Search

We report a 60-year-old woman who developed spinal cord infarction (SCI) with anti-aquaporin (AQP) 4 antibody seropositive. She was admitted to our hospital with acute onset of flaccid paraparesis and urinary disturbances that completed within a few minutes after acute pain in her lower back. Neurological examination revealed flaccid paraparesis, bladder and bowel dysfunction and dissociated sensory loss below the level of Th11 spinal cord segment. Diffusion weighted imaging (DWI) and T2-wighted imaging (T2WI) of thoracic spine MRI showed high signal intensity in the spinal cord between Th9 and Th12 vertebral levels with decreased apparent diffusion coefficient (ADC). We diagnosed her as having SCI. Thereafter the serum examination on admission was reported as positive for anti-aquaporin 4 (AQP4) antibody. Cerebrospinal fluid (CSF) analysis revealed pleocytosis, and the spinal cord lesions became enlarged in MRI on 12 days after the onset. We, therefore, suspected that the pathophysiology of neuromyelitis optica spectrum disorder (NMOSD) accompanied SCI. The patient underwent two courses of high dose intravenous methylprednisolone (IVMP) for three days (1 g/day). Her neurological symptoms did not improve significantly, but the size of T2WI MRI high signal lesion improved to that of the initial MRI scan. Anti-AQP4 antibody seropositivity may have modified the SCI pathology in the present patient.

OBJECTIVE To present an alternative surgical technique in treating cases of Chiari I Malformation with mild-to-moderate syringomyelia after decompressive suboccipital craniectomy: incising only the outer layer of the dura mater, then dissecting it from the inner layer without opening the latter. PATIENTS AND METHODS We utilized this technique in a short series of three cases who were admitted to our department for mild symptoms such as intermittent headache and dissociated sensory loss in the upper limbs, caused by a Chiari Malformation Type I. The patients were placed in the sitting position. We performed a reduced median suboccipital craniectomy and resection of the posterior arch of C1 adapted to the level of tonsil descent, from a limited superior half to complete resection. Afterward, we incised the outer dural layer, while sparing the inner one. Using a fine dissector, we then split apart the outer and inner layers to the margin of the craniectomy. Through the transparency of the inner layer and the arachnoid, the cerebellum and the medulla were visible and pulsating. An autologous fascia duraplasty was then performed. RESULTS The postoperative course was favorable in all cases, the patients being discharged without any deficits and with complete symptom resolution. Follow-up at 3, 6, and 12 months after surgery revealed a significant reduction in brainstem compression and syringomyelia. CONCLUSIONS Interlayer dural split technique can be used effectively in treating symptomatic cases of type I Chiari malformation in adults, with mild-to-moderate syringomyelia. It is less invasive than opening the dura and possibly more effective than decompressive craniectomy and C1 laminectomy alone. This technique must be validated in a larger case-control series.

Objective: To present an alternative surgical technique in treating cases of Chiari I Malformation with mild-to-moderate syringomyelia after decompressive suboccipital craniectomy: incising only the outer layer of the dura mater, then dissecting it from the inner layer without opening the latter. Methods and Results: We utilized this technique in a short series of three cases who were admitted in our department for mild symptoms such as intermittent headache and dissociated sensory loss in the upper limbs, caused by a Chiari Malformation Type I. The patients were placed in the sitting position. We performed a reduced median suboccipital craniectomy and resection of the posterior arch of C1 adapted to the level of tonsil descent, from a limited superior half to a complete resection. Afterwards, we incised the outer dural layer, while sparing the inner one. Using a fine dissector, we then split apart the outer and inner layers to the margin of the craniectomy. Through the transparency of the inner layer and the arachnoid, the cerebellum and the medulla were visible and pulsating. An autologous fascia duraplasty was then performed. The postoperative course was favorable in all cases, patients being discharged without any deficits and with complete symptom resolution. Conclusions: Interlayer dural split technique can be used effectively in treating symptomatic cases of type I Chiari malformation in adults, with mild-to-moderate syringomyelia. It is less invasive than opening the dura and possibly more effective than decompressive craniectomy and C1 laminectomy alone. This technique must be validated in a larger case-control series.

Non-Hodgkin lymphomas are a heterogeneous group of tumours, with diffuse large B-cell lymphomas (DLBCL) being particularly common. Cases of DLBCLs developing in the central nervous system, especially in the spinal cord, are extremely rare and thus pose significant diagnostic and therapeutic problems, particularly for orthopaedists and neurologists since these are the specialists the patients first consult. The tumours often appear in immunosuppressed patients and standard chemotherapy is ineffective. This paper presents the case of a 44-year-old male with a lymphoma located in the spinal cord at the C7-Th1 level. The symptoms appeared suddenly and progressed rapidly, with dissociated sensory loss and limb paresis being the most pronounced. Imaging studies and the rapid symptom progression suggested neoplastic disease. An emergency surgical procedure was performed in order to decompress the spinal cord and thus limit neurological deficits. Tumour resection allowed for making a diagnosis. By four weeks after the surgery, the tumour had grown larger than before the procedure. Chemotherapy with MTX and Ara-C and intrathecal MTX resulted in full remission. Consolidation was achieved with radiotherapy. Currently, with the low incidence of such tumours, there are no standards of management in patients with DLBCLs of the CNS. The literature contains only a few case reports on successfully treated spinal cord DLBCLs.

