Neurovascular relationship at the trigeminal root entry zone in persistent idiopathic facial pain: findings from MRI 3D visualisation

BACKGROUND: Patients with atypical neuralgia or atypical facial pain have been surgically treated with microvascular decompression (MVD) of the trigeminal root entry zone (TREZ). There are no data regarding the sensitivity and specificity of a vessel-TREZ relationship as a cause of pain in patients with persistent idiopathic facial pain (PIFP) according to the definition given by the International Headache Society (IHS). METHODS: The TREZ was visualised by 3D CISS MRI in 12 patients with unilateral PIFP according to the IHS criteria. RESULTS: The frequency of artery-TREZ, vein-TREZ, or vessel (artery/vein)-TREZ contacts on the symptomatic and asymptomatic sides did not differ significantly. On the symptomatic side, vessel-TREZ contact was found in 58% of patients (sensitivity). On the asymptomatic side, vessel-TREZ contact was absent in 33% of patients (specificity). CONCLUSIONS: On the basis of the low sensitivity and specificity found in the present study, PIFP cannot be attributed to a vessel-TREZ contact, and therefore, pain relief after MVD cannot be expected.     

Pediatric Chiari I malformations: do clinical and radiologic features correlate?

BACKGROUND: Although Chiari I malformation is increasingly recognized in children, little is known about its clinical presentation in this age group. OBJECTIVE: To evaluate the relationship between clinical and MRI features of pediatric Chiari I malformations. METHODS: We performed a chart review and MRI analysis of 49 children with Chiari I malformation. The degree of tonsillar ectopia was compared with age at onset, presence of syringomyelia, and a neurologic severity score as follows: asymptomatic = 0, symptomatic with normal neurologic examination = 1, and symptomatic with abnormal examination = 2. RESULTS: Age at onset of symptoms ranged from 10 months to 14 years. Fifty-seven percent of patients were asymptomatic. Headache and neck pain were the most frequent complaints. Syringomyelia was detected in 14% of patients and skull base abnormalities in 50%. The magnitude of tonsillar ectopia (5 to 23 mm) correlated with severity score (p = 0.04) but not with other clinical measures. CONCLUSIONS: The clinical symptoms of Chiari I malformations in children are nearly identical to those seen in adults. Children with greater amounts of tonsillar ectopia on MRI are more likely to be symptomatic.     

平山病患者的临床、肌电图和磁共振研究

目的探讨平山病(HD)患者的临床表现、肌电图和磁共振(MRI)特点。方法收集2009-05-2012-02收治的HD患者的人口学资料、临床表现、电生理和颈椎MRI表现,并进行分析。另设年龄、性别匹配的健康对照组进行电生理检查。结果 (1)HD患者共25例,均为男性,年龄15～24岁,平均年龄(17.64±2.20)岁。临床表现局限于单侧者22例,其中右侧15例、左侧7例;双侧者3例。手或/和前臂肌肉萎缩25例,冷麻痹13例,手指震颤5例。(2)有临床表现侧小指展肌和拇短展肌肌肉电位波幅均减低,且二者波幅比值减小。(3)异常自发活动出现率在第一骨间肌和小指展肌100%、拇短伸肌89.3%、拇短展肌85.7%;在单侧有临床表现的22例中,11例(50%)患者的无症状侧显示异常自发活动。(4)完成MRI扫描的14例中,自然位均显示第2～7颈椎体后方下缘连线与椎体相交、下段颈髓萎缩变扁平;屈曲位时硬膜腔后壁前移7例。结论 HD多见于男性,以右侧多发。结合临床、肌电图以及影像学表现,有助于临床更全面认识HD。     

