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PubMed1994

[Multiple sclerosis with syrinx formation in the spinal cord: a case report].

A 35-year-female developed tetraparesis following a period of back pain, urinary retension and paraparesis. She was admitted to our hospital on September 22, 1990. Neurological examination revealed tetraparesis with a sensory level at C3. Deep tendon reflexes were hyperactive in all four extremities with bilateral Hoffmann and Babinski reflexes. Spinal MR imaging demonstrated cavity formation within the cervical cord, extending from C2 to C6 without any anomalies at the craniocervical junction including Chiari malformation. The cavity was not enhanced with Gd-DTPA. Cerebrospinal fluid (CSF) examination revealed no abnormality. Although a syringo-subarachnoid shunt was performed on October 18, 1990, her symptoms did not improve. On December 17, 1990, she developed optic neuritis in the right eye and her paraparesis deteriorated. Delayed metrizamide CT scans showed another syrinx from Th3 to Th9. Repeated CSF contained 37 cells/mm3, protein 88 mg% and sugar 49 mg%. CSF oligoclonal bands were present and the CSF myelin basic protein was 6.6 ng/ml (normal < 4). A pulse therapy of steroid improved her vision and paraparesis. However, she developed paraparesis again on October 29, 1991. A brain T2-weighted MR image demonstrated multiple periventricular high signal intensity spots. Her paraparesis improved again with steroid. MR images in September, 1992 revealed a cord atrophy and disappearance of the cavity. Based on these clinical courses and radiological findings, a definite diagnosis of multiple sclerosis (MS) was made. We think MS may have caused the syringomyelia.

Adult↗
PubMed2002

Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report.

STUDY DESIGN: A case report of craniocervical spine lesions including basilar impression, atlantoaxial dislocation, and syringomyelia, with osteogenesis imperfecta is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the surgical management of craniocervical spine lesion with osteogenesis imperfecta. SUMMARY OF BACKGROUND DATA: Osteogenesis imperfecta is known to have various spine lesions as complications. However, few reports have described craniocervical lesions associated with osteogenesis imperfecta. METHODS: A 14-year-old girl with osteogenesis imperfecta, Silence classification IVB, experienced difficulty walking, with marked motor disturbance and muscle weakness in the extremities. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities, and positive Babinski reflex and ankle clonus were observed bilaterally. Basilar impression, atlantoaxial dislocation, and syringomyelia were shown by plain radiography, tomography, three-dimensional computed tomography, and magnetic resonance imaging. RESULTS: In the reported patient, posterior fossa decompression and atlantoaxial posterior fusion could not be performed because the foramen magnum and upper cervical spine invaginated to the base of the skull. Therefore, occipitocervical spine fusion using titanium loop and wires was performed at the reduced position of the atlantoaxial dislocation, resulting in improvement of neurologic deficits. CONCLUSIONS: For patients with atlantoaxial dislocation, syringomyelia, and basilar impression without clinical symptoms or signs of brain stem compression, occipitocervical spine fusion alone at the reduction of the atlantoaxial dislocation may be indicated because these procedures improve neurologic deficits and prevent postoperative development of basilar impression and enlargement of syringomyelia.

Adolescent↗
PubMedDate not supplied

[Neonatal reflexes variability in the normal full term neonate during the neurological exam].

It has been stated that findings in neonatal neurological examinations can play a role as a pointer to adverse developmental outcome. The description of the examinations, and their presence or absence differed among authors without clear reference to their physiological variability ranges. We approached the study of some neonatal behaviours and reflexes in 143 examinations made on 113 healthy newborn babies from the maternity wards by looking at the influence that perinatal environmental factors which are considered normal, can play in these examinations. The results showed that the flexion answer of the Babinski reflex increased significantly between the first and third day of life (c2= 4.4478; p= 0.03495) in the same way there was an increase in the stepping reflex (c2= 3.999; p= 0.04552) in the righting reflex (c2= 4.9342; p= 0.02633) and in the supporting reaction (c2= 11.7874; p= 0.0006). This was significantly reduced in the caesarean section deliveries (c2= 7.1209; p= 0.0076). Head reaction was only detectable during the Brazelton behavioural states 1 to 3 (c2= 3.8911; p= 0.04854) and the same thing happened with the stepping reflex (c2= 4.9370; p= 0.02629). We discuss the utility of neonatal reflexes scoring scales in predicting the neurodevelopmental outcome of the newborn baby.