A clinical case of surgical treatment of a female patient with post-traumatic syringomyelia which led to tetraparesis and dissociated sensory loss in the trunk and upper and lower limbs is presented. Clinical manifestations of these spinal cord changes occurred 21 years after complicated fracture of the L1 vertebra associated with complex posttraumatic spinal deformity. Multistage surgical intervention made it possible to restore liquorodynamics, perform the necessary correction of severe kyphotic deformity of the spine, and reduce the risk of torso imbalance. As a result, the syrinx practically disappeared at all levels of the study.

Background: Craniometric studies document different subtypes of craniocervical junction malformations (CCJM). Here, we identified the different types and global signs and symptoms (SS) that correlated with these malformations while further evaluating the impact of syringomyelia. Methods: Prospective data concerning SS and types of CCJM were evaluated in 89 patients between September 2002 and April 2014 using Bindal’s scale. Results: The mean Bindal’s scores of each type of CCJM were Chiari malformation (CM) = 74.6, basilar invagination Type 1 (BI1) = 78.5, and BI Type 2 (BI2) = 78. Swallowing impairment and nystagmus were more frequently present in the BI patients. Symptomatic burdens were higher in patients with syringomyelia and included weakness, extremity numbness, neck pain, dissociated sensory loss, and atrophy. Conclusion: There were no statistically significant differences in SS between the different CCJM types. BI patients had more swallowing and nystagmus complaints versus CM patients, but there were no significant differences in clinical SS between BI1 and BI2 patients. Notably, those with attendant syringomyelia had a higher SS burden.

Hypereosinophilic syndrome (HES) is a rare disorder characterized by a persistent eosinophilia with a multi-organ involvement including neurological manifestation. A 59-year-old man was referred from a neurosurgery unit with a spastic triparesis with predominant left side involvement, dissociated sensory loss to Th7, and metastasis-like lesions in a brain CT. MRI examination of the thoracic spine revealed an intraspinal T2-hyperintensive lesion with a subtle central gadolinium enhancement at Th4-Th8 level. MRI of the cervical spine showed a C1-Th1 long T2-hyperintensive lesion with a partial gadolinium enhancement and MRI of the brain revealed a large tumefactive T2-hyperintensive lesion in the right hemisphere. Blood tests showed an increased number of eosinophils (1790 cells/µl; 18.3%). Common causes of the eosinophilia were excluded. After corticosteroid treatment moderate neurological improvement was observed however in the brain MRI new T2-hyperintensive lesions were revealed. The patient was referred to the Department of Allergology and qualified for a treatment with mepolizumab, a monoclonal antibody against IL-5, with subsequent clinical and radiological improvement. To the best of our knowledge, this is the first case of hypereosinophilic syndrome with brain and spinal cord involvement treated with mepolizumab.

Objectives Despite its relative common occurrence, definitive diagnosis of small fiber neuropathy (SFN) remains problematic. In practice, patients with pain, numbness, and/or paresthesias in their lower limbs are diagnosed with SFN if found to have dissociated sensory loss in their feet, that is, impaired pinprick perception (PP) but relatively preserved vibration. We sought to assess the sensitivity and specificity of clinical examination and various diagnostic tools available for screening SFN. Methods Medical records of 56 patients diagnosed with SFN were reviewed. Diagnosis was based on symptoms, detailed neurological examination that included PP, and abnormal results on at least one testing modality-quantitative sudomotor axon reflex (sweat) test (QSART), quantitative sensory testing (QST), and heart rate variability (HRV) testing. Results Sensitivity of PP was relatively consistent between modalities of about 63% in presence of appropriate sensory symptoms. Laboratory testing diagnosed 88% of patients when both QSART and QST are employed. QST was most sensitive for detection of SFN with the heat-pain testing having higher sensitivity than cooling. Heart rate variability testing revealed low correlation across all groups. Conclusions The diagnostic yield for SFN increases by combining clinical features with various testing modalities. In symptomatic patients, we propose the following diagnostic criteria for diagnosis of SFN: Definite SFN-abnormal neurological examination and both QSART and QST; Probable SFN-abnormal neurological examination, and either QSART or QST; Possible SFN-abnormal neurological exam, QSART, or QST.

We present a retrospective study of 6 patients with spinal cord infarction in the territory of the Adamkiewicz artery. In all patients, the clinical picture was stereotyped: sudden onset of paraplegia and bilateral radicular pain, dissociated sensory loss below the level of infarction and sphincter dysfunction. Emergency neuroradiological investigation ruled out a compressive lesion in all cases. In one patient, spinal angiography was performed and identified an occlusion of the Adamkiewicz artery. Treatment was supportive and all patients had a substantial recovery over a period of weeks.

An 80-year-old woman was admitted to our hospital with acute onset of flaccid paraplegia and sensory and urinary disturbances that developed soon after acute pain in her lower back and leg. Neurological examination revealed, severe flaccid paraplegia, bladder and rectal disturbances and dissociated sensory loss below the level of L1 spinal cord segment. MR imaging with T2 weighted imaging (T2WI) and diffusion weighted imaging (DWI) on day 2 showed hyper signal intensity in the spinal cord at the vertebral level of L1 while initial apparent diffusion coefficient (ADC) showed decreased signal intensity in the lesion. We diagnosed spinal cord infarction, and anticoagulant and neuroprotective agents were administrated. Serial MRI findings revealed that the DWI signal of the lesion attenuated with time, and pseudo-normalization of the ADC occurred approximately 1 month after onset. These findings were similar to those seen in brain infarction. Our patient demonstrated serial MRI changes of spinal cord infarction showing anterior spinal cord syndrome.