单肢肌萎缩/平山病:41例患者临床、电生理和颈椎MRI特征分析

目的 探讨单肢肌萎缩(MMA)/平山病患者的临床、肌电图及颈椎MRI特征。方法 2009年5月至2014年5月就诊本科,符合诊断标准的患者,连续登记并详细记录及分析人口学资料、临床、电生理和颈椎自然位和屈曲位MRI资料。结果(1)共41例,男39例、女2例,发病年龄14～24岁、平均年龄(16.87±2.62)岁。病程1～121月、平均病程(22.13±26.25)月。双上肢均有临床症状者6例(14.6%),症状局限单侧者35例(85.4%); 单侧者左12例(29.3%)、右23例(56.1%)。冷麻痹22例,指震颤9例,手麻木4例; 41例均有手固有肌萎缩,均无感觉障碍;(2)症状侧尺和正中神经运动潜伏期延长,小指展肌、拇短展肌运动波幅减低,小指展肌/拇短展肌波幅比值减小;(3)症状侧针肌电图显示异常自发活动者的出现率,在第一骨间肌和小指展肌为100%、拇短伸肌90.1%、拇短展肌86.3%、肱桡肌16.8%、肱二头肌13.8%; 在仅限于单侧症状的35例患者中,无症状侧手固有肌也显示异常自发活动者占51.4%;(4)32例患者完成颈椎MRI检查。自然位时32例均显示颈2-颈7椎体后方下缘连线与椎体相交; 均显示下段颈髓萎缩变扁平,其中位于C5-C7节段14例,C5-C6节段6例,C6-C7节段7例,C5-T1节段5例; 屈颈位时15例显示硬膜腔后壁前移,移位的硬膜后方可见硬膜外占位,内有流空信号,恢复自然位后占位消失。9例显示髓内可疑T₂异常高信号。结论 MMA/平山病主要见于青少年男性; 电生理表现为低位颈髓前角细胞病变,且无症状侧可显示临床下神经源性损害; 小指展肌/拇短展肌波幅比值减小,是有鉴别意义的电生理指标; 屈颈位颈椎MRI对于诊断很重要。结合临床、神经电生理及影像表现,有助于更全面认识本病。     

Somatosensory evoked potentials following nerve and segmental stimulation do not confirm cervical radiculopathy with sensory deficit.

Twenty eight patients with unilateral cervical radiculopathy were studied by somatosensory evoked potentials (SEPs) from nerve stimulation at the wrist and from skin stimulation at the first, third or fifth finger depending on the root involved. In order to evaluate the reliability of various "radicular SEP patterns" as described in the literature, absolute latencies and side-to-side differences of the brachial plexus component from the supraclavicular fossa (N9), the medullary component (N13) from the cervical vertebra Cv7, and the primary cortical component (N20, P25) were assessed. Side-to-side differences of the amplitudes of N20/P25 and of the conduction times across the intervertebral fossa (interval N9-N13) were analysed. After nerve stimulation, 68% of the patients had false negative findings on the symptomatic, while 36% had positive findings on the asymptomatic side. After segmental stimulation, 72% of the patients had false negative findings on the symptomatic, while 22% had positive findings on the asymptomatic side. It is concluded that SEPs following nerve and segmental stimulation do not reliably confirm clear-cut already established diagnoses of unilateral radiculopathy with sensory and motor deficit. Therefore, they will not be helpful in the electrophysiological investigation of cervicobrachialgias of unknown origin.     

Association between trigeminal neuralgia and multiple sclerosis: role of magnetic resonance imaging.

Seven consecutive patients with multiple sclerosis and trigeminal neuralgia were investigated with MRI to determine the occurrence of a lesion which would account for the patients' pain. Two patients had bilateral symptoms. In the patients with unilateral trigeminal neuralgia vascular compression of the nerve by an artery at the root entry zone on the symptomatic side was confirmed in three patients and an epidermoid tumour distorting the nerve on the symptomatic side was identified in one patient. A demyelinating plaque was identified in only one patient, affecting the trigeminal nerve at the root entry zone at the pons. In those with bilateral symptoms neurovascular compression was identified on both sides in one patient and on one side only in the remaining patient. Microvascular decompression cured the pain in two patients with neurovascular compression. The variable aetiology of trigeminal neuralgia is stressed even in patients with coexistent neurological conditions such as multiple sclerosis, which can cause trigeminal neuralgia independent of other causes.     

