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The epiconus syndrome presenting with radicular-type neurological features.

We report 15 patients with an epiconus syndrome presenting with radicular-type clinical features involving the lower extremities and identify the vertebral level at which the spinal cord terminated. All patients, consisting of 10 men and five women, aged 37 to 71 years, presented with unilateral or bilateral leg muscle atrophy as well as sensory deficit. Unilateral or bilateral reduction or absence of deep tendon reflexes were present in 13 patients, while pathological reflexes were seen in three cases and bladder dysfunction in 10. Prior to presentation, five patients with the epiconus syndrome were mistakingly diagnosed as having different spinal diseases, which were treated surgically. The level of epiconus compromise detected on radiological examination ranged from the lower level of the T10 vertebra to T12-L1 intervertebral disc level, but most frequently at T11-12 disc level. The level of termination of the spinal cord was between T12 and L1-2 disc level, but most frequently at the middle portion of the L1 vertebra, which was on average 1.6 +/- 0.4 (mean +/- standard deviation) vertebrae distal to the epiconus lesions. The presence of different neurological features related to epiconus compromise should be carefully examined with reference to the level of termination of the spinal cord.

Adult↗

Ischaemic myelopathy following aortic surgery or traumatic laceration of the aorta.

Paraplegia is one of the major complications following repair of aortic aneurysms or congenital malformations and from trauma of the aorta. In a series of 12 surviving patients we describe the clinical features as well as the evolution and pathophysiology of ischaemic lesions of the spinal cord. The clinical characteristics: loss of tendon reflexes, preservation of light touch sensation and bladder function, and the special topography of pin prick impairment, suggest involvement of the central grey matter. This lesion of the grey matter is incomplete in most of the patients and tends to extend for 2-10 segments. In some cases it can extend downward to the conus resulting in complete flaccid paraplegia. On follow-up we have observed limited improvement in most cases. No patient has recovered fully. Except in cases of traumatic laceration, where symptoms existed before surgery, paraplegia followed surgical repair in all other cases. Ischaemia can be related to the duration and the site of crossclamping of the aorta. Clamping above the left subclavian artery and/or a ligation of the intercostal arteries without previous visualisation of the spinal cord arteries can be dangerous. Other factors such as the phenomena of revascularisation and the presence of free radicals are discussed. These could explain delayed postischaemic spinal cord hypoperfusion.

Adolescent↗

Early autonomic dysreflexia.

INTRODUCTION: During the stage of spinal shock the conventional view is that autonomic activity is abolished. Here, evidence is presented that autonomic activity is still present. PATIENTS: Four patients with acute cord transactions are presented: one new case and three from the literature. DEFINITIONS: The definitions of spinal shock and autonomic dysreflexia are given. METHODS: All four cases showed acute autonomic dysreflexia between 7 and 31 days after acute cord transection at a stage when the tendon reflexes were abolished. RESULTS: Two cases showed a severe rise in blood pressure; the two earlier cases, before blood pressure was routinely recorded, profuse sweating. In two cases autonomic dysreflexia was obtained when the bladder was overdistended with 1000 ml and 1600 ml. In the other two cases it occurred in response to traumatic catheterisation. This was found when supramaximal stimuli were applied. It has not been recorded routinely as, with modern management, the bladder does not get overdistended or traumatised. DISCUSSION: Other evidence, the blood pressure, and urethral tone is presented to show that sympathetic reflex activity of the cord is not abolished during spinal shock. CLINICAL SIGNIFICANCE: The clinical importance of this is that autonomic dysreflexia can be seen at an early stage and it should be considered in the differential diagnosis of a sick patient immediately after spinal injury.

Autonomic Dysreflexia↗

Intrathecal baclofen in tetraplegia of spinal origin: efficacy for upper extremity hypertonia.

STUDY DESIGN: Retrospective analysis. OBJECTIVES: To evaluate the efficacy of intrathecal baclofen (ITB) for upper extremity spastic hypertonia in tetraplegia of spinal origin. SETTING: University of Alabama at Birmingham hospital. METHODS: The medical records of 14 individuals with tetraplegia of spinal origin who underwent intrathecal baclofen pump placement were reviewed. The effects of intrathecal baclofen on spasm frequency, deep tendon reflexes, and tone (Ashworth scale) were assessed for the upper and lower extremities for a 1-year follow-up period. RESULTS: There were statistically significant declines in upper extremity spasm scores (1.8 points, P=0.012), reflex scores (1.4 points, P<0.0001) and Ashworth scores (0.6 points, P<0.0001) for the 1-year follow-up period. For the lower extremities, all decreases were significant (P<0.0001). There was also a statistically significant (P<0.0001) increase in intrathecal baclofen dosage requirements during the 1-year follow-up period to maintain the reductions in spasm frequency, reflexes and tone. CONCLUSIONS: Intrathecal baclofen is a safe and effective intervention for treating upper extremity hypertonia of spinal origin. In addition, the level of intrathecal catheter placement is felt to be of importance.