The prevalence of symptomatic syringomyelia in patients with chronic spinal cord injury (SCI) is around 4.5%.1 It is an important cause of treatable neurologic deterioration, although surgical shunting of the syrinx or arachnoid adhesiolysis of tethered elements have unpredictable results.2 Classically, syrinx extension causes progressive neuropathic pain, often with dissociated sensory loss due to compression of the more central, spinothalamic projection neurons with relative preservation of dorsal column function. We report an unusual case of the total spontaneous resolution of an extensive cervicothoracic syrinx cavity in a patient with symptomatic posttraumatic syringomyelia (PTS) and describe the changes in contact heat-evoked potentials (CHEPs) associated with its development and disappearance.

A 64-year-old woman presented with left occipital headache and right dissociated sensory loss due to hematomyelia on the left ventral side of C1 caused by rupture of an aneurysm on one of the feeders extending from the anterior spinal artery to complex epidural or dural and intradural arteriovenous fistulas (AVFs). Branches from the left occipital and ascending pharyngeal arteries and those from the left C2 radicular, left posterior spinal and anterior spinal arteries formed these multiple shunts, linking with a common venous drain flowing into the right petrosal vein. Surgical interception of all the shunts was achieved, making it unnecessary to directly treat the aneurysm in the spinal cord. The feeders, aneurysm and AVFs were not visualized on postoperative angiography, and the patient returned to a normal working life.

Spinal extradural arachnoid cysts (ACs) have an infrequent predilection for the sacrum. As with their counterparts in other regions of the spine, cysts in this location are mostly asymptomatic. Common presentations in symptomatic cases include pain in the low back or perineum, radiculopathy, and sphincteric dysfunction. The authors report a hitherto undescribed presentation in which the predominant symptoms are those related to an associated holocord syrinx. This 15-year-old boy presented with fluctuating, spastic paraparesis and a dissociated sensory loss in the trunk. Admission MR imaging of the spine showed an extradural AC from S-2 to S-4 and a holocord, nonenhancing syrinx. The patient underwent S-2 laminectomy, fenestration of the cyst, and partial excision of its wall. Intradural exploration revealed a normal-looking filum terminale and the absence of any dural communication with the cyst. At a follow-up visit 6 months after surgery, his motor and sensory deficits had resolved. Follow-up MR imaging showed complete resolution of the syrinx in the absence of the sacral AC. This is the first report of a sacral extradural AC causing holocord syringomyelia. Because conventional theories of syrinx formation were not helpful in elucidating this case, a hypothesis is postulated to explain the clinicoradiological oddity.

Background Spinal cord infarction from anterior spinal cord syndrome (ASAS) in children is a rare pathology and comprises the following clinical symptoms: sudden onset of pain and flaccid para- or tetraparesis, bladder dysfunction, and dissociated sensory loss with impairment of pain and temperature perception. Deep sensibility is not affected. Patient A 13-year-old male patient presented to our emergency department with a bilateral leg weakness. 1 week before, he had suffered a leg strain in a Taekwondo-fight from which he recovered completely. On physical examination our patient's legs were in flaccid paralysis, tone was decreased and he had dissociated sensory loss and acute retention of urine. Blood count, ESR, electrolytes, serologic tests for various pathogens and CSF examination all were normal. However, tests for values of an acute endothelial lesion were increased and he was a homozygous carrier of MTHFR-polymorphism. MRI performed on the day of admission was normal but showed dramatic changes 2 days later with increased signal intensity in the ventral aspect of the spinal cord, characteristic for an ASAS. Treatment included highdose methylprednisolone, a suprapubic bladder catheter, sufficient anticoagulation and a rapid transfer to a rehabilitation centre. Discussion We assume that a combination of the patient's prothrombotic risk factor (MTHFR-polymorphism with elevated homocysteine levels) and his trauma in the taekwondo-fight with consecutive vessel injury caused an occlusion of the artery by late emboli or a growing thrombus.

We present a case which demonstrates the classical clinical symptoms and signs evident in an anterior spinal cord syndrome and explains the anatomical basis for the features seen with respect to the ascending and descending tracts in the spinal cord. It also demonstrates the clinical importance in conducting a detailed sensory examination to look for dissociated sensory loss and ascertaining the level of the pathology within the cord. The patient had made improvements following over a month of extensive rehabilitation on a specialist stroke unit.

Sensory neuropathy usually impairs tactile sensations related to large myelinated afferents (Abeta) as well as thermal-pain sense related to small myelinated (Adelta) and unmyelinated (C) afferents. By selectively affecting large or small fibres, some sensory neuropathies may also provoke a dissociated sensory loss. Standard nerve conduction studies and somatosensory evoked potentials assess Abeta-fibre function only. Laser pulses selectively excite free nerve endings in the superficial skin layers and evoke Adelta-related brain potentials (LEPs). From earlier studies and new cases we collected data on 270 patients with sensory neuropathy. LEPs often disclosed subclinical dysfunction of Adelta fibres and proved a sensitive and reliable diagnostic tool for assessing small-fibre function in sensory neuropathy.