When does the patient with a disc herniation undergo lumbosacral discectomy?

OBJECTIVES: To design a model that could accurately predict eventual lumbar disc surgery in the patient initially presenting with clinical findings of nerve root compression. METHODS: Prospective study in 183 patients with nerve root compression sampled from a primary care population. All patients underwent a standardised history, physical examination, and MRI. Surgery carried out in the next 6 months was recorded. Models were constructed to predict whether patients eventually received surgery. RESULTS: Two models were constructed. Reduced model A was based on baseline findings, only, and contained six variables. Model B incorporated change over time as well and contained 10 variables. The area under the curve (in a receiver operating characteristic) for these models was 0.86 and 0.92, respectively. It was shown that at a probability cut off of 0.60, model B predicted eventual surgery with a sensitivity of 57% and a specificity of 100%. CONCLUSIONS: Given the requirement of a high specificity, eventual operation could be adequately predicted in a sample of 183 patients with clinical nerve root compression. The application of the model in patients with nerve root compression might lead to earlier operation in a subset of patients resulting in a reduction of duration of illness and associated indirect costs. An important prerequisite for future application would be the validation of the prediction rule in another population.     

Clinical and neuroradiological features of syringomyelia associated with Chiari malformation

The clinical presentation and radiological features were analyzed in 30 cases of syringomyelia associated with Chiari malformation. None of the patients had spinal dysraphism. The age on admission ranged from 6 to 59 years with a mean of 27 years. Syringomyelia was diagnosed by CT myelography and or MRI from 1982 to 1988. The initial symptoms were skeletal abnormality (43%) such as scoliosis (12 cases) or pes cavus (one case), unilateral pain or numbness (40%) and unilateral motor weakness (17%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (57%), muscle weakness (57%), muscle atrophy (37%) and lower cranial nerve palsy (40%). The neurological findings were asymmetrical in all patients. The characteristic neurological findings in the cases presenting under 20 years of age were unilateral sensory and motor deficits (61%) with decreased or absent deep tendon reflex on the same side. The localization of the syrinx in axial section varied according to the level even in the same case. In 15 cases with unilateral sensory disturbance or unilateral sensory and motor deficit, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance in the cervical or thoracic level. On the other hand, in 15 cases with bilateral sensory and motor deficit, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side.(ABSTRACT TRUNCATED AT 250 WORDS)     

Electroencephalographic and Magnetic Resonance Correlations in Children with Intractable Seizures of Lennox-Gastaut Syndrome and Epilepsia Partialis Continua

Summary: A study was performed of EEG-magnetic resonance imaging (MRI) abnormalities in 7 Lennox-Gastaut syndrome (LGS) children and 3 epilepsia partialis continua (EPC) children with intractable generalized and partial motor seizures, respectively. In 4 children with LGS and 3 children with EPC, depth electrodes were implanted in the centromedian thalamic nuclei (CM) for seizure control. In all children with LGS, hyperdense, T₂-weighted MRI signals were observed at the mesencephalic level of the brainstem, whereas none of the 3 children with EPC had this finding. Patients with idiopathic LGS without cerebral hemisphere MRI abnormalities showed generalized bilateral and symmetrical spike-wave EEG activity. Patients with symptomatic LGS with unilateral hemispheric MRI abnormalities demonstrated asymmetrical EEG activity with higher amplitude spike-and-wave over the normal hemisphere. Patients with EPC with unilateral hemispheric lesions had lateralized higher amplitude spike-wave over the damaged hemisphere. These data suggest that abnormal mesencephalic MRIs are a sign of bad prognosis in patients with LGS but not with EPC. Maximal amplitude paroxysmal EEG activities may indicate the abnormal hemisphere in patients with EPC or the normal hemisphere in those with LGS.     

