral SCI patients group was slightly lower than that in control group (One-way ANOVA: P=0.939). During defecatory maneuvers, 23 of 26 (88.5%) patients with lumbosacral SCI and 1 of 13 (7.7%) in the control group showed pelvic floor dysfunction (PFD) (Fisher's exact test: P<0.0001). Rectoanal inhibitory reflex (RAIR) was identified in both patients with lumbosacral SCI and the controls. The rectal volume for sustained relaxation of the anal sphincter tone in lumbosacral SCI patients group was significantly higher than that in the control group (Independent-Samples t test: P<0.0001). The mean rectal volume to generate the first sensation was 92.7 ml+/-57.1 ml in SCI patients, 41.5 ml+/-13.4 ml in the control group (Independent-Samples t test: P<0.0001). CONCLUSIONS: Most of the patients with lumbosacral SCI show PFD during defecatory maneuvers and their rectal sensation functions are severely damaged. Some patients exhibit abnormal cough reflex. Anorectal manometry may be helpful to find the unidentified supraconal lesions. RAIR may be modulated by central nervous system (CNS).

Adolescent↗

PubMed2005

Food-cobalamin malabsorption in elderly patients: clinical manifestations and treatment.

PURPOSE: Approximately 15% of people aged more than 60 years old have a cobalamin (vitamin B12) deficiency, mainly in relation with food-cobalamin malabsorption (FCM). To date, no study has documented this disorder in the elderly. There is also little information on clinical consequences. SUBJECTS AND METHODS: We studied 92 elderly patients with well-established FCM who were extracted from an observational cohort study (1995-2004) of 172 consecutive elderly patients with documented cobalamin deficiency. RESULTS: The median patient age was 76 +/- 8 years; 60 patients were women. The most common clinical manifestations were neurologic or psychologic: mild sensory polyneuropathy (44.6%), confusion or impaired mental functioning (22.8%), and physical asthenia (20.7%). Hematologic abnormalities were reported in at least one third of the patients: anemia (21%), leukopenia (10.9%), thrombopenia (8.7%), and pancytopenia (6.5%). All patients had low serum vitamin B12 levels (<200 pg/mL), with a mean value (+/- standard deviation) of 131 +/- 38 pg/mL and total serum homocysteine level of 22.1 +/- 9.3 micromol/L. The mean hemoglobin level was 10.9 +/- 2.5 g/dL and the mean erythrocyte cell volume 95.7 +/- 12.7 fL. Correction of the serum vitamin B12 levels and hematologic abnormalities was achieved equally well in patients treated with either intramuscular or oral crystalline cyanocobalamin. CONCLUSIONS: This study suggests that in elderly patients, FCM may be associated with significant neurologic, psychologic, and hematologic abnormalities, which seem to respond equally well to either oral or parenteral vitamin B12 therapy.

Aged↗

PubMed2006

Early clinical signs and imaging findings in Gerstmann-Sträussler-Scheinker syndrome (Pro102Leu).

OBJECTIVE: To determine the clinical and radiologic features of Gerstmann-Sträussler-Scheinker syndrome caused by Pro102Leu mutation in PRNP (GSS102). METHODS: The authors report 11 patients (nine families) with clinically and radiologically diagnosed GSS102. RESULTS: All patients showed mild gait disturbance, dysesthesia and hyporeflexia of the lower legs, and truncal ataxia, and 9 of 11 patients showed proximal leg muscle weakness during the early stage of the disease. Dementia was not a main symptom during the early stage. Brain MRI and EEG abnormalities were not prominent initially. SPECT (N-isopropyl-p-[(123)I]iodoamphetamine) analyzed by the three-dimensional stereotactic surface projection (SSP) method detected abnormalities in five patients early during the course of the illness. SPECT findings showed diffusely decreased cerebral blood flow, demonstrated by a mosaic pattern, with the lowest perfusion noted in the occipital lobes. In contrast, blood flow to the cerebellum was preserved. These studies suggested sites of pathology in GSS102, with the main lesions probably located in the cerebrum and the spinal cord (posterior horn and spinocerebellar tract) instead of the cerebellum. CONCLUSIONS: Key features for early diagnosis of Gerstmann-Sträussler-Scheinker syndrome caused by Pro102Leu mutation in PRNP (GSS102) are truncal ataxia, dysesthesia and hyporeflexia of the lower legs, and mild dysarthria. Normal cerebellar MRI and abnormal cerebral SPECT findings are characters of early GSS102.

Amyloid↗

PubMed1982

Heredofamilial syndrome of spastic paraplegia, dysarthria and cutaneous lesions in ive siblings.

Five children, three sisters and two brothers aged between three months and 12 years, are described. They all developed a facial desquamating rash of butterfly distribution at the age of about two months, and motor retardation which later was characterized by spasticity, predominantly affecting the lower limbs. The three children who were old enough for speech to be tested had dysarthria. There was no family history of neurological disease, nor was there consanguinity among the parents or grandparents. EEGs were diffusely abnormal in four of the five children, but did not show any specific or diagnostic features. Plasma immunoglobulin tests were normal, and tests for collagen disease were negative. The authors are not aware of previous reports of this condition, but believe that it is a variant of familial spastic paraplegia, with atypical features.

Child↗