The cortically generated P60 component of the posterior tibial nerve somatosensory evoked potential (PTSEP) is occasionally found to be absent in neurological patients,while the preceding P40 is preserved ("Absent P60" pattern). A retrospective analysis of 24 such cases showed them to represent a different clinical population from that represented by 24 age- and sex-matched but otherwise unselected patients with entirely normal PTSEPs. The most frequent diagnoses of the patients with normal PTSEPs (conversion disorder and definite or suspected multiple sclerosis) were significantly less prevalent in the patients with the Absent P60 pattern, while miscellaneous other diseases affecting the peripheral and/or central sensory pathways were more frequent. In comparison with a second matched patient group with abnormal P40 in addition to P60, the patients with the Absent P60 pattern had a significantly lower incidence of "large fibre" sensory deficits (impaired vibration and/or joint-position sense). The incidence of "small fibre" deficits (impaired pain and/or temperature sensation) was similar in both groups with PTSEP changes. In conjunction with previously published findings in normal subjects, the data suggest that the P60 is a late response of the primary sensorimotor cortex due to activation of large diameter myelinated sensory fibres, but which is also tonically influenced by small fibre input. The Absent P60 pattern may be recognized as a distinct PTSEP abnormality, although its occurrence in some normal individuals should be noted.

The anterior spinal artery (ASA) has multiple anastomoses along its path and therefore spinal stroke due to ASA occlusion is a rare event. Following an ASA occlusion, collateral blood flow, although inadequate to meet the needs of the spinal cord, may be enough to prevent a sudden stroke and instead may result in a gradual loss of cord function. The etiologies are mostly embolic, inflammatory, and traumatic. Atherosclerosis and resultant stroke is very rare in the ASA. The acute ASA syndrome is characterized by bilateral flaccid paresis or plegia below the lesion level, loss of motor reflexes, and dissociated sensory loss. Treatments are focused on the prevention of secondary ischemic events and on management of complications related to spinal cord dysfunction.

AIMS Clinical observation has led to the idea that there might be a distinctive form of selective sensory and autonomic neuropathy affecting patients with Type 1 diabetic mellitus with severe symptomatic autonomic neuropathy (Type 1-DAN) and this study was conducted to evaluate the presence of such a neuropathy in Type 1-DAN. METHODS Nineteen Type 1 diabetic patients presenting for treatment of severe symptomatic autonomic neuropathy were examined (all had > or = 2 autonomic symptoms; age 39.3 +/- 10.2 years; duration of disease 25.6 +/- 10.5 years). For comparison, 19 Type 1 diabetic patients with neuropathic foot ulcers (age 44.5 +/- 6.6 years; duration of disease 26.7 +/- 9.2 years), 14 clinically uncomplicated Type 1 diabetic patients (age 39.9 +/- 5.6 years; duration of disease 22.9 +/- 9.3 years) and 16 non-diabetic healthy people as controls (age 39.3 +/- 10.7 years) were also examined. Results The large fibre modalities (light touch and vibration perception) were better preserved in the Type 1-DAN group than in the foot ulcer group. Thus, light touch sensation was normal in 11 out of 19 Type 1-DAN patients compared to only three out of 19 foot ulcer patients (P < 0.01), and vibration perception was 24.9 +/- 15.0 V and 40.5 +/- 7.9 V, respectively (P < 0.002) with some of the Type 1-DAN patients in the normal range. In contrast, the small fibre modalities, thermal perception and autonomic function, were grossly abnormal in both groups (hot thermal perception 14.1 +/- 2.5 degrees C and 12.6 +/- 3.7 degrees C; cold thermal perception 13.8 +/- 2.7 degrees C and 10.9 +/- 4. 7 degrees C; heart rate variation 2.9 +/- 1.5 beats/min and 4.8 +/- 4.0 beats/min, respectively). CONCLUSIONS There is indeed a subgroup of Type 1 diabetic neuropathy patients who suffer from severe autonomic symptoms associated with a selective small fibre sensory and autonomic loss with relatively preserved large fibre sensory modalities.

Objectives : Cortical potentials evoked by carbon dioxide laser pulses have been applied in clinical practice to study nociceptive pathways for several years. In this study, we evaluate the properties of an infrared laser (thulium-YAG) with a penetration depth in the skin that matches the intracutaneous depth of nociceptors. Methods : Temperature measurements and modelling showed that the thulium laser generates painful intracutaneous temperatures with less surface heating than the carbon dioxide laser and with no side effects (up to 600 mJ pulse energy). To develop clinical evaluation criteria, laser-evoked potentials (LEPs) were recorded from 3 midline positions (Fz, Cz, Pz) versus linked earlobes in 23 healthy subjects. Within a session, two skin areas were studied twice in a balanced sequence using randomized interstimulus intervals and two intensities in randomized order. Results : After hand and foot stimulation with 540 mJ pulses, all subjects showed reproducible biphasic vertex potential, consisting of a negativity (hand: 210 ms, foot: 250 ms) and a positivity (hand: 330 ms, foot: 380 ms). Mean habituation of the vertex potential amplitude across runs was 25% (hand) or 16% (foot); due to the balanced sequence it did not affect the other comparisons. Following foot stimulation, peak latencies were significantly longer (by 40–50 ms) and amplitudes were significantly smaller than following hand stimulation (22.5±6.7 vs. 30.3±10.9 μV, mean±SD). Using 2.5 standard deviations from the mean as a cut-off, absolute normative values were determined for peak latencies and amplitudes. In addition, relative normative values were determined for paired comparisons (hand–hand, foot–foot, hand–foot). Conclusions : The thulium-YAG laser is a useful tool for assessment of impaired pain sensitivity. Representative case reports illustrate that unlike for early SEP components, the most frequent LEP abnormalities were amplitude differences.