Female↗
PubMed1990

Neurological phenomena during emergence from enflurane or isoflurane anaesthesia.

During emergence from anaesthesia, transient neurological signs that would usually be considered pathological may appear. The objective of this randomized, patient (n = 30) and observer-blinded study was to compare prospectively the incidence and duration of post-anaesthetic neurological abnormalities in healthy patients undergoing minor elective procedures following thiopentone and succinylcholine induction, and enflurane-N2O or isoflurane-N2O anaesthesia. Patients were studied for 60 min after anaesthesia. Arousal state, muscle tone, deep tendon reflexes, plantar reflex, sustained clonus, shivering, intense muscular spasticity and temperature were assessed. Results of neurological examination were correlated with the patient's state of arousal. Transient emergent neurological abnormalities occurred more frequently following enflurane-N2O anaesthesia than isoflurane N2O anaesthesia. This was statistically significant (P less than 0.05) for quadriceps hyperreflexia, upgoing toes (positive Babinski reflex) and intense muscular spasticity. Neurological abnormalities occurred most commonly 5-20 min after anaesthesia and all abnormalities resolved within 60 min. Following enflurane anaesthesia, as patients became more alert the incidence of abnormalities declined, while the arousal state did not affect the incidence of abnormalities after isoflurane. There was no significant difference between axillary temperatures of those patients who shivered and those who did not. In conclusion, temporary emergent neurological abnormalities occurred more often following enflurane-N2O than after isoflurane-N2O anaesthesia.

Adult↗
PubMed1994

Mechanisms of tizanidine action on spasticity.

This investigation estimated the mechanisms of tizanidine action on spasticity using a battery of neurophysiological methods. Thirty patients with old post-stroke spastic hemiparesis took part in the investigation. They were treated with tizanidine-mean daily dose 15.8 +/- 5.6 mg for a mean of 23.3 +/- 4.8 days. A questionnaire for assessment of subjective improvement after treatment used a 5-point scale. For standardization of the neurological examination 5-point scales were used to assess muscle tone, muscle force and tendon reflexes. A battery of neurophysiological methods was used to analyze different mechanisms of spasticity: for alpha motoneuron excitability--the F wave parameters; for presynaptic inhibition--the ratio of H reflex amplitudes before and after vibration of the achilles tendon (Hvibr/Hmax); for common interneuron activity--the flexor reflex parameters. Our results revealed that tizanidine reduces spastically increased muscle tone, but has no influence on muscle force, tendon reflexes, Babinski sign and ankle clonus. Tizanidine is supposed to act by increasing the presynaptic inhibition and decreasing of alpha motoneuron excitability. When spasticity has decreased presynaptic inhibition and increased motoneuron excitability, it is better to treat with tizanidine.

Adult↗
PubMed1997

Multidimensional assessment of motor function in a child with cerebral palsy following intrathecal administration of baclofen.

This case report describes an 11-year-old boy with spastic diplegia whose reflex status, range of motion (ROM), strength, and motor performance were measured before and after implantation of an indwelling system for delivery of intrathecally administered baclofen. Before baclofen use, the subject experienced clonus that interfered with walking, needed assistance with transfers, and was unable to independently put on underwear and socks. Measures of spasticity, kinematics and electromyographic activity during voluntary movements, ROM, Gross Motor Function Measure (GMFM) scores, and self-reports of change were obtained at baseline, before and after bolus baclofen injection, during a double-blind placebo-controlled clinical trial of baclofen administration via an indwelling pump, and after 1 and 2 years of baclofen therapy. Spasticity, Babinski reflexes, clonus, strength, and coactivation of antagonist muscles during voluntary movement were decreased shortly after baclofen administration began. Hip and ankle ROM increased, upper-extremity movement speed increased, independence in dressing and transfers improved, and orthoses were discarded. After 1 and 2 years, GMFM scores were 7.8% and 6.4% above baseline, respectively; the subject won a fitness award. After 2 years, ROM was worse than at baseline and concerns regarding hip subluxation arose. Single-joint movement control and independence improved and spasticity decreased during baclofen administration.