Diagnostic value of history, physical examination and needle electromyography in diagnosing lumbosacral radiculopathy

To examine the diagnostic value of history, physical examination and needle EMG in predicting nerve root compression on MRI in patients with clinical suspicion of lumbosacral radicular syndrome (LSRS). Subjects comprised 202 consecutive patients from January 2006 to March 2007 with suspicion of LSRS referred by general practitioners. Clinical evaluation consisted of history, physical examination, EMG and MRI. Bivariate and multiple logistic regression analyses were used to calculate the diagnostic value of each test item compared to radiological nerve root compression. 95 patients (47%) had radiological nerve root compression. Significant predictors of radiological nerve root compression were dermatomal radiation [odds ratio (OR) 2.1], more pain on coughing, sneezing or straining (OR 2.4), positive straight leg raising (OR 3.0) and ongoing denervation on EMG (OR 4.5). 15 patients (7%) had ongoing denervation on EMG without radiological nerve root compression. In clinical practice, dermatomal radiation, more pain on coughing, sneezing or straining, positive straight leg raising and ongoing denervation on EMG may be used to predict nerve root compression on MRI. EMG may also be of additional value in patient with clinical suspicion of lumbosacral radicular syndrome without nerve root involvement on MRI.     

Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures.

Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.     

Sacral Tarlov cysts: Neurophysiology abnormalities and correlation with pelvic sensory and visceral symptoms

Background and purpose

Recent studies suggest a possible association between Tarlov cysts (TCs), usually considered as incidental radiological findings, and neurological symptoms such as pain, numbness and urogenital complaints. The aim was to explore the relationship between TCs and sacral nerve root functions using pelvic neurophysiology tests, and to correlate changes with clinical symptoms and magnetic resonance imaging (MRI) findings.

Methods

Consecutive patients with sacral TCs, referred for pelvic neurophysiology testing and presenting with at least one symptom related to the pelvic area, participated in a cross-sectional review of symptoms using validated questionnaires. Findings of pelvic neurophysiology (pudendal sensory evoked potentials, sacral dermatomal sensory evoked potentials, external anal sphincter electromyography) and urodynamics testing were collected retrospectively. The relationship between neurophysiology, MRI findings and patients' symptoms was assessed using Fisher and ANOVA tests.

Results

Sixty-five females were included (mean age 51.2 ± 12.1 years). The commonest symptom was pain (92%). Urinary (91%), bowel (71%) and sexual (80%) symptoms were also frequently reported. Thirty-seven patients (57%) had abnormal neurophysiology findings reflecting sacral root dysfunction. No association was seen between MRI findings (size, location of the cysts, severity of compression) and neurophysiology. A negative association was observed between neurophysiology abnormalities and occurrence of urgency urinary incontinence (p = 0.03), detrusor overactivity (p < 0.01) and stress urinary incontinence (p = 0.04); however, there was no association with voiding difficulties.

Conclusions

Contrary to current understanding, TCs are associated with injury to the sacral somatic innervation in the majority of patients with presumed symptomatic cysts. However, urinary incontinence is unlikely to be related to TC-induced nerve damage.     

Warm and cold sensory thresholds in patients with unilateral sciatica: C fibers are more severely affected than A-δ fibers

Objectives - In order to evaluate if nerve root compression or inflammation is the most important pathogenetic mechanism in lumbar radicular pain, we investigated unmyelinated C-fiber function (warm sensation) and myelinated A-delta fiber function (cold sensation) in patients with unilateral L₅ or S: sciatica. Material and methods - Forty consecutive patients with clinical and radiological evidence of unilateral L₅ ( n =29) or S₁ ( n = 11) sciatica were studied. The warm and cold sensory thresholds (Somedic thermotest, method of limits) were measured on the anterolateral leg (L₅ dermatome) and on the calf (S₁ dermatome) on both sides. Results - Warm thresholds were significantly higher on the symptomatic side compared to the non-symptomatic side (8.4±3.0 vs 6.2±2.5°C, P <0.0005) in the affected dermatome. In a subgroup with confirmed disk herniation at surgery (32 of the 34 operated), significant differences between the symptomatic and the non-symptomatic side for the affected dermatome, were found for both warm ( P <0.0005) and cold ( P =0.003) thresholds. No threshold difference was seen in patients with disk herniations contained within the outer annulus fibrosis ( n = 22) compared to those with non-contained herniations ( n = 10). Conclusions - Patients with unilateral sciatica and L₅ or S₁ nerve root involvement had increased warm thresholds suggesting impaired C-fiber function. Cold thresholds were significantly elevated in a subgroup with operatively confirmed disk herniation. Because myelinated axons are affected more by compression than unmyelinated ones, our results suggest that nerve root inflammation is more important than compression per se in the generation of sciatic pain.     