Laser pulses selectively excite mechano-thermal nociceptors and evoke brain potentials that may reveal small-fiber dysfunction. We applied CO2-laser pulses to the perioral and supraorbital regions and recorded the scalp laser-evoked potentials (LEPs) and reflex responses in the orbicularis oculi, masticatory, and neck muscles in 30 controls and 10 patients with facial sensory disturbances. Low-intensity pulses readily evoked scalp potentials consisting of a negative component with a latency of 165 ms followed by a positive component at 250 ms. In vertex recordings, the amplitude of LEPs exceeded 30 microV. Although only high-intensity pulses evoked reflex responses, some subjects showed--even to low-intensity pulses--an orbicularis oculi (blink-like) response that markedly contaminated the scalp recording. Scalp LEPs were abnormal in patients with hypalgesia and normal trigeminal reflexes and normal in patients with normal pain sensitivity and abnormal trigeminal reflexes. Possibly because of the high receptor density in this area and the short conduction distance, laser stimulation of the trigeminal territory yields low-threshold and large LEPs, which are useful for detecting dysfunction in peripheral and central pain pathways.

We studied 25 patients with an acute thalamic stroke (infarct or hemorrhage) on CT or MRI scan and sensory dysfunction, among the 3,628 patients with first-time stroke included in the Lausanne Stroke Registry. Twelve patients had a right-sided infarct, 11 a left-sided infarct, and 2 a left-sided thalamic hemorrhage. Sensory symptoms or signs were the only clinical abnormality. The presumed causes of stroke were small artery disease in 21 patients including both cases of hemorrhage, emboligenic heart disease in 2, while the etiology of ischemic stroke was undetermined in 2 patients. Nine patients had a loss of all modalities of sensation with faciobrachiocrural distribution, 5 patients suffered dissociated sensory loss with faciobrachiocrural distribution and 11 patients showed a dissociated involvement of sensation with a partial distribution pattern. The inferolateral region (thalamogeniculate arteries) was involved in all patients. Six patients complained of pain and/or dysesthesias during the stroke; 5 of them had involvement of the nucleus ventrocaudalis (in 3 with damage to the nucleus ventro-oralis intermedius, and in one to the pulvinar) and 1 patient had involvement of the nucleus ventro-oralis intermedius. Eighteen patients complained of paresthesias in the contralateral part of the body; 16 of them had involvement of the nucleus ventrocaudalis (in 4 with damage to the nucleus ventro-oralis intermedius, in 1 with damage to the nucleus ventro-oralis intermedius, and nucleus ventro-oralis externus, and in one with damage to the nucleus parvocellularis and pulvinar). Four patients developed delayed pain and/or dysesthesias; all of them had involvement of the nucleus ventrocaudalis (in 1 with damage to the nucleus parvocellularis and pulvinar). Time lag from stroke onset to developing pain ranged from 2 to 15 days (mean 10.5 days). One patient with dissociated involvement of sensation with a partial distribution pattern had paresthesias and dissociated hemisensory loss involving position sense without pain and temperature sensations. This patient had involvement of the posterolateral part of the nucleus ventrocaudalis. In conclusion, sensory dysfunction and delayed pain are more often found in thalamic lesions that involve the nucleus ventrocaudalis, and nucleus ventro-oralis intermedius. Restricted sensory abnormalities correlate with very small lesions located in critical areas within these nuclei.

We report on a 34-year-old man, who is a wheel-chair bound T4 level paraplegic following a roadtraffic accident 16 years ago. He presented to ouraccident and emergency department with defor-mity and bruising around the right shoulder. Thishad occurred after he heard a ‘‘crack’’ whilst trans-ferring to his bed from his wheel-chair with thesupport of his arm.Radiographsrevealedadisplacedfractureofthescapular body and neck. The appearances weresuggestive of a high energy injury (Fig. 1). Radio-graphs of the chest done 4 months prior to pre-sentation showed an intact scapula, furthersuggesting a fresh fracture. A Computerised Tomo-graphy (CT) scan was arranged to better define thefracture configuration and displacement of thefracture fragments. Despite this injury pattern,hehadlittlediscomfortin hisshoulder. Hehadwellmuscledupperextremitiesandhadnomotorweak-ness. He did not complain of any numbness, and hehad normal sensation to light touch in his upperlimbs.Surgical fixation of the scapular fracture wascarried out (Fig. 2) and the fracture healed inabout 4 months following surgery. Our patientdid not have the expected post-operative pain,which alerted us to the possibility of a dissociatedsensory loss. Further questioning and examinationelicited blunting of pain and temperature sensa-tions in both upper limbs and on examination,there was evidence of dissociated sensory loss inboth the upper limbs. We decided to investigatethis further and he underwent a Magnetic Reso-nance Imaging scan (MRI) of his cervical and thor-acicspinalcord.Thisrevealedasyrinx-lesionofthecervical and upper thoracic spinal cord. The dis-sociative sensory loss was due to the syrinx. Thesyrinx extended from the level of the first cervicalvertebra to the lower border of the first thoracicvertebra (Fig. 3).Our neurosurgical colleagues were consulted,and a decompression of the syrinx is beingconsidered.