Adult↗

Effects of thyroid hormones on contractility and cation transport in skeletal muscle.

Skeletal muscle is one of the major target organs for thyroid hormone. The muscles most commonly affected are those used during prolonged effort (slow-twitch muscles). One of the major clinical features is the shortening of the Achilles-tendon reflex time in hyperthyroidism and its prolongation in hypothyroidism. Most of the peripheral effects of the thyroid hormones can be ascribed to the action of triiodothyronine (T2), which is produced by de-iodination of thyroxine (T4) in liver and kidney. From the plasma, T3 is actively transported into skeletal muscle. The Ca2+ ATPase in skeletal muscle is responsible for removal of Ca2+ ions from the cytosol into the sarcoplasmic reticulum (SR) during relaxation, and the Na+, K+ ATPase in the plasma membrane is responsible for restoration of the membrane potential after excitation. The concentrations of Ca2+ ATPase and Na+, K+ ATPase in rat skeletal muscle have been shown to increase four- and 10-fold, respectively, in the transition from the hypothyroid to the hyperthyroid state. In humans, a linear correlation between the Na+, K+ ATPase concentration of skeletal muscle and the free T4 index was established. Significant effects of T3 on Ca2+ ATPase and Na+, K+ ATPase can be detected 24 h after a single injection. These effects are mediated by increased production of mRNA for the respective proteins, initiated by binding of T3 to nuclear receptors. Passive fluxes of Ca2+, Na+ and K+ also show a significant rise after T3 treatment. The increase in passive fluxes of Na+ and K+ can be detected before the rise in the concentration of Na+, K+ ATPase, suggesting that T3. In addition to its nuclear effects, may have a direct effect on the plasma membrane. Apart from their significance for muscle function in thyroid disease, the changes in Ca2+ ATPase and Na+, K+ ATPase will be important in other conditions where T3 and T4 levels show dramatic changes, i.e. during postnatal development, starvation and undernutrition, as well as in non-thyroidal illness (low-T3 syndrome).

Animals↗

Postpartum thyroid dysfunction: clinical assessment and relationship to psychiatric affective morbidity.

OBJECTIVE: Postpartum thyroid dysfunction (PPTD), diagnosed using biochemical criteria, is usually transient with a wide range of reported prevalence rates. The specific clinical and psychiatric morbidity associated with PPTD is still uncertain. The aims of the study were to determine the point prevalence of PPTD in Australian women at 6 months postpartum and to assess the specific clinical and psychiatric morbidity in these women. DESIGN: Women who were Caucasian, aged 20-45 years and 4.5-5.5 months postpartum, were randomly selected and invited into the study. The respondents were assessed for biochemical and psychiatric morbidity. PPTD for this study was defined as TSH or free T4 outside the adult reference range. A double blind clinical assessment of PPTD women and their matched controls used standardized clinical hypo- and hyperthyroid clinical indices. PATIENTS: From the total randomly selected sample size of 1816 women, 748 participated. MEASUREMENTS: Biochemical measurements were serum TSH, free T4, microsomal antibody (MsAb) and thyroid peroxidase antibody (TPOAb), and thyroid receptor antibodies (only in women with low TSH). Psychiatric assessment involved screening all participants using the General Health Questionnaire 28, followed by classifying and quantifying severity of cases using DSM-III-R categories for depression and anxiety. Clinical signs and symptoms of hypo- and hyper-thyroidism were measured using weighted standardized indices. Thyroid size was assessed by palpation. Achilles tendon reflex time was measured by photomotograph. RESULTS: The prevalence of PPTD in the participants was 11.5% (95% CI 9.2-13. 8%), giving a minimum prevalence for the randomly selected sample of 4.7% (95% CI 3.7-5.7%). In the PPTD women, 54% had an elevated TSH, 30% had a suppressed TSH and the remainder had a low fT4 and normal TSH. Positive thyroid autoantibody titres in the PPTD group were 46. 5% for microsomal antibody (MsAb) and 63.9% for thyroid peroxidase antibody (TPOAb), and in the non-PPTD group were 1.7% and 4.9%, respectively. The 6 month point prevalence rates of depression, generalized anxiety disorder and panic disorder and/or agoraphobia were 9.4%, 1.4% and 3.1%, respectively. No relationship was found between PPTD status and the diagnosis of current depression or between thyroid antibody status and current depression. In women who were diagnosed as anxious at the time of assessment, the number of anxiety symptoms was higher in the PPTD group (P < 0.05). There was no difference in signs and symptom scores for the hypo- and hyper-thyroid clinical indices between PPTD women and their controls. CONCLUSION: This study has shown a high prevalence of postpartum thyroid dysfunction but there was no difference in the clinical and psychiatric signs and symptoms between cases and controls. In the social, psychological, physical and endocrine setting of the postpartum period, women with postpartum thyroid dysfunction are identifiable by the attending physician only by their abnormal thyroid function tests.

