Carr, Carrie M.

ABSTRACTBACKGROUND AND PURPOSEThere is a relative paucity of radiology literature on spinal cord cavernous malformations (SCMs). We hypothesized that the classic MRI features of cavernous malformations are not highly prevalent. The purpose was to review MR imaging findings of SCMs with a focus on prevalence of hemorrhagic features, including adjacent intramedullary hemorrhage at initial presentation.METHODSIn this single‐institution study, 78 SCMs in 76 patients diagnosed on imaging/pathology were retrospectively evaluated for size, location, cord expansion, exophytic component, popcorn morphology, signal characteristics, including T1 weighted (T1w) and T2w rims, blood‐fluid levels, and adjacent intramedullary hemorrhage. Intramedullary hemorrhage was defined as centrally and/or eccentrically located linear or flame shaped non‐edematous signal abnormality extending longitudinally away from SCMs, distinct from lesional rim.RESULTSSixty‐three percent (49/78) of SCMs measured <1 cm in length, 78% (61/78) extended to the cord surface, 65% (51/78) were expansile, and 32% (25/78) were exophytic. Popcorn morphology was seen in 15% (12/78) on T1w and 22% (17/78) on T2w. Internal blood‐fluid levels were evident in 4% (3/78). Sixty‐nine percent (54/78) showed T2w hypointense rims; the T2w rim was complete/near‐complete in 37% (29/78). A rim on T1w was visible in 49% (38/78); this was hyperintense in 58% (22/38). Adjacent intramedullary hemorrhage was seen in 45/78 (58%), was most commonly bidirectional in 67% (30/45) and eccentric in 68% (30/44). Cord edema was seen in 22% (17/78); 53% (9/17) also demonstrated intramedullary hemorrhage with edema.CONCLUSIONSThe classically described popcorn morphology, internal blood‐fluid levels, and complete/near‐complete T2w hypointense rims are often absent on MRIs in SCMs. Adjacent intramedullary hemorrhage is frequently present, often eccentric and bidirectional.

A 69-year-old female presented with subacute onset ascending weakness and paraesthesias. She was initially diagnosed with Guillain–Barré syndrome (GBS) based on her clinical presentation and cerebrospinal fluid (CSF) analysis showing albuminocytological dissociation. However, she was later found to have anti-neuronal nuclear antibody 1 (ANNA-1/anti-Hu)-positive CSF and was subsequently diagnosed with small-cell lung cancer. Her neurological symptoms were ultimately attributed to ANNA-1/anti-Hu-associated paraneoplastic polyneuropathy. During the course of her evaluation, she had magnetic resonance imaging findings of dorsal predominant cauda equina nerve root enhancement, which has not been previously described. The only previously reported case of cauda equina enhancement due to ANNA-1-associated polyneuropathy described ventral predominant findings. The distinction between ventral and dorsal enhancement is important, since it suggests that different patterns of nerve root involvement may be associated with this paraneoplastic syndrome. Therefore, ANNA-1-associated paraneoplastic inflammatory polyneuropathy can be considered in the differential diagnosis of cauda equina nerve root enhancement with ventral and/or dorsal predominance. This can potentially be helpful in differentiating ANNA-1 polyneuropathy from GBS, which classically has ventral predominant enhancement.

Background: Positive-pressure myelogram (PPM) is an emerging radiologic study used to localize spinal dural defects. During PPM, cerebrospinal fluid pressure (CSFp) is increased by injecting saline with contrast into the cerebrospinal fluid. This has the potential to increase intracranial pressure and compromise cerebral perfusion. Methods: We performed a retrospective chart review and analysis of 11 patients. The aim was to describe the periprocedural anesthetic management of patients undergoing PPM. Results: All patients underwent PPM with general anesthesia and intra-arterial blood pressure and near-infrared spectroscopy monitoring of regional cerebral tissue oxygen saturation. Mean±SD maximum lumbar CSFp was 58±12 mm Hg. Upon intrathecal injection, mean systolic blood pressure increased from 115±21 to 142±32 mm Hg (P<0.001), diastolic blood pressure from 68±12 to 80±20 mm Hg (P≤0.001), and mean blood pressure from 87±10 to 98±14 mm Hg (P=0.02). Ten of 11 patients received blood pressure augmentation with phenylephrine to minimize the risk of reduced cerebral perfusion secondary to increased CSFp after intrathecal injection. The mean heart rate before and following injection was similar (68±15 vs. 70±15 bpm, respectively; P=0.16). There was a decrease in regional cerebral oxygen saturation after positioning from supine to prone position (79±10% to 74±9%, P=0.02) and a further decrease upon intrathecal injection (75±10% to 69±9%, P≤0.01). Conclusions: Systemic blood pressure increased following intrathecal injection during PPM, possibly due to a physiologic response to intracranial hypertension/reduced cerebral perfusion or administration of phenylephrine. Regional cerebral oxygen saturation decreased with the change to prone position and further decreased upon intrathecal injection. Cerebral near-infrared spectroscopy has a potential role to monitor the adequacy of cerebral perfusion and guide adjustment of systemic blood pressure during PPM.