Activities of Daily Living↗
PubMed2002

Investigations of micro-organic brain damage (MOBD) in heterozygotes of metachromatic leukodystrophy.

Potential damage of central and peripheral nervous system expressed as micro-organic brain damage (MOBD) was investigated in 27 unrelated heterozygotes with metachromatic leukodystrophy (MLD). Arylsulfatase A (ARSA) was determined in peripheral blood leukocytes and sulfatide excretion was estimated in 24-hour urine collections. Genomic DNA was analyzed for the ARSA pseudodeficiency (PD) allele by a PCR method. Clinical investigations included examination of hyper-reflexia, Babinski reflex, Wechsler Adult Intelligence Scale, Benton test, evoked potentials, and nerve conduction velocity (NCV). In our study, a higher incidence of evident or possible micro-organic brain damage was observed in true MLD/PD and MLD heterozygotes (NO/MLD, where NO means the wild allele) than in controls. On the basis of the Benton test, MOBD was suggested or indicated in 67% of MLD heterozygotes, 50% of MLD/PD heterozygotes, and 26% of controls. In our small group of carriers with MLD and PD mutations, persons NO/MLD(PD) with one wild-type allele did not show MOBD and displayed higher ARSA/beta-galactosidase ratios, unlike true MLD/PD compound heterozygotes who carry MLD-causing mutation in one allele and the ARSA-PD polymorphism in the second. Theoretically, this is a shift from autosomal recessive to autosomal dominant-like inheritance, especially when one cannot exclude the influence of polymorphisms (like ARSA-PD) in the wild allele. Since all psychological tests were age-matched, it can be assumed that the MOBD observed in MLD carriers does not have a progressive character unlike in MLD patients. However, it should be mentioned that MOBD appears to have no overt clinical consequences.

Adult↗
PubMed1995

Spinocerebellar degeneration and cerebral hypomyelination in a family.

The proband is a 24-year-old woman who developed symptoms of a spinocerebellar degeneration in early childhood. Neurological examination revealed normal cognitive function, optic atrophy, dysarthria, titubation, action tremors, increased deep tendon reflexes, Babinski's signs, and a spastic scissoring gait. The magnetic resonance imaging (MRI) showed an abnormal increased signal on long TR images involving white matter throughout the cerebral hemispheres, most striking in the subcortical white matter, and to a lesser degree in the brainstem, compatible with diffuse hypomyelinating or dysmyelinating diseases. Metabolic and chromosomal studies were normal. Her 49-year-old mother developed similar symptoms in her 20s and is now wheelchair-bound. Findings on neurological examination and MRI were similar to her daughter but more severe. The proband's maternal grandfather had a female cousin who had a neurological illness beginning in her 20s with similar symptoms and signs and died at the age of 44 years. Spinocerebellar degenerations are a group of syndromes with similar clinical manifestations but heterogeneous etiology. We report a family with spinocerebellar degeneration with distinct MRI findings compatible with hypomyelination or dysmyelination which has not heretofore been described. This family may represent a new spinocerebellar syndrome due to an abnormality of as yet an undetermined gene.

Adult↗
PubMed2006

Human T-cell lymphotropic virus type I (HTLV-I) confirmed by PCR-Southern blot and sequencing analysis in a woman with tropical spastic paraparesis.