Evidence for shoulder girdle dystonia in selected patients with cervical disc prolapse.

Some patients with cervical disc herniation suffer from persistent nuchal pain and muscle spasms after decompressive surgery despite the lack of clinical and radiological signs for actual spinal root compression. Sonographic examination of the brain in some of these patients showed increased echogenicity of the lentiform nuclei as described in patients with idiopathic dystonia. This has been linked to an altered Menkes protein level and copper metabolism. We suggest a relationship between persistent nuchal pain after adequate cervical disc surgery and dystonic movement disorders. Thirteen patients with persistent nonradicular nuchal pain after at least one cervical disc surgery and without evidence of continuing spinal root compression and 13 age-matched controls were included. All patients had a complete neurological examination, ultrasound, and MRI scan of the brain. In addition, Menkes protein mRNA levels of leucocytes were analyzed in patients and controls. All patients with persistent nuchal pain exhibited a constant tonic unilateral shoulder elevation associated with an ipsilateral hypertrophy of the trapezius muscle. Ultrasound examination showed an increased echogenicity of the lentiform nucleus in one patient unilaterally and in 10 patients bilaterally but in none of the controls. On MRI the T2-values of the lentiform nuclei were found to be higher in patients exhibiting a hyperechogenicity of the lentiform nuclei compared to controls (P = 0.01). In addition, Menkes protein mRNA levels were decreased in patients with cervical disc herniation (P = 0.03). Clinical, neuroimaging, and biochemical findings of this selected patient sample with chronic nuchal pain and muscle spasms after cervical disc surgery resemble alterations in patients with idiopathic cervical dystonia. This suggests a link between both disorders. A peripheral trauma to the nerve roots may precipitate dystonic movements in susceptible patients and chronic dystonic muscle contraction would account for the persistent nuchal pain.     

Retrospective study of a large population of patients with asymptomatic or minimally symptomatic raised serum creatine kinase levels

A retrospective evaluation of asymptomatic subjects with persistent elevation of serum creatine kinase (CK) levels (hyperCKemia) was made in order to verify the presence of subclinical myopathy or idiopathic hyperCKemia and to define the most appropriate diagnostic pathway. Persistently increased serum CK levels are occasionally encountered in healthy individuals. In 1980 Rowland coined for them the term idiopathic hyperCKemia. Despite the increase of scientific knowledge, several healthy subjects with hyperCKemia still represent a problem for the clinician. We made a retrospective evaluation of 114 asymptomatic or minimally symptomatic individuals with incidentally detected persistent hyperCKemia. They underwent neurological examination and laboratory/instrumental evaluation. Skeletal muscle biopsy was performed and thoroughly investigated. Biochemical and genetic investigations were added in selected cases. Logistic regression analysis was applied. We diagnosed a neuromuscular disorder in 21 patients (18.4 %), and found, by muscle biopsy and/or EMG, pathological but not conclusive findings in 57 subjects (50 %). The statistic correlation between elevated serum CK levels and the probability of making a diagnosis changed according to the age of the patient. Conclusions Muscle biopsy is the basic tool for screening asymptomatic subjects with hyperCkemia. It allowed us to make a diagnosis of disease in 18.4 % of patients, and to detect skeletal muscle abnormalities in 38.6 % of the subjects. Interestingly, 31.6 % of individuals had completely normal muscle findings. These best fit the “diagnosis” of idiopathic hyperCKemia. Received: 13 March 2001, Received in revised form: 3 July 2001, Accepted: 5 July 2001     

