Craniocervical Magnetic Resonance Imaging Research Articles

Chiari type 1.5 malformation as a cause of secondary trigeminal neuralgia: case report and literature discussion

BackgroundChiari syndrome is a very rare cause of secondary trigeminal neuralgia (TN). There are a few cases of TN associated with Chiari syndrome in the literature, and all of these cases were reported as Chiari type 1. In this report, we present a case of secondary trigeminal neuralgia caused by Chiari type 1.5 for the first time unlike the literature.Case presentationA 38-year-old male patient, who had frequent and severe attacks of pain under the orbit, at the chin and rim of the mouth, was evaluated with craniocervical Magnetic Resonance Imaging (MRI) and revealed Chiari type 1.5 malformation and syringomyelia.ConclusionsThe mechanism of TN associated with Chiari malformation is unclear. However, the main concepts emphasized in cases presented in the literature are compression and stretching. The rapid relief of pain after decompression surgery in these cases also supports this situation. It can be predicted that compression and tension forces will become more pronounced in Chiari type 1.5 malformation, in which the brainstem elongation and ventral pressure are more prominent. Decompression of the foramen magnum provides rapid and effective pain control in the treatment of TN accompanying Chiari 1.5 syndrome that does not respond to medical treatment.

Value of 3T craniocervical magnetic resonance imaging following nonfatal strangulation.

The aims of this study were (1) to provide an overview of craniocervical magnetic resonance imaging (MR) findings following nonfatal strangulation (NFS), (2) to detect the time dependency of the presence of these findings, and (3) to explore the additional value of MR with regard to the forensic interpretation of NFS. All 633 victims of manual strangulation between October 2011 and March 2018 were examined, including the case history and external findings. Following written consent, 114 cases were included in the study. The duration between the event, clinical forensic examination, and MR was noted. Radiologic images were reviewed by a clinical and a forensic radiologist. The case group consisted of 90 women and 24 men with a mean age of 32.5years. Delimitable external findings were present in 93% (N = 106) of cases. MR yielded a positive finding in 43% of cases (N = 49). There was no significant difference in the mean time interval between examinations between MR-positive and MR-negative cases. Perilaryngeal fluid accumulation was associated with difficulty swallowing and victims put in a chokehold. All cerebral MR were unremarkable, except for one patient with edema of the corpus callosum. The role of craniocervical MR following NFS is currently limited, particularly with regard to the forensic interpretation of NFS. MR may reveal internal injury in victims who report subjective symptoms of airway compression and in those who were placed in a chokehold. The presence of MR findings is not dependent on immediate examinations following the assault. • Magnetic resonance imaging does not currently provide additional value for the estimation of the severity of nonfatal manual strangulation. • Magnetic resonance imaging of the neck may reveal internal injury in cases without external findings, particularly in victims placed in a chokehold and with symptoms of airway compression. • The incidence of carotid artery dissections and laryngeal fractures is low in victims of nonfatal manual strangulation.

Investigation of sensory thresholds in Cavalier King Charles Spaniels with and without Chiari-like malformations and syringomyelia.

BackgroundCavalier King Charles Spaniels (CKCS) suffer pain associated with Chiari‐like malformation and syringomyelia (CMSM). People suffer from a similar condition and describe numerous sensory abnormalities. Sensory changes have not been quantified in affected CKCS.ObjectivesTo use quantitative sensory testing (QST) to quantify thermal and mechanical thresholds in CKCS and to compare QST in dogs with and without syringomyelia (SM).AnimalsForty‐four CKCS.MethodsProspective study. Dogs underwent neurological examinations and craniocervical magnetic resonance imaging (MRI). Thermal testing was performed over the humerus and thorax (n = 32); mechanical testing was performed on the paw and neck (n = 44). Latencies, thresholds, and response rates were compared with presence and severity of SM on MRI, presence of pain reported by the owner and pain identified on examination.ResultsThirty dogs had SM, 30 were painful on examination, 29 were owner‐reported symptomatic. Thermal and mechanical variables were not significantly different based on presence or severity of SM. Dogs with pain on examination had decreased mechanical thresholds on the paw (0.38 kg, SD = 0.18) and neck (2.05 kg, SD = 0.74) compared to thresholds of dogs without pain on examination on the paw (0.60 kg, SD = 0.30) and neck (2.72 kg, SD = 0.57; P = .021).Conclusions and Clinical ImportanceMechanical and thermal sensitivity does not appear to be related to the presence of SM, but mechanical sensitivity appears to be related to the presence of pain and clinical signs. Mechanical testing may be useful for assessing sensory abnormalities during clinical trials.