Human T-cell lymphotropic virus type I (HTLV-I) is a human retrovirus and the aetiological agent of a progressive neurological disease called tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM), as confirmed by evidence accumulated in HTLV-I seroprevalence studies. TSP/HAM is rarely diagnosed in Italy, given the low prevalence of HTLV-I in the population. TSP/HAM begins insidiously in the fourth decade, mainly with spastic paraparesis of the lower extremities and positive Babinski reflex, as well as interfering with bowel and bladder functions. In this study we report the clinical, virological and haemato chemical data of a 54-year-old woman, born in the Ivory Cost, with symptoms suggestive of TSP. The presence of HTLV-I infection was demonstrated by the detection of antibodies in serum and in cerebrospinal fluid by immunoenzymatic assay and Western blot analysis. In addition, viral isolation was carried out in peripheral blood cells, and the presence of HTLV-I proviral DNA was confirmed by polymerase chain reaction/Southern blot and sequencing analysis. According to our results, HTLV-I testing might be useful when TSP/HAM is suspected.

Blotting, Southern↗
PubMed1992

Spinal cord syphilis associated with human immunodeficiency virus infection: a treatable myelopathy.

A 33-year-old woman, seropositive for human immunodeficiency virus type 1 (HIV-1), presented with progressive weakness and numbness of the lower extremities, gait difficulties, and urinary frequency. Physical examination revealed bilateral lower extremity weakness, a left-sided Babinski reflex, and a thoracic sensory level to pinprick at T8. Serum rapid plasma reagin was 1:64, and fluorescent treponemal antibody-absorption (FTA-ABS) was 4+. Examination of the cerebrospinal fluid showed a mononuclear pleocytosis and reactive FTA-ABS. The myelopathy responded promptly to high-dose intravenous aqueous penicillin. Syphilis needs to be considered in the differential diagnosis of any patient who develops a myelopathy in association with HIV-1 infection. Because of the diverse nature in which syphilis may affect the spinal cord, treatment with intravenous aqueous penicillin, 12 to 24 million units daily, for a minimum of 10 days, should be considered in any HIV-1-seropositive patient with a progressive, unexplained myelopathy and positive serologic studies for syphilis.

Adult↗
PubMed1993

Hereditary spastic paraparesis. Clinical and genetic data from a large Dutch family.

A large Dutch family is presented with pure hereditary spastic paraparesis. Pedigree analysis indicates autosomal dominant inheritance with complete penetrance and possibly anticipation in successive generations. These observations may have implications for genetic studies. Linkage studies in this family have excluded more than 40% of the autosomal genome.

Central Nervous System Diseases↗
PubMed1993

A prospective study to identify high-yield criteria associated with acute intracranial computed tomography findings in head-injured patients.

We conducted a multicenter, prospective study of head-injured patients to identify high-yield clinical criteria for acute intracranial injuries. Emergency patients with a history of blunt head trauma occurring within 2 weeks and who underwent nonenhanced cranial computed tomography (CT) were entered onto the study during a 12-month period. Of the 264 patients, 32 (12%) had abnormal CT findings. Nine high-yield variables were associated with abnormal CT findings: alcohol use before injury, antegrade amnesia, prolonged loss of consciousness, anisocoria and/or fixed and dilated pupils, abnormal Babinski reflex, focal motor paralysis, cranial nerve deficit, Glasgow coma scale score of less than 15, and clinical signs of basilar skull fracture. Patients 2 years old or younger or older than 60 years of age showed a significantly greater prevalence of abnormal CT findings than patients of other ages.

Acute Disease↗
PubMed1998

[Endoscopic projectile extraction in treatment of a gunshot injury to the head].

HISTORY AND CLINICAL FINDINGS: A 14-year-old boy had in war-torn Bosnia sustained a transcranial gunshot wound from a 7.65 mm bullet. After primary medical care with craniotomy and the removal of bony fragments and cerebral debris followed by a duraplasty, he was transported to the French-German Field Hospital. On arrival he was breathing spontaneously and in stable cardiovascular state but with impaired responsiveness and somnolent. His pupils were moderately dilated with slight anisocoria (right > left). His gaze was deviated to the left and he had vertical gaze paralysis as well as right central facial nerve paresis. In addition he had a mild diencephalic syndrome, right hemiplegia and a right hemihypaesthesia with increased muscle tone, especially of the leg, paratonia and right positive Babinski reflex. There also was a marked ciliospinal reflex and he had a bulbar speech as well as cognitive and memory abnormalities. INVESTIGATIONS: Haemoglobin and haematocrit were below normal (12.1 g/dl and 35.0%, respectively), while biochemical tests were normal. Cranial computed tomography localized the bullet in the pineal recess of the 3rd ventricle and the lamina quadrigemina. DIAGNOSIS, TREATMENT AND COURSE: These findings indicated endoscopic transcranial removal of the bullet, achieved with a rigid endoscope and forceps along the entry track. Subsequent intensive care proceeded without complication. On discharge the boy was normal oriented and ready to make contact. The neurological defects were regressing. CONCLUSION: Endoscopic transcranial removal of a bullet wedged in the brain is a relatively sparing neurological procedure which, under unusual circumstances and conditions, can achieve a satisfactory result even with limited facilities.