Brain abnormalities in neuromyelitis optica

BACKGROUND: Neuromyelitis optica (NMO) is a severe demyelinating disease defined principally by its tendency to selectively affect optic nerves and the spinal cord causing recurrent attacks of blindness and paralysis. Contemporary diagnostic criteria require absence of clinical disease outside the optic nerve or spinal cord. We have, however, frequently encountered patients with a well-established diagnosis of NMO in whom either asymptomatic or symptomatic brain lesions develop suggesting that the diagnostic criteria for NMO should be revised. OBJECTIVE: To describe the magnetic resonance image (MRI) brain findings in NMO. DESIGN: Observational, retrospective case series.Patients We ascertained patients through a clinical biospecimens database of individuals with definite or suspected NMO. We included patients who (1) satisfied the 1999 criteria of Wingerchuk et al for NMO except for the absolute criterion of lacking symptoms beyond the optic nerve and spinal cord and the supportive criterion of having a normal brain MRI at onset; (2) had MRI evidence of a spinal cord lesion extending 3 vertebral segments or more (the most specific nonserological feature to differentiate NMO from MS); and (3) were evaluated neurologically and by brain MRI at the Mayo Clinic. MAIN OUTCOME MEASURES: Magnetic resonance images were classified as normal or as abnormal with either nonspecific, multiple sclerosis-like or atypical abnormalities. We evaluated whether brain lesions were symptomatic and analyzed the neuropathologic features of a single brain biopsy specimen. RESULTS: Sixty patients (53 women [88%]) fulfilled these inclusion criteria. The mean +/- SD age at onset was 37.2 +/- 18.4 years and the mean +/- SD duration of follow-up was 6.0 +/- 5.6 years. Neuromyelitis optica-IgG was detected in 41 patients (68%). Brain MRI lesions were detected in 36 patients (60%). Most were nonspecific, but 6 patients (10%) had multiple sclerosis-like lesions, usually asymptomatic. Another 5 patients (8%), mostly children, had diencephalic, brainstem or cerebral lesions, atypical for multiple sclerosis. When present, symptoms of brain involvement were subtle, except in 1 patient who was comatose and had large cerebral lesions. CONCLUSIONS: Asymptomatic brain lesions are common in NMO, and symptomatic brain lesions do not exclude the diagnosis of NMO. These observations justify revision of diagnostic criteria for NMO to allow for brain involvement.     

Post-surgical thoracic pseudomeningocele causing spinal cord compression

Pseudomeningoceles are extradural cerebrospinal fluid collections categorized into three groups: traumatic, congenital, and iatrogenic. Iatrogenic pseudomeningoceles occur after durotomy, usually after cervical or lumbar spine surgery. Although many remain asymptomatic, pseudomeningoceles can compress or herniate the spinal cord and nerve roots. We present a 57-year-old woman who had a thoracic laminectomy and discectomy. Two weeks after surgery, she presented with lower extremity weakness and gait difficulty. Physical examination revealed hyperreflexia and a T11 sensory level. MRI revealed a pseudomeningocele compressing the thoracic spinal cord. The patient underwent surgical drainage of the cyst. On follow-up, she had complete resolution of her symptoms, and MRI did not show a residual lesion. To our knowledge, this is the second documented post-operative pseudomeningocele causing symptomatic spinal cord compression of the thoracic spine. In this article, a review of the literature is presented, including four reported patients with post-traumatic pseudomeningocele causing myelopathic symptoms and 20 patients with iatrogenic pseudomeningocele that resulted in neurological decline due to herniation or compression of neural tissue. Treatment options for these lesions include conservative management, epidural blood patch, lumbar subarachnoid drainage, and lumbo-peritoneal shunt placement. Surgical repair, usually by primary dural closure, remains the definitive treatment modality for iatrogenic symptomatic pseudomeningoceles.     