Adult↗

Signs of neuropathy in the lower legs and feet of patients with acute intermittent porphyria.

OBJECTIVE: To assess signs of distal neuropathy in patients with acute intermittent porphyria (AIP). DESIGN: A population-based study. SUBJECTS: All patients with DNA-verified AIP >/= 18 years of age in the four most northerly counties of Sweden. INTERVENTION: Validated neuropathic signs and tests such as monofilament test, neuropathic pain, dry feet, extensor digitorum brevis (EDB) test, loss of forefoot arch, hammer toes and ulceration. RESULTS: A total of 356 patients were registered and 339 of them (95%) participated in the neuropathy study. The chronic neurological signs were symmetrical and similar to those in type 1 diabetic patients. Significant impairment was found concerning perception, EDB test, lower leg pain, ankle and knee tendon reflexes, but not concerning dry feet, loss of forefoot arch and hammer toes, on comparing patients with manifest versus latent AIP. The neurological signs were more severe in the diabetic patients (n = 298). Five AIP patients had permanent quadriplegia after severe attacks. CONCLUSIONS: Patients with manifest AIP had significantly more signs of distal chronic, symmetrical neuropathy of axonal type than did patients with latent AIP. More grave neurological lesions appear to develop after severe attacks.

Adolescent↗

Ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

We reported a 64-year-old male with an eight-month history of gait disturbance and sensory impairment. The patient initially noticed unsteadiness of gait and numbness in his feet, and these symptoms progressed until he was unable to walk without assistance five months later. Vibratory sensation and position sense were markedly diminished, and deep tendon reflexes were absent in all extremities. Motor conduction velocities were slow with prolonged distal latencies, and sensory nerve action potentials (SNAP) were not elicited. Sural nerve biopsy revealed a mild loss of myelinated fibres and segmental demyelination. Cerebrospinal fluid showed normal cell count with protein 526 mg/dL. Anti-GM1, anti-GM2 and anti-GA1 antibodies in serum were positive. We diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) presenting ataxia. Steroid therapy provided immediate improvement of symptoms and signs. This case suggests that CIDP should be considered as one of the potential causes of ataxic neuropathy.

Ataxia↗

PIBI(D)S: clinical and molecular characterization of a new case.

The term PIBI(D)S has been used to indicate a rare recessively inherited genetic disorder characterized by photosensitivity, mild non-congenital ichthyosis, brittle sulphur-deficient hair with trichoschisis (trichothiodystrophy), impaired intelligence, occasionally decreased fertility and short stature. To the best of our knowledge, about 20 cases have been reported in the literature. Here we report the characterization of the hair, brain, ultraviolet sensitivity and DNA excision repair defects of a new patient affected by PIBI(D)S. The diagnosis of PIBI(D)S syndrome was made in our patient on the basis of the clinical features and then confirmed by hair microscopy and biochemical analysis. Our patient has increased muscular tone, alteration of the deep tendon reflexes and psychomotor retardation, all consistent with hypomyelination of the brain showed by magnetic resonance imaging and computed tomography. A deficiency of DNA repair capacity was demonstrated in our patient. Furthermore, complementation analysis by cell fusion assigned our patient to xeroderma pigmentosum group D. The nucleotide excision repair defect of the other reported patients with PIBI(D)S falls generally into the same group as xeroderma pigmentosum group D and carry a mutation on the same repair gene (XPD). The relationship between these molecular characteristics and the clinical spectrum of PIBI(D)S is discussed.