Objective Cerebrospinal fluid-venous fistula is an uncommon cause of spontaneous spinal cerebrospinal fluid leak (SSCSFL). We aim to describe the clinical presentation, imaging evaluation, treatment and outcome of SSCSFL secondary to cerebrospinal fluid-venous fistula. Methods A retrospective review was undertaken of SSCSFL cases secondary to cerebrospinal fluid-venous fistula confirmed radiologically or intraoperatively, seen at our institution from January 1994 to March 2019. Cases with undetermined SSCSFL etiology, alternative etiology or unconfirmed fistula were excluded. Results Forty-four of 156 patients met the inclusion criteria (31 women, 13 men). Mean age of symptom onset was 52.6 years (SD 8.7, range 33–71 years). Headache was the presenting symptom in almost all, typically daily (69%), and most often in occipital/suboccipital regions. Headache character was most commonly pressure (38%), followed by throbbing/pulsing (21.4%). Orthostatic headache worsening occurred in 69% and an even greater percentage of patients (88%) reported Valsalva-induced headache exacerbation or precipitation. Headache occurred in isolation to Valsalva maneuvers in 12%. Of 37 patients with documented cerebrospinal fluid opening pressure, 13% were <6 cmH 2 O; 84%, 7–20 cmH 2 O; and one, 25 cmH 2 O. Fistulas were almost exclusively thoracic (95.5%). Only one patient responded definitively to epidural blood patch (EBP). Forty-two patients underwent surgery. Most improved following surgery; 48.7% were completely headache free and 26.8% had at least 50% improvement. Conclusion In our series, cerebrospinal fluid-venous fistula was associated with a greater occurrence of Valsalva-induced headache exacerbation or precipitation than orthostatic headache and did not respond to EBP. Surgery provided significant improvement. Cerebrospinal fluid-venous fistula should be considered early in the differential diagnosis of Valsalva-induced (“cough”) headache.

Benign notochordal cell tumors (BNCTs) are considered to be benign intraosseous lesions of notochord origin; however, recent spine studies have suggested the possibility that some chordomas arise from BNCTs. Here, the authors describe two cases demonstrating histological features of BNCT and concomitant chordoma involving the clivus, which, to the best of the authors’ knowledge, have not been previously documented at this anatomical site.An 18-year-old female presented with an incidentally discovered clival mass. Magnetic resonance imaging revealed a 2.8-cm nonenhancing lesion in the upper clivus that was T2 hyperintense and T1 hypointense. She underwent an uneventful endoscopic transsphenoidal resection. Histologically, the tumor demonstrated areas of classic chordoma and a distinct intraosseous BNCT component. The patient completed adjuvant radiation therapy. Follow-up showed no recurrence at 18 months.A 39-year-old male presented with an incidentally discovered 2.8-cm clival lesion. The nonenhancing mass was T2 hyperintense and T1 hypointense. Surgical removal of the lesion was performed through an endoscopic transsphenoidal approach. Histological analysis revealed areas of BNCT with typical features of chordoma. Follow-up did not demonstrate recurrence at 4 years.These cases document histologically concomitant BNCT and chordoma involving the clivus, suggesting that the BNCT component may be a precursor of chordoma.

Study Design. Retrospective matched cohort study. Objective. To determine if low-pressure lumbar provocation discography (PD) results in long-term accelerated disc degeneration, internal disc disruption, or disc herniation in patients with symptomatic low back pain (LBP). Summary of Background Data. Study of subjects without clinically-significant LBP suggests that high-pressure PD may accelerate disc degeneration. Methods. Consecutive patients with symptomatic LBP who underwent magnetic resonance imaging (MRI), PD, and repeat MRI more than 7 years later, but did not undergo subsequent spinal fusion surgery, were included. Punctured discs were matched (1:2 to 1:4) to corresponding discs in a control cohort by age, BMI, Pfirrmann score (±2), and presence of disc herniation; control cohort inclusion required MRIs for symptomatic LBP, separated by more than 7 years. The primary outcome of the study was a progression in Pfirrmann score category (I–II, III–IV, V). MRI disc-to-CSF T2 signal-intensity ratio, disc height, disc herniations, high intensity zones (HIZs), and Modic changes were assessed. Results. Baseline and follow-up MRIs were available for 77 discs exposed to PD, and for 260 discs in the matched control cohort. There was no difference in the proportion of punctured discs that advanced in Pfirrmann score category in the PD group (17%, 95% CI 9–27%) compared with corresponding discs in the Control group (21%, 95% CI 17–27%), P = 0.3578, or in non-punctured discs in the PD group (35%, 95% CI 21–51%) compared with corresponding discs in the Control group (34%, 95% CI 27–42%), P = 0.1169. There were no differences in disc-to-CSF T2 signal-intensity ratio, presence of disc herniations, HIZs, or Modic changes following puncture in the PD versus matched cohort discs or in the non-punctured PD cohort discs versus corresponding control cohort discs (P > 0.05). Conclusion. Patients with symptomatic LBP who underwent low-pressure PD, but who did not undergo a subsequent spinal fusion surgery, developed disc degeneration and new disc herniations at a similar rate to corresponding discs in matched control patients. Level of Evidence: 3

Autoimmune epilepsy (AE) is becoming increasingly recognized as a potentially reversible cause of frequent or medically intractable seizures and cognitive deterioration. We describe various presentations of autoimmune encephalopathy which have specifically presented with seizure and describe reported imaging findings. This is organized as a review of the more common autoantibodies which can specifically precipitate seizure according to the intracellular or extracellular location of the targeted antigen. For each antibody, we illustrate their pathophysiology, characteristic clinical presentations with typical effective treatments and prognoses and imaging findings on MRI and PET/CT exams. Parenchymal involvement is variable with the limbic structures typically affected; however, non-limbic cortex, cerebellum, brainstem and basal ganglia can also be involved. In the acute setting, affected regions typically demonstrate T 2 hyperintensity with mild mass effect from edema and increased 18F-fludeoxyglucose uptake. Chronically involved parenchyma will often undergo atrophy and demonstrate decreased metabolism; mesial temporal sclerosis is often the end result when the limbic system is involved. Without treatment, long-term effects from AE range from ongoing cognitive dysfunction and refractory seizures to death. Familiarity with AE may prompt appropriate antibody screening, particularly in cases of refractory seizure disorders. Early investigation and proper management of AE cases may help to prevent parenchymal and neurologic deterioration in these patients.