Cervical epidural hematoma with Brown-Sequard syndrome caused by an epidural injection: a case report

Epidural hematoma with Brown-Sequard syndrome caused by an epidural injection is a rarely found condition in the emergency department (ED). We report an unusual case of Brown-Sequard syndrome in a 55-year-old man who presented at the ED with right-sided weakness and contralateral loss of pain and temperature sensation after a cervical epidural injection for shoulder pain. Cervicla spine magnetic resonance imaging showed an epidural hematoma from C4 to C6. After admission, his right hemiparesis and contralateral sensory loss improved within eight days, and surgical decompression was not required. Diagnosing spinal lesions in the ED is challenging, especially in patients with acute neurological signs requiring immediate evaluation for stroke. In this case, definite hemiparesis and some contralateral sensory loss were noted. Therefore, a potential spinal lesion was suspected rather than a stroke. This case emphasized the importance of conducting a focused neurological examination after history taking.

Difficult-to-Treat Polyradiculoneuropathy

A 51-year-old healthy man sought care for a 6-month history of progressive, distal, lower extremity weakness, imbalance, and numbness in the feet. Neurologic examination showed a steppage gait, upper and lower extremity weakness, distal greater than proximal, absent tendon reflexes, and large fiber–predominant sensation loss in the feet. Nerve conduction studies showed marked temporal dispersion and slowed conductions. Cerebrospinal fluid analysis showed an increased protein concentration, 1 white blood cell/µL, and normal glucose level. Lumbar spine magnetic resonance imaging showed enlargement and enhancement of the nerve roots in the cauda equina, along with hypointensity in lumbar vertebral bodies. He underwent right sural nerve biopsy that showed an inflammatory demyelinating process. The patient was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and started on intravenous immunoglobulin. He was markedly worse at 12-week follow-up, with severe proximal and distal weakness and requiring the use of a walker. The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy was revisited. Lumbar spine magnetic resonance imaging again showed enhancement of the nerve roots. Because of concern for neurolymphomatosis, a proximal fascicular nerve biopsy of the right sciatic nerve was performed. It showed the hallmark pathologic features of chronic inflammatory demyelinating polyradiculoneuropathy: endoneurial inflammation and signs of long-standing demyelination and remyelination with stacks on Schwann cell processes. The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy was confirmed. Intravenous immunoglobulin was stopped, and the patient was started on an aggressive plasma exchange regimen. He had modest improvement. Azathioprine was also started. The patient continued to improve. He remained on this regimen for 2 years. Over the next year, the intravenous methylprednisolone dose was reduced. He was weaned off plasma exchange and intravenous methylprednisolone. At the last follow-up the disease was still in remission. Chronic inflammatory demyelinating polyradiculoneuropathy was described and named in 1975. It is a fairly symmetric peripheral neuropathy that usually presents with proximal and distal weakness, imbalance, and large fiber sensory dysfunction. Cerebrospinal fluid analysis shows albuminocytologic dissociation in 80% to 95% of those with typical chronic inflammatory demyelinating polyradiculoneuropathy.

Diagnostic value of gadolinium contrast administration for spinal cord magnetic resonance imaging in multiple sclerosis patients and correlative markers of lesion enhancement

Background Magnetic resonance imaging is essential for monitoring people with multiple sclerosis, but the diagnostic value of gadolinium contrast administration in spine magnetic resonance imaging is unclear. Objective To assess the diagnostic value of gadolinium contrast administration in spine magnetic resonance imaging follow-up examinations and identify imaging markers correlating with lesion enhancement. Methods A total of 65 multiple sclerosis patients with at least 2 spinal magnetic resonance imaging follow-up examinations were included. Spine magnetic resonance imaging was performed at 3 Tesla with a standardized protocol (sagittal and axial T2-weighted turbo spin echo and T1-weighted post-contrast sequences). T2 lesion load and enhancing lesions were assessed by two independent neuroradiologists for lesion size, localization, and T2 signal ratio (T2 signallesion/T2 signalnormal appearing spinal cord). Results A total of 68 new spinal T2 lesions and 20 new contrast-enhancing lesions developed during follow-up. All enhancing lesions had a discernable correlate as a new T2 lesion. Lesion enhancement correlated with a higher T2 signal ratio compared to non-enhancing lesions (T2 signal ratio: 2.0 ± 0.4 vs. 1.4 ± 0.2, **** p < 0.001). Receiver operating characteristics analysis showed an optimal cutoff value of signal ratio 1.78 to predict lesion enhancement (82% sensitivity and 97% specificity). Conclusion Gadolinium contrast administration is dispensable in follow-up spine magnetic resonance imaging if no new T2 lesions are present. Probability of enhancement correlates with the T2 signal ratio.

Case Report: Pansynostosis, Chiari I Malformation and Syringomyelia in a Child With Frontometaphyseal Dysplasia 1

Frontometaphyseal dysplasia 1 (FMD1) is a rare otopalatodigital spectrum disorder (OPDSD) that is inherited as an X-linked trait and it is caused by gain-of-function mutations in the FLNA. It is characterized by generalized skeletal dysplasia, and craniofacial abnormalities including facial dysmorphism (supraorbital hyperostosis, hypertelorism, and down-slanting palpebral fissures). The involvement of the central nervous system in patients with OPDSD is rare. Herein, we present the case of a 12-year-old boy with facial dysmorphism, multiple joint contractures, sensorineural hearing loss, scoliosis, craniosynostosis, and irregular sclerosis with hyperostosis of the skull. Brain and whole-spine magnetic resonance imaging revealed Chiari I malformation with extensive hydrosyringomyelia from the C1 to T12 levels. Targeted next-generation sequencing identified a hemizygous pathologic variant (c.3557C&gt;T/p.Ser1186Leu) in the FLNA, confirming the diagnosis of FMD1. This is the first report of a rare case of OPDSD with pansynostosis and Chiari I malformation accompanied by extensive syringomyelia.

Physiological pseudo-thickened cauda equina associated with dural sac dilatation on magnetic resonance imaging

Objectives In daily clinical practice, the assessment of the thickness of the cauda equina on lumbar spine magnetic resonance imaging is an important parameter. However, its relevance to the size of the dural sac in non-pathological conditions is unknown. To examine the relationship between the size of the dural sac and the apparent thickness of the cauda equina nerve root using lumbar spine magnetic resonance imaging in non-pathological conditions. Methods We retrospectively measured the dural sac diameter and vertebral body diameter, counted the apparent number, and calculated total cross-sectional area of the cauda equina, dural sac ratio and the area of one apparent nerve root of cauda equina in 100 cases. Spearman's rank correlation coefficient ( ρ) was used. Results Dural sac ratio and diameter were positively correlated with the area of one apparent nerve root ( ρ=0.77, P<0.001; ρ=0.74, P<0.001; respectively) and negatively correlated with the apparent number of cauda equina in a single cross-section ( ρ=–0.63, P<0.001; ρ=–0.52, P<0.001; respectively). Conclusions A larger dural sac ratio and diameter was associated with an apparently thicker cauda equina and lower visible number. In a larger dural sac, the physiologically clumped and apparently thicker cauda equina should not be misdiagnosed as pathological.