Child↗

The stroke impairment assessment set: its internal consistency and predictive validity.

OBJECTIVE: To study the scale quality and predictive validity of the Stroke Impairment Assessment Set (SIAS) developed for stroke outcome research. DESIGN: Rasch analysis of the SIAS; stepwise multiple regression analysis to predict discharge functional independence measure (FIM) raw scores from demographic data, the SIAS scores, and the admission FIM scores; cross-validation of the prediction rule. SETTING: Tertiary rehabilitation center in Japan. PATIENTS: One hundred ninety stroke inpatients for the study of the scale quality and the predictive validity; a second sample of 116 stroke inpatients for the cross-validation study. MAIN OUTCOME MEASURES: Mean square fit statistics to study the degree of fit to the unidimensional model; logits to express item difficulties; discharge FIM scores for the study of predictive validity. RESULTS: The degree of misfit was acceptable except for the shoulder range of motion (ROM), pain, visuospatial function, and speech items; and the SIAS items could be arranged on a common unidimensional scale. The difficulty patterns were identical at admission and at discharge except for the deep tendon reflexes, ROM, and pain items. They were also similar for the right- and left-sided brain lesion groups except for the speech and visuospatial items. For the prediction of the discharge FIM scores, the independent variables selected were age, the SIAS total scores, and the admission FIM scores; and the adjusted R2 was .64 (p < .0001). Stability of the predictive equation was confirmed in the cross-validation sample (R2 = .68, p < .001). CONCLUSIONS: The unidimensionality of the SIAS was confirmed, and the SIAS total scores proved useful for stroke outcome prediction.

Adult↗

Painless aortic dissection presenting as high paraplegia: a case report.

Acute aortic dissection is a catastrophic episode that usually presents as a sudden, painful, ripping sensation in the chest or back. Physical findings may include loss of pulses and aortic regurgitation. It is associated with neurologic sequelae in as many as one third of patients. Painless dissection occurs in 5% of patients. We report a case of painless aortic dissection, presenting as acute paraplegia. The patient was a 77-year-old woman who presented with paraplegia, with no chest or back pain. On examination, strength was 5/5 in both upper extremities and 0/5 in both lower extremities. Deep tendon reflexes were absent in her legs. She had no voluntary anal contraction. Sensation was absent from T6 through S5. Computed tomography of the chest revealed a type A dissecting aneurysm. The vascular supply to the spinal cord and the differential diagnosis for new onset paraplegia are discussed.

Aged↗

Continuously infused intrathecal baclofen over 12 months for spastic hypertonia in adolescents and adults with cerebral palsy.

OBJECTIVE: To determine if the continuous intrathecal delivery of baclofen will control spastic hypertonia caused by long-standing cerebral palsy (CP). DESIGN: Case series. SETTING: Tertiary care outpatient and inpatient rehabilitation center directly attached to a university hospital. PATIENTS: Thirteen CP patients (average age, 25yr; range, 13--43yr) with intractable spastic hypertonia and quadriparesis (one of whom had predominate diplegia) who had not responded to oral medications including baclofen. INTERVENTION: Patients were screened via a bolus injection of baclofen intrathecally. Those who dropped an average of 2 points on their lower extremity (LE) Ashworth scores were offered computer-controlled pump implantation for 12 months of continuous delivery of intrathecal baclofen (ITB). MAIN OUTCOME MEASURES: Ashworth rigidity scores, spasm scores, and deep tendon reflex scores were collected for both the upper extremities (UEs) and LEs. Differences over time were assessed via descriptive statistics and Wilcoxon's signed-rank test. RESULTS: After 1 year of continuous ITB treatment, the average LE Ashworth score +/- standard deviation decreased from 3.4 +/- 1.2 to 1.5 +/- 0.7 (p <.0001), spasm score from 1.4 +/- 1.6 to 0.6 +/- 1.2 (p =.1024), and reflex score from 2.5 +/- 1.2 to 0.7 +/- 1.1 (p <.0001). The average UE Ashworth score decreased from 3.0 +/- 1.2 to 1.7 +/- 1.0 (p <.0001), spasm score from 1.2 +/- 1.6 to 0.2 +/- 0.6 (p =.0135), and reflex score from 2.3 +/- 0.7 to 0.5 +/- 0.9 (p <.0001). The average ITB dose required to attain these effects at 1 year was 263 +/- 91microg continuously infused per day. CONCLUSION: Continuously infused ITB can reduce spastic hypertonia in the UEs and LEs associated with long-standing CP. This reduction in tone will allow more freedom of movement and the potential for improved function.