Questionnaire-based Analysis of Owner-reported Scratching andPain Signs in Cavalier King Charles Spaniels Screened for Chiari-like Malformation and Syringomyelia.

BackgroundChiari‐like malformation (CM) and syringomyelia (SM) cause a pain syndrome in Cavalier King Charles spaniels (CKCS). Clinical signs are not consistently apparent on neurologic examination, and owner reporting of signs provides vital clinical history. However, owner questionnaires for this disease are not well developed.ObjectivesTo develop a tool to capture owner‐reported clinical signs for use in clinical trials and to compare owner‐reported signs with the presence of pain on neurologic examination and SM on magnetic resonance imaging (MRI).AnimalsFifty client‐owned CKCS.MethodsOwners completed a questionnaire and pain/scratch map. Each dog underwent a neurologic examination and craniocervical magnetic resonance imaging (MRI). Questionnaire responses were developed into scores, area of shading for pain/scratch maps was measured, and consistency of responses between these tools was assessed. Owner‐reported findings were compared with neurologic examination findings and presence and severity of SM on MRI.ResultsThirty‐three dogs were symptomatic and 17 asymptomatic; 30 had SM. The most common sign of pain was crying out when lifted (n = 11). Extent of shaded areas on maps positively correlated with questionnaire scores for pain (r 2 = 0.213, P = 0.006) and scratch (r 2 = 0.104, P = 0.089). Owner‐reported findings were not significantly associated with presence or severity of SM or neurologic examination findings. Owner‐reported lateralization of signs was significantly associated with SM lateralization (P < 0.0001).ConclusionsThe questionnaire and maps may be useful for clinical trials. Lack of association of owner‐reported signs with SM highlights our lack of understanding of the pathophysiology of pain in this disease.

Ruptured spinal artery aneurysm associated with coarctation of the aorta

A 55-year-old woman presented with unconsciousness and tetraparesis. Routine blood tests were normal. Computed tomography (CT) of the brain showed ventricular hematoma and associated mild hydrocephalus (Fig. 1). Urgent external ventricular drainage was performed. Craniocervical magnetic resonance imaging showed an intradural paramedullary lesion primarily consistent with an aneurysm at the C6 level. Diffuse spinal hematoma and aneurysm was also seen on CT (Fig. 2). Computed tomography angiography showed coarctation of the aorta (Fig. 3). Spinal angiography showed coarctation of aorta with a ruptured, 10-mm, dissecting spinal artery aneurysm (SAA). The aneurysm was occluded successfully with endovascular coiling (Fig. 4). Fig. 2Sagittal T2-weighted cervical MRI and sagittal reformatted CT images show a mass lesion (black arrows) located anteriorly at the level of the C6 vertebra. The lesion compressed the spinal cord. Diffuse spinal hematoma was also noted on CT as well as MRI (white arrows). Cervical spinal cord hyperintensity represents myelomalacia. View Large Image Figure Viewer Download Hi-res image Fig. 3Computed tomography angiography section shows coarctation of the aorta (arrow). View Large Image Figure Viewer Download Hi-res image Fig. 4(Left) Angiography shows spinal artery aneurysm with coarctation of the aorta. Red arrow indicates the radiculomedullary artery. Green arrow indicates the aneurysm. Black arrow indicates the anterior spinal artery. Blue arrow indicates the collateral flow that supplies the thoracic aorta distally from coarctation. (Middle) Microcatheter injection of the related aneurysm. (Right) Angiography shows occlusion of the aneurysm (arrow) with endovascular coiling. View Large Image Figure Viewer Download Hi-res image

