Gadolinium enhancement of cranial nerves: Implications for interstitial fluid drainage from brainstem into cranial nerves in humans.

Drainage of interstitial fluid and solutes from the brainstem has not been well studied. To map one drainage pathway in the human brainstem, we took advantage of the focal blood-brain barrier disruption occurring in a multiple sclerosis brainstem lesion, coupled with intravenous injection of gadolinium, which simulates an intraparenchymal injection of gadolinium tracer within the restricted confines of this small brain region. Using high-resolution MRI, we show how it is possible for interstitial fluid to drain into the adjacent trigeminal and oculomotor nerves, in keeping with a pathway of communication between the extracellular spaces of the brainstem and cranial nerve parenchyma.

Infraorbital canal variants and its clinical and surgical implications. A systematic review.

BACKGROUND: Recent literature highlights anomalous cranial nerves in the sinonasal region, notably in the sphenoid and maxillary sinuses, linked to anatomical factors. However, data on the suspended infraorbital canal (IOC) variant is scarce in cross-sectional imaging. Anatomical variations in the sphenoid sinuses, including optic, maxillary, and vidian nerves, raise interest among specialists involved in advanced sinonasal procedures. The infraorbital nerve's (ION) course along the orbital floor and its abnormal positioning within the orbital and maxillary sinus region pose risks of iatrogenic complications. A comprehensive radiological assessment is crucial before sinonasal surgeries. Cone-beam computed tomography (CBCT) is preferred for its spatial resolution and reduced radiation exposure. OBJECTIVE: The aim of this study was to describe the prevalence of anatomical variants of the infraorbital canal (IOC) and report its association with clinical condition or surgical implication. METHODS: We searched Medline, Scopus, Web of Science, Google Scholar, CINAHL, and LILACS databases from their inception up to June 2023. Two authors independently performed the search, study selection, data extraction, and assessed the methodological quality with assurance tool for anatomical studies (AQUA). Finally, the pooled prevalence was estimated using a random effects model. RESULTS: Preliminary results show that three types are prevalent, type 1: the IOC does not bulge into the maxillary sinus (MS); therefore, the infraorbital foramen through the anterior wall of MS could be used for identification of the ION. Type 2: the IOC divided the orbital floor into medial and lateral aspects. Type 3: the IOC hangs in the MS and the entire orbital floor lying above the IOC. From which the clinical implications where mainly surgical, in type 1 the infraorbital foramen through the anterior wall of MS could be used for identification of the ION, while in type 2, since the lateral orbital floor could not be directly accessed an inferiorly transposition of ION is helpful to expose the lateral orbital wall directly with a 0 scope; or using angled endoscopes and instruments, however, the authors opinion is that direct exposure potentially facilitates the visualization and management in complex situations such as residual or recurrent mass, foreign body, and fracture located at the lateral aspect of the canal. Lastly, in type 3, the ION it's easily exposed with a 0° scope. CONCLUSIONS: This systematic review identified four IOC variants: Type 1, within or below the MS roof; Type 2, partially protruding into the sinus; Type 3, fully protruding into the sinus or suspended from the roof; and Type 4, in the orbital floor. Clinical recommendations aim to prevent nerve injuries and enhance preoperative assessments. However, the lack of consistent statistical methods limits robust associations between IOC variants and clinical outcomes. Data heterogeneity and the absence of standardized reporting impede meta-analysis. Future research should prioritize detailed reporting, objective measurements, and statistical approaches for a comprehensive understanding of IOC variants and their clinical implications. Open Science Framework (OSF): https://doi.org/10.17605/OSF.IO/UGYFZ .

The value of 3D high-resolution IR-prepared fast spoiled gradient-recalled MRI in the diagnosis of meningeal carcinomatosis involving the cranial nerves.