A 21-year-old right-handed man, with no medical history of note, presented to neurosurgery outpatient department in Pakistan, with a 5-year history of numbness and pain in his upper limbs. The patient gave a troubled account of constant, current-like pain, tracking down his arms and sometimes over his chest, which frequently disturbed his sleep. The numbness he experienced made it difficult to turn pages of the newspaper. Upon further questioning, he also admitted to suffering with occipital headaches for a number of …

A marked, left-sided segmental hyperhidrosis, verified by quantitative evaporimetry, corresponded to an area of dissociated sensory loss in 2 siblings (male 49 years old, female 47 years old). MRI scans of the head and whole cord demonstrated a Chiari type I malformation in both. In the male, there was a syrinx of the neck. The level of this did not correspond to the hyperhidrosis and dissociated sensory loss areas of the trunk and thigh. The female, who had no syrinx, had hyperhidrosis and dissociated sensory loss of the head, neck, upper trunk, and arm, and anisocoria with the larger pupil ipsilateral to the sweating. This rare disorder may be due to a disinhibition of the preganglionic sympathetic neurons caused by cord lesion(s) interrupting inhibitory descending and spinal pathways which are important for the normal thermoregulatory sweating.

The recognition that dissociated sensory loss affecting selectively pain and temperature results from lesions of the operculo-insular cortex is due to Biemond in 1956. This contrasted with the prevailing view that the sensory aspects of pain did not imply regions above the thalamus. Anatomical data in non-human primates, as well as electrophysiology and functional imaging in humans have now abundantly demonstrated that the opercular–insular region is the main cortical target of the spinothalamic system, and a vast number of reports have confirmed the relation between lesions in this region and the development of dissociated sensory symptoms and central neuropathic pain. Operculo-insular pain (parasylvian pain) is a distinct entity that can be clinically suspected and objectively diagnosed with combined radiological and electrophysiological methods, in particular evoked potentials to spinothalamic (laser) input. The region comprising the posterior insula and medial operculum may deserve being considered as a third somatosensory cortex (S3) contributing to the spinothalamic attributes of somatic perception.

Background: A presentation of all aspects of the dorsolateral medulla oblongata syndrome is clinically very rare to find. In most cases patients present with fragmentary symptoms, e.g. ipsilateral axial lateropulsion, nystagmus, dysarthria, dysphagia or hemiataxia. However, the clinical presentation and lesion anatomy at the level of the medulla oblongata is still unsatisfactory. The aim of this study was to correlate the functional deficit with structural MRI-data. Methods: We included thirteen patients (eight male, five female, mean age 65.5) with medulla oblongata infarction with clinically predominant ipsilateral axial lateropulsion and correlated clinical with structural deficits. Results: Magnetic resonance imaging lesion mapping demonstrated ipsilateral axial lateropulsion to result from lesions of the spinocerebellar tract, the inferior cerebellar peduncle or the inferior vestibular nucleus. Nystagmus was associated with lesions of the inferior vestibular nucleus, dissociated sensory loss with the spinothalamic tract and hemiataxia with the spinocerebellar tract. Conclusions: Correlating dysfunction and lesion anatomy is a promising approach to enhance our knowledge on medulla oblongata topography.

Brief heat stimuli, elicited by a CO2 laser (10.6 microns wave length), activate the most superficial cutaneous nerve terminals of the thin myelinated A delta and unmyelinated C fibres which mediate heat and pain sensations. This paper investigates late cerebral potentials (SEPs) in response to laser pulses in comparison with those to conventional electrical stimulation in 18 patients with a dissociated sensory deficit (intact mechano-sensibility and disturbed temperature and pain sensation). Patients were stimulated in the most disturbed limb (affected area) and in a corresponding control area. In all 18 patients the SEPs elicited by laser stimuli were able to identify the body site with heaviest disturbances in pain and thermo-sensibility: the SEPs from the affected area were reduced or delayed, compared to the control area. In contrast, no alterations in SEPs could be observed after conventional electrical nerve stimulation, in agreement with the normal mechano-sensibility. However, the degree of SEP modulation in response to cutaneous heat stimuli did not correspond to the severity of the subjectively reported sensory deficit. Highest correlations between sensory deficits and abnormal SEPs were found in all those patients in whom computer tomography or MR imaging documented a localized destructive process in the CNS. All patients with the smallest SEP modulations despite a considerable sensory deficit had an inflammatory aetiology. Preliminary criteria to define a laser-evoked SEP as pathological are discussed.

Patients with syringomyelia are usually diagnosed by its characteristic symptoms and signs including dissociated sensory loss, muscle weakness and atrophy of hands and arms, pyramidal tract signs in the lower extremities and disturbed autonomic nerve function. It is difficult to diagnose cases lacking in these classical clinical features, especially in those lacking sensory deficit. Recently developed MRI, however, has been reported to reveal the spinal cavitation in abortive cases of syringomyelia. We found a 49 year-old female who showed no sensory disturbance in spite of having a large syrinx demonstrated by MRI.Her neurological findings were as follows : absent or diminished deep reflexs in the upper extremities and hyperactive reflexes in the lower extremities with positive extensor plantar responses : muscle weakness and atrophy of hands and forearms with occasionally observed fasciculation ; and mild thoracic scoliosis. Sensations to touch, pain and temperature were preserved as well as deep sensation. MRI clearly delineated a large syrinx and swollen spinal cord below the third cervical segment with a narrowed subarachnoid space. Chiari-Arnold type 1 malformation was also demonstrated. Myelography and CT myelography with metrizamide confirmed the MRI findings.Her neurological findings might have led us to diagnose her as motor neuron disease if the neuroradiological examinations were not performed. Therefore, it should be noted that because the diagnostic importance of sensory loss in syringomyelia is stressed, a patient without definite sensory loss is apt to be misdiagnosed as motor neuron disease. It is essential to differentiate the two disorders, since a shunt operation is known to improve the neurological deficits in syringomyelia while motor neuron disease is still a fatal disorders. For this purpose, our case clearly showed the diagnostic usefulness of MRI.It is generally accepted that dissociated sensory loss in syringomyelia is caused by disruption of the ventral white commissure distended and compressed by a expanded syrinx. Although the syrinx seemed large enough to damage the commissure in the present case, there was no sensory disturbance. Several authors have recently reported that there is no significant relationship between the neurological deficit and the dimensions of the syrinx. This means that the clinical symptoms and signs are not produced simply by intraspinal tissue being directly damaged by the expanded syrinx. The effects of disturbed circulation secondary to the compression, the difference in sensitivity to the ischemia or the compression, the rate of growth of the syrinx, and the compensatory or reserve capacity of the tissue should be all considered.