Primary Diffuse Leptomeningeal Gliomatosis Mimicking Tuberculous Meningitis

Primary diffuse leptomeningeal gliomatosis is a disease with an aggressive course that can result in death. To date, 82 cases have been reported. Here, the case of a 3-year-old male patient presenting with strabismus, headache, and restlessness is reported. Physical examination revealed paralysis of the left abducens nerve, neck stiffness, and bilateral papilledema. Tuberculous meningitis was tentatively diagnosed, and antituberculosis treatment was initiated when cranial imaging revealed contrast enhancement around the basal cistern. Craniocervical magnetic resonance imaging (MRI) was performed when there was no response to treatment, and it revealed diffuse leptomeningeal contrast enhancement around the basilar cistern, in the supratentorial and infratentorial compartments, and in the spinal region. Primary diffuse leptomeningeal gliomatosis was diagnosed by a meningeal biopsy.

Type I Chiari malformation presenting central sleep apnea

Sleep apnea is a rare but a well-known clinical feature of type I Chiari malformation. It may be obstructive or central in nature. Sleep apnea in patients with type I Chiari malformation rarely presents without accompanying neurological signs or symptoms. We here report a case of a 10-year-old girl who presented with central sleep apnea without any other neurological signs but was ultimately diagnosed with type I Chiari malformation. The patient initially showed mild improvement in symptoms after administration of an acetazolamide. Finally, posterior fossa decompression dramatically improved her respiratory status during sleep, both clinically and on polysomnography. This case suggests that type I Chiari malformation should be considered in the differential diagnoses of central apneas in children, even if there are no other neurological signs and symptoms. Furthermore, sagittal craniocervical magnetic resonance imaging may be necessary for a definitive diagnosis.

Infiltrative cervical lesions causing symptomatic occipital neuralgia

Occipital neuralgia is a well-recognized cause of posterior head and neck pain that may associate mild sensory changes in the cutaneous distribution of the occipital nerves, lacking a recognizable local structural aetiology in most cases. Atypical clinical features or an abnormal neurological examination are alerts for a potential underlying cause of pain, although cases of clinically typical occipital neuralgia as isolated manifestation of lesions of the cervical spinal cord, cervical roots, or occipital nerves have been increasingly reported. We describe two cases (one with typical and another one with atypical clinical features) of occipital neuralgia secondary to paravertebral pyomyositis and vertebral relapse of multiple myeloma in patients with relevant medical history that aroused the possibility of an underlying structural lesion. We discuss the need for cranio-cervical magnetic resonance imaging in all patients with occipital neuralgia, even when typical clinical features are present and neurological examination is completely normal.

Primary cough headache

Primary cough headache is defined as head pain brought on by coughing or other Valsalva maneuvers, but not by prolonged physical exercise, in the absence of any intracranial disorder. Primary cough headache is considered to be a rare condition, accounting for 0.4% of all headaches consulting our Neurology Department. Its pathophysiology remains a mystery. Primary cough headache is a sudden-onset headache that usually lasts from 1 second to 30 minutes, tends to be bilateral and posterior, does not begin earlier than the fifth decade of life, is more frequent in men, is not accompanied by other neurologic manifestations, and responds to indomethacin. These clinical characteristics allow its differential diagnosis from other entities, even though a craniocervical magnetic resonance imaging study is mandatory to rule out posterior fossa lesions, especially Chiari type-I malformation.

Evaluation of the craniocervical junction in Down syndrome: correlation of measurements obtained with radiography and MR imaging.

Measurement of the atlas-dens interval is the radiographic standard for identification of patients with Down syndrome at high risk for neurologic injury from spinal cord compression. In 17 pediatric patients with Down syndrome, measurements of the atlas-dens interval, distance between the clivus and posterior odontoid process, and width of the neural canal obtained with plain radiographs were compared to predict the width of the subarachnoid space on magnetic resonance (MR) images. In all patients, cervical radiography and craniocervical MR imaging were prospectively performed during lateral flexion and extension. The width of the subarachnoid space was measured with MR imaging. When the 95% confidence interval for correlation coefficients of regression was used, subarachnoid space width on MR images correlated with neural canal width on radiographs better than with either atlas-dens interval or clivus-posterior odontoid process distance (P = .05). Measurement of neural canal width is a better predictor of potential spinal cord compression than the atlas-dens interval or clivus-posterior odontoid process distance and should be emphasized in screening examinations performed with plain radiography.