PURPOSE: The purpose of this study was to investigate the clinical utility of three-dimension (3D) high-resolution inversion recovery (IR)-prepared fast spoiled gradient-recalled (SPGR) magnetic resonance imaging (MRI) in the diagnosis of cranial nerve meningeal carcinomatosis (MC). METHODS: A total of 114 patients with MC from January 2015 to March 2020 were enrolled and their MRIs were analyzed retrospectively. All patients underwent MRIs before being administered a contrast agent. Both a 2D conventional MRI sequence and a 3D IR-prepared fast SPGR high-resolution T1-weighted (BRAVO) scan sequence were measured after contrast agent administration. The characteristics of MC and the involved cranial nerves were then examined. RESULTS: Among the 114 MC patients, 81 (71.05%) had cranial nerve enhancement on contrast-enhanced 3D-BRAVO imaging, while only 41 (35.96%) had image enhancement on conventional MRI. The contrast-enhanced 3D-BRAVO displayed stronger image contrast enhancement of the cranial nerves than the conventional MRI (P < 0.001). Furthermore, detection rates for the facial and auditory nerves, trigeminal nerve, oculomotor nerve, sublingual nerve, optic nerve, glossopharyngeal/vagal/accessory nerve, and abductor nerve on contrast-enhanced 3D-BRAVO imaging were 58.77%, 47.37%, 9.65%, 8.77%, 5.26%, 3.51%, and 0.88%, respectively. We found a statistically significant difference between the affected facial and auditory nerves, as well as the trigeminal nerve, oculomotor nerve, sublingual nerve, and optic nerve. CONCLUSION: In MC, contrast-enhanced 3D-BRAVO imaging displayed the cranial nerves more effectively than 2D conventional enhanced MRI. The facial, auditory, and trigeminal nerves are the primary nerves involved in MC, and improved scanning of these nerves would aid in the early detection and treatment of MC.

Enhancement of cranial nerves in Lyme neuroborreliosis: incidence and correlation with clinical symptoms and prognosis.

PURPOSE: Symptoms of cranial neuritis are a common presentation of Lyme neuroborreliosis (LNB). Imaging studies are scarce and report contradictory low prevalence of enhancement compared to clinical studies of cranial neuropathy. We hypothesized that MRI enhancement of cranial nerves in LNB is underreported, and aimed to assess the prevalence and clinical impact of cranial nerve enhancement in early LNB. METHODS: In this prospective, longitudinal cohort study, 69 patients with acute LNB were examined with MRI of the brain. Enhancement of cranial nerves III-XII was rated. MRI enhancement was correlated to clinical findings of neuropathy in the acute phase and after 6 months. RESULTS: Thirty-nine of 69 patients (57%) had pathological cranial nerve enhancement. Facial and oculomotor nerves were most frequently affected. There was a strong correlation between enhancement in the distal internal auditory canal and parotid segments of the facial nerve and degree of facial palsy (gamma = 0.95, p < .01, and gamma = 0.93, p < .01), despite that 19/37 nerves with mild-moderate enhancement in the distal internal auditory canal segment showed no clinically evident palsy. Oculomotor and abducens nerve enhancement did not correlate with eye movement palsy (gamma = 1.00 and 0.97, p = .31 for both). Sixteen of 17 patients with oculomotor and/or abducens nerve enhancement had no evident eye movement palsy. CONCLUSIONS: MRI cranial nerve enhancement is common in LNB patients, but it can be clinically occult. Facial and oculomotor nerves are most often affected. Enhancement of the facial nerve distal internal auditory canal and parotid segments correlate with degree of facial palsy.

Hyperintense areas in the cisternal segments of the cranial nerves: a magnetic resonance imaging study.

PURPOSE: The study aimed to explore hyperintense areas in the cisternal segments of the cranial nerves using magnetic resonance imaging (MRI). METHODS: Seventy outpatients underwent thin-sliced, coronal constructive interference steady-state (CISS) sequence and sagittal T2-weighted MRI following conventional MRI examination. RESULTS: With the coronal CISS sequence, hyperintense areas were located in the central parts of the olfactory bulbs in 65.7% of patients. For the intracranial optic nerve and optic chiasm, hyperintense areas were detected in 98.6% of the CISS sequences and 100% of the T2-weighted images. In the optic tract, hyperintense areas were detected in 51.4% of cases. In 35% of the patients who underwent the CISS sequence, the intracranial optic nerves were considerably compressed by the internal carotid and anterior cerebral arteries, with hyperintense areas similar to those in patients without vascular compression. Hyperintense areas of the cisternal segments of the oculomotor nerve and trigeminal root were identified in 52.9% and 87.1% of the patients, respectively. CONCLUSIONS: The hyperintense areas found within the cisternal segments of the cranial nerves delineated on the coronal CISS sequence and sagittal T2-weighted imaging may indicate the intracranial part of the glymphatic pathway through the cranial nerves. The cranial nerves may function as part of the glymphatic pathway.

High-resolution MRI demonstrates signal abnormalities of the 3rd cranial nerve in giant cell arteritis patients with 3rd cranial nerve impairment.

OBJECTIVE: To determine the sensitivity and specificity of high-resolution (HR) MRI for detecting signal abnormalities of cranial nerves (CN) in giant cell arteritis (GCA) patients presenting with diplopia. METHODS: This IRB-approved retrospective single-center study