Adolescent↗
PubMed1995

[Lacunar cerebral infarction in a young woman].

HISTORY AND FINDINGS: A 29-year-old woman was admitted to hospital with an acute right-sided hemiplegia and sensory disorders, as well as upper right quadrant anopsia. There were no other significant abnormalities. She had previously been healthy and was free of any predisposing risk factors for thromboembolism. Neurological examination elicited a homonymous right upper quadrant hemianopsia, dysesthesia of the right half of the face and hypesthesia and hypalgesia of the right side of the body. In addition there was paresis of the right arm and a positive right Babinski reflex. INVESTIGATIONS: There was no evidence for any underlying haematological, metabolic, infectious or vascular disease. Computed tomography of the head revealed a small hypodense area immediately adjacent to the posterior part of the left internal capsule, compatible with a lacunar infarction, a finding confirmed by magnetic resonance imaging and relating to the area supplied by the thalamic branch of the posterior cerebral artery. Transoesophageal echocardiography demonstrated a patent foramen ovale. TREATMENT AND COURSE: Almost complete regression of all signs occurred within two months on anticoagulation with heparin intravenously for two weeks followed by oral phenprocoumon (Quick's value 30-40%) and intensive physiotherapy. Five weeks after onset of treatment the paresis was obviously regressing and pyramidal tract signs had disappeared. Sensitivity to touch over the right half of the body was still diminished and the homonymous paracentral scotoma still present. CONCLUSION: Lacunar infarction of the brain in young patients has an excellent prognosis, as long as it is treated intensively according to its cause.

Acute Disease↗
PubMed1981

Neurologic changes during awakening from anesthesia.

Transient hyperreflexia, the Babinski reflex, and other transient neurologic signs may follow anesthesia in healthy persons. The authors describe the appearance and durations of these signs and relate them to anesthetic agent, time following cessation of anesthesia, and state of arousal. Twenty-nine neurologically normal male patients undergoing superficial operations were anesthetized with halothane-nitrous oxide, enflurane-nitrous oxide, or nitrous oxide-narcotic. During awakening, patients experienced transient hyperreflexia and shivering following all anesthetic techniques. Quadriceps hyperreflexia occurred in three of eight patients after halothane-N2O, seven of 12 patients after enflurane-N2O, and no patient after n2O-narcotic anesthesia. Sustained ankle clonus and upgoing plantar responses were observed most frequently following enflurane-N2O (50 percent of patients). Following halothane-N2O, ankle clonus was observed in 12.5 per cent of patients and upgoing plantar responses in 25 per cent of patients. Neither sign occurred after N2O-narcotic anesthesia. Shivering, too, was observed most frequently following enflurane anesthesia (66 per cent of patients). There were equal reductions of body temperature in shivering and nonshivering patients. In every patient, regardless of the anesthetic used, the lash reflex and the pupillary response to light returned to normal with the return of consciousness as measured by the response to verbal commands. It is concluded that the neurologic profile during awakening from general anesthesia is most abnormal following enflurane-N2O, compared with halothane-N2O and N2O-narcotic anesthesia. Abnormalities are found most frequently when patients are poorly responsive to verbal commands. Although their incidence diminishes with time, abnormal responses to neurologic examination may persist for 40 min or more after cessation of anesthesia.

Adult↗