Pure intramedullary spinal-cord metastases (ISCM) are a rare manifestation of cancer. We report a case of ISCM from gastric cancer. A 68-year-old man, treated with total gastrectomy for a gastric cancer, presented 9 months later with paresis of the left arm, pain and dissociated sensory loss. Magnetic resonance imaging revealed a pure intramedullary lesion at the C3-C5 level. After surgical resection, pathological findings revealed an undifferentiated adenocarcinoma of gastric origin. To our knowledge, this is only the second report of ISCM from gastric cancer in the literature.

Background Anterior spinal artery (ASA) syndrome results in motor palsy and dissociated sensory loss below the level of the lesion, accompanied by bladder dysfunction. When the cervical spine is involved, breathing disorders may be observed. Objective To describe the polysomnographic findings in a patient with cervical ASA syndrome complicated by a sleep breathing disorder. Setting Unit of neurology at a sleep center. Patient A 30-year-old man had an ischemic lesion that affected the anterior cervical spinal cord (C2-C6) bilaterally because of an ASA thrombosis. He developed ASA syndrome associated with respiratory impairment during sleep. Results The polysomnographic study during sleep showed a severe sleep disruption caused by continuous central apneas that appeared immediately after falling asleep. Treatment by intermittent positive pressure ventilation normalized the respiratory pattern and sleep architecture. Conclusions The sleep breathing pattern was compatible with central alveolar hypoventilation due to automatic breathing control failure caused by a lesion of the reticulospinal pathway, which normally activates ventilatory muscles during sleep. This autonomic sleep breathing impairment resembles that found as a complication in patients who undergo spinothalamic tract cervical cordotomy for intractable pain. This surgical complication is known as the Ondine curse.

We describe a patient who has been complaining of brief jerk-like, rhythmic, involuntary movements involving the second digit of the left hand for the last three months. These involuntary jerks produced an adduction movement of the second digit and were unaffected by peripheral sensory stimuli. In addition, the patient experienced loss of dexterity in the left hand. On examination the patient showed hypotrophy of the first dorsal interosseous (FDI) muscle of the left hand and a dissociated sensory loss involving the C8-T1 dermatomes. Magnetic resonance imaging of the brain and spinal cord revealed a tonsilar hemiation characteristic of the Chiari I malformation associated with a syrinx extending from C4 to D5 that did not communicate with the fourth ventricle. The electrophysiological evaluation indicated the presence of a focal myoclonus of spinal origin that is likely to be caused by the syrinx.

The diagnosis of anterior spinal artery syndrome can be made with high accuracy by thorough clinical examination in combination with typical magnetic resonance imaging findings. Sudden onset of tetra- or paraparesis and dissociated sensory loss with bladder dysfunction are the leading clinical signs. We discuss clinical and radiologic findings in an adolescent presenting with anterior spinal artery syndrome. The laboratory results showed a hereditary protein S deficiency. (J Child Neurol 2001;16:134-135).

Objectives : Syringomyelia may present with confusing, unilateral patterns of segmental muscle involvement and dissociated sensory loss. The objective of this study was to report a patient with Chiari malformation type 1 (CM1) and syringomyelia who had an unusual presentation suggesting ulnar neuropathy at the elbow. Results : A 24-year-old woman presented with clinical evidence of ulnar neuropathy at the elbow except that there was disproportionate abductor digiti mini (ADM) atrophy and weakness, equivocal ipsilateral abductor pollicis brevis weakness and hyporeflexia in both arms. Nerve conduction studies revealed marked amplitude reduction of the left ulnar ADM-compound muscle action potential (ADM-CMAP) with a normal first dorsal interosseous-CMAP amplitude, no focal slowing or conduction block, and a normal ulnar sensory response amplitude. Electromyography (EMG) showed multi-segmental, left C7-T1 fibrillations and chronic reinnervation changes. Magnetic resonance imaging (MRI) of the cervical spine demonstrated CM1 and syringomyelia. Conclusions : Syringomyelia may clinically mimic ulnar neuropathy at the elbow.

Background —Various sensory syndromes in lateral medullary infarctions are described. A small variation in the location of a lesion may lead to very different clinical features, owing to the complex anatomy of the medulla oblongata. MRI may identify the location and extent of the ischemic lesions, allowing a clear clinical-anatomical correlation. Case Description —We describe a man with an ischemic lesion in the right portion of the lower medulla that presented a contralateral impairment of spinothalamic sensory modalities and an ipsilateral impairment of lemniscal modalities with a restricted distribution (left forearm and hand, right hand and fingers, respectively). The restricted and dissociated sensory abnormalities represent the only permanent neurological consequence of that lesion. Conclusions —The atypical sensory syndrome may be explained by the involvement of the medial portion of spinothalamic tract and the lateral portion of archiform fibers at the level of the lemniscal decussation.