Adolescent↗

Prospective assessment of tizanidine for spasticity due to acquired brain injury.

OBJECTIVE: To determine if orally delivered tizanidine will control spastic hypertonia due to acquired brain injury. DESIGN: Randomized, double-blind, placebo-controlled, crossover design, with 2 8-week treatment arms separated by a 1-week washout period at baseline. Patients were randomly assigned to receive tizanidine or a matching placebo. SETTING: Tertiary care outpatient and inpatient rehabilitation center attached to a university hospital. PARTICIPANTS: Seventeen persons recruited in a consecutive manner, 9 of whom had suffered a stroke and 8 a traumatic brain injury, and had more than 6 months of intractable spastic hypertonia. INTERVENTION: Over a 6-week period, subjects were slowly titrated up to their maximum tolerated dose (up to 36 mg/d). Following a 1-week drug taper and 1-week period in which no study drug was administered, patients were then crossed over to the other study medication following an identical titration regime. MAIN OUTCOME MEASURES: Subjects were evaluated for dose and effect throughout the trial as well as for side effects. Data for Ashworth rigidity scores, spasm scores, deep tendon reflex scores, and motor strength were collected on the affected upper extremity (UE) and lower extremity (LE). Differences over time were assessed via descriptive statistics, Friedman's analysis, and Wilcoxon's signed-rank. Data are reported as the mean +/- 1 standard deviation. RESULTS: Following 4 weeks of treatment when subjects reached their maximal tolerated dosage, the average LE Ashworth score on the affected side decreased from 2.3 +/- 1.4 to 1.7 +/- 1.1 (p <.0001). The spasm score decreased from 1.0 +/- 0.9 to 0.5 +/- 0.8 (p =.0464), while the reflex score was not statistically significant decreasing from 2.2 +/- 1.0 to 2.0 +/- 1.1 (p =.0883). The average UE Ashworth score on the affected side decreased from 1.9 +/- 1.1 to 1.5 +/- 0.9 (p <.0001). There was no significant change in the UE spasm and reflex scores. While there were positive placebo effects on motor tone, the active drug was still significantly better than placebo for decreasing LE tone (p =.0006) and UE tone (p =.0007). With a reduction in motor tone, there was an increase in motor strength (p =.0089). The average dosage at 4 weeks was 25.2mg/d. CONCLUSION: Tizanidine is effective in decreasing the spastic hypertonia associated with acquired brain injury, which is dose-dependent. There are limitations on its use due to side effects related to drowsiness.

Administration, Oral↗

A family with X-linked dominant Charcot-Marie-Tooth caused by a connexin32 mutation.

A family with a hereditary peripheral neuropathy is presented. Pedigree analysis suggested X-linked dominant mode of inheritance. The index patient became symptomatic at the age of 12 years. Clinical examination at 14 years revealed footdrop on the left, bilateral pes cavus, slight atrophy of thenar eminences, decreased muscle strength in both legs and brisk deep tendon reflexes. Electrophysiological studies were compatible with an axonal neuropathy. A novel point mutation located in codon 126 of the connexin32 gene, substituting a histidine for a tyrosine, was found in the index patient, in the mother, in two sisters and in a brother. The mother and the eldest sister had pes cavus bilaterally although they were asymptomatic. The younger brother and sister showed no signs of the disease.

Adolescent↗

Usefulness of a brief assessment battery for early detection of Charcot foot deformity in patients with diabetes.

Charcot neuroarthropathy is a significant limb-threatening complication that develops in some patients with long-term diabetes mellitus. Early diagnosis is vital to secondary prevention of the destructive process and avoidance of consequent deformity and, ultimately, amputation. The purpose of this study was to determine which historical and physical findings would be more accurate risk factor indicators in those diabetics with and without Charcot foot deformity. A controlled series of tests, historical findings, and physical examinations were performed on 41 patients with diabetes who were Charcot-free and 18 patients with diabetes with known chronic CD of the foot by using inexpensive hand-held instruments in a clinical setting. Physical examination included evaluation of vascular and neurologic characteristics. Historical findings consisted of those normally elicited from systems review or past medical history. The results indicate that simple neurologic testing combined with a thorough patient history were the most beneficial tools to determine diabetics with a higher probability of developing CD. Specifically, history of retinopathy (P <.02), nephropathy (P <.003), and previous foot ulcer (P <.01) were found to be predictive. The neurologic findings of vibratory sensation (P <.001), deep tendon reflexes (P <.05), and the 5.07 (10 g) Semmes-Weinstein monofilament test (P <.001) were also highly correlative for the development of Charcot foot deformity. Vascular examinations were found to differentiate poorly between groups. The application of this data may provide for earlier detection of Charcot arthropathy based on the predictive capabilities.