Late whiplash syndrome: a clinical and magnetic resonance imaging study

Cervical hyperextension injuries are common and are associated with significant morbidity. Clinically two syndromes are described: "acute" whiplash syndrome and "late" whiplash syndrome (in which the patients are still symptomatic after six months despite normal physical and radiological examination). In order to clarify the pathology of the persistent pain in late whiplash syndrome we performed a cervical spine magnetic resonance imaging (MRI) in 33 consecutive patients suffering from this condition. Twenty-six patients (78.8%) showed MRI abnormalities, the most common MRI finding (57.6%) was pre-existent spondylosis. Indeed, the group of patients with spondylosis and other MRI changes had higher clinical scores than those without MRI abnormalities as measured by a three-point grading system based upon the symptoms and signs shown. Several MRI changes, most of them already demonstrable by standard X-ray were seen among 33 patients suffering from late whiplash syndrome. Although no one of these findings appears to be specific and certainly related to the previous neck injury, they could represent a risk factor for a longer pain duration.     

Follow-up of 89 asymptomatic patients with adrenoleukodystrophy treated with Lorenzo's oil

OBJECTIVES: To identify asymptomatic boys with X-linked adrenoleukodystrophy who have a normal magnetic resonance image (MRI), and to assess the effect of 4:1 glyceryl trioleate-glyceryl trierucate (Lorenzo's oil) on disease progression. METHOD: Eighty-nine boys (mean +/- SD baseline age, 4.7 +/- 4.1 years; range, 0.2-15 years) were identified by a plasma very long-chain fatty acids assay used to screen at-risk boys. All were treated with Lorenzo's oil and moderate fat restriction. Plasma fatty acids and clinical status were followed for 6.9 +/- 2.7 years. Changes in plasma hexacosanoic acid levels were assessed by measuring the length-adjusted area under the curve, and a proportional hazards model was used to evaluate association with the development of abnormal MRI results and neurological abnormalities. RESULTS: Of the 89 boys, 24% developed MRI abnormalities and 11% developed both neurological and MRI abnormalities. Abnormalities occurred only in the 64 patients who were aged 7 years or younger at the time therapy was started. There was significant association between the development of MRI abnormalities and a plasma hexacosanoic acid increase. (For a 0.1-microg/mL increase in the length-adjusted area under the curve for the hexacosanoic acid level, the hazard ratio for incident MRI abnormalities in the whole group was 1.36; P = .01; 95% confidence interval, 1.07-1.72.) Results for patients aged 7 years or younger were similar (P = .04). CONCLUSIONS: In this single-arm study, hexacosanoic acid reduction by Lorenzo's oil was associated with reduced risk of developing MRI abnormalities. We recommend Lorenzo's oil therapy in asymptomatic boys with X-linked adrenoleukodystophy who have normal brain MRI results.     

脑神经疾患“血管压迫”病因的放射学诊断研究——磁共振断层成像脑血管造影术的应用

目的:探讨磁共振影像学对“压迫血管”病因诊断的可能性和特征。方法:采用稳定快速进动程序以提供脑实质、脑神经和后颅窝脑血管的高分辨率磁共振成像(MRI),即一种“白血”磁共振断层成像血管显影术(magnetic resonance tomographic angiography,MRTA)。分别检查11例三叉神经痛和21例面肌痉挛,同时10例行后颅窝探查及显微血管减压术(microvascular decompression,MVD),对照和评估MRTA的确诊率。结果:32例单侧三叉神经痛及面肌痉挛的MRTA中,确认患侧有“血管压迫”28例,无血管压迫4例。而正常侧仅见2例“血管接触或压迫”影像。1例三叉神经痛术中发现岩静脉为压迫血管,而MRTA提示无异常,因而未再行增强扫描、10例接受探查术发现有或无压迫血管,与MRTA诊断相符9例。结论:MRTA可作为脑神经疾患的”血管压迫”病因之术前诊断的一种特殊而可靠的检查方法,以便有的放矢地指导此类疾患的治疗。