We report a new syndrome that we call "recurrent optic neuromyelitis with endocrinopathies" in eight Antillean women from Martinique and Guadeloupe Ocular involvement was either monocular or binocular, whereas myelopathy was acute or subacute. In seven patients, myelopathic symptoms recurred, and in six patients, visual problems recurred. Spinal cord involvement was a consistent band-like pseudo-syringomyelic dissociated sensory loss. All eight patients had endocrinopathies consisting of amenorrhea, galactorrhea, diabetes insipidus, hypothyroidism, or hyperphagia. Spinal cord MRI revealed cavitation-like images. Various immunosuppressant treatments had little effect on the uniformly deteriorating course, ending in blindness and paraplegia. Six patients died within 5 years of onset, and an autopsy in one patient showed multiple demyelinizing lesions of the spinal cord with thickened blood vessels walls without evidence of inflammation. These cases appear to constitute a syndrome distinct from MS and from classic Devic's syndrome, not only because of the association with endocrinopathies but because of the stereotypy of the recurrences, the absence of MRI lesions in the cerebral white matter, and the unusual image of cavitation of the spinal cord. The syndrome is also distinct from HTLV-I-associated paraparesis, which is endemic in the West Indies.

Six of 48 stroke patients had functionally limiting dysaesthesiae induced by repetitive light touch, joint movement, or neuromuscular electrical stimulation (NMS). Only one of these six patients had a thalamic lesion. Quantitative sensory testing showed substantial impairment of pain and temperature sensation in all six patients, whereas light touch, vibration and position sense, and graphaesthesia were normal (three patients) or relatively spared (three patients). By contrast, none of 15 stroke patients in whom NMS did not evoke dysaesthesiae had clinical evidence of dissociated sensory loss. Conscious perception of joint movement and light touch is mediated mainly by the same population of large myelinated fibres activated preferentially by low intensity electrical stimulation. It is suggested that activation of these non-nociceptive, presumably dorsal column, afferents may contribute to dysaesthesiae in some patients with sensory loss after stroke.

Three patients with symptoms due to the anterior spinal artery syndrome were treated by direct perfusion of dexamethasone sodium phosphate and urokinase into the artery of Adamkiewicz. Their symptoms were paraparesis with dissociated sensory loss and sphincter dysfunction, and there was no evidence of the possible cause. In the early phase of the disease, three consecutive injections were carried out with an interval of a week between each. All the patients made a good recovery.

Three male and two female patients with anorgasmia and dissociated sensory loss due to an anterior spinal cord syndrome are described. Clinical, neurophysiological and quantitative sensory evaluation revealed preservation of the large fibre dorsal column functions from the lumbosacral segments with concomitant severe dysfunction or absence of the small fibre neospinothalamic mediated functions. These findings indicate a role for the spinothalamic system in orgasm.

To the Editor: In a recent article published in Stroke , Cerrato et al1 described a patient with right lateral medullary infarction (LMI) who presented with abnormal spinothalamic sensation in the left arm and impaired dorsal column sensation in the right hand. Although this is an interesting observation, I have several concerns about this report. First, the authors wrote that “dissociated sensory pattern involving upper limbs has not yet been reported.” However, I previously described 3 patients who had ipsilateral dorsal column sensory impairment who had otherwise typical symptoms/signs of LMI.2 Actually, one of them (case 3) showed exactly identical sensory pattern as described by Cerrato et al: dissociated sensory impairment limited to upper extremities. I suggested that involvement of the fibers at the nucleus gracilis/cuneatus or the crossing fibers …

Syringomyelia is defined as a chronic, progressive degenerative disorder of the spinal cord characterised by dissociated sensory loss and brachial amyotrophy with pathological evidence of central cord cavitation 1 . When this cavity extends into the medulla, it is called syringobulbia 1 . The expanding cavity gradually destroys the second order neurones of spinothalamic tracts, anterior nerve cells and lateral corticospinal tracts 1 .

A. Gass, MD, T. Back, MD, S. Behrens, MD, A. Maras, MD, Mannheim, Germany A 55-year-old woman experienced lower back pain and over the next 3 hours developed a flaccid paraplegia, sphincter paresis, and dissociated sensory loss distally to the level of L1. There was slight bilateral hypesthesia, but sensation of light touch was largely preserved. T2-weighted MRI 12 hours after symptom onset showed a hyperintense lesion in the central …

• A 59-year-old man had loss of pinprick and temperature sensation over his head, face, neck, trunk, and all extremities while light touch, vibration, joint position sense, and deep pain were preserved. This was the cumulative result of infarctions in the territories of the right superior cerebellar and left posterior inferior cerebellar arteries that occurred three years apart. To our knowledge, a syndrome of bilateral discrete interruption of spinothalamic tracts and of the spinal tracts of the trigeminal system in the brain stem has not been reported. Dissociated sensory loss enveloping the entire body accompanied by truncal and limb ataxia without weakness demonstrated a striking clinical picture. The preservation of deep somatic and visceral pain when cutaneous pain sensation was lost was another notable feature. We review the causes of widespread dissociated sensory loss and discuss the implications of the dissociation of deep from superficial pain.

Two patients with cervical spondylotic myelopathy presented with features suggesting syringomyelia. One patient had a Charcot joint of the wrist, and both had dissociated sensory loss in the upper limbs. The possible vascular basis of these central cord lesions is discussed.

A patient sustained C7-C8 incomplete myelopathy with dissociated sensory loss after a whiplash injury. Cervical radiograms showed no fracture or dislocation but separation of the C4-C5 and C5-C6 spino