Aged↗

A case of reversible paraparesis following celiac plexus block.

BACKGROUND AND OBJECTIVES: Permanent and acute reversible paraplegia following celiac plexus block (CPB) have been reported. We report a case of prolonged reversible paraparesis after alcohol celiac plexus block. CASE REPORT: A 72-year-old man with primary multicentric pancreatic tumor and multiple hepatic metastases underwent alcohol celiac plexus neurolysis for severe abdominal pain radiating to the back. The patient had complete pain relief after the block but developed paresthesia of the left leg, which then spread to the right leg. Subsequently, loss of flexion and extension of the muscles supplying the left hip, knee, and foot developed. Deep tendon reflexes were brisk on the left compared to the right, and both plantar reflexes gave flexor responses. Magnetic resonance imaging and myelography were normal. Motor-evoked potential recordings showed a spinal cord lesion with involvement of the pyramidal and spinothalamic tracts. Somatosensory-evoked potentials indicated a relative sparing of dorsal column pathways. Physiotherapy was started, the sensory changes gradually subsided, and the patient was discharged 30 days after the block with clinically insignificant neurological deficit. CONCLUSIONS: Paraparesis following alcohol celiac plexus block may be reversible over an extended period of time.

Aged↗

Congenital muscular dystrophy: a clinico-pathological and follow-up study of 15 patients.

Fifteen patients with a presumptive diagnosis of congenital muscular dystrophy were followed for up to 15 years. The diagnosis was based on clinical, enzyme, histological and neurophysiological examinations. The group formed nine per cent of the 160 children suffering from neuromuscular disorders seen at the same hospital during a period of ten years. The muscle weakness was generalized and also involved respiratory muscles and the face. 60 per cent of the children had congenital contractures; these were well amenable to treatment. However, there was a strong tendency for new contractures to form from the second to third year onwards. There were also other signs indicating that the disease process was changing with time. The deep tendon reflexes were present in the beginning but later were usually lost. The serum creatine kinase was raised even to high levels in the first one to two years and gradually sank to normal or near normal values. The histopathological findings changed with time from relatively slight changes compatible with a muscle destroying process to inactive type lesions characterized by fibrotic and particularly adipose tissue replacing muscle fibres. On the basis of these findings it can be assumed that the active disease process is at its height during intrauterine and early postnatal life and then wanes leaving an outburnt or cicatrical state in which new contractures easily develop causing possible deterioration with time. Active treatment is thus of great importance both to overcome neonatal contractures and to prevent new ones to develop.

Adolescent↗

[Posttraumatic syringomyelia].

The improvement of preclinical emergency medicine, better surgical and conservative therapies, and the development of intensive care units and specialized centers have improved the survival rate for patients with serious spinal cord injuries. Therefore, more sequelae of chronic spinal cord injuries such as post-traumatic spinal cord cavitations also occur. The first such case was described by Bastian in 1867. Generally, these cavitations were diagnosed from 2 months up to 32 years after the trauma. The overall prevalence of post-traumatic syringomyelia (PTS) is not known; however, with the increasing use of magnetic resonance imaging (MRI), its diagnosis has increased, ranging from 2.3% of paraplegic and tetraplegic patients in 1976 and 3.2% in 1985, to nearly 50% in a selected group of patients in 1991 and 1993. In 1995, a 4.45% incidence was reported. In our clinic we are currently treating 440 cases of syringomyelia, 140 of which are PTS. Several observations suggest more than one potential mechanism for the evolution of a post-traumatic cyst or PTS. Various factors, such as hemorrhage or, in particular, ischemia within the spinal cord, blockage of the cerebrospinal fluid (CSF) pathways around the cord or localized meningeal fibrosis either alone or in combination with other factors, may be involved. Clinically, sensory disturbances, loss of motor function, pain, and modification of the deep tendon reflexes are observed in most patients. On MRI, PTS is seen as a longitudinal, cystic cavity within the spinal cord, giving a hypointense signal on T1-weighted images and a hyperintense signal on T2-weighted images. For treatment planning it is mandatory to identify the lower and upper end of the PTS on the MRI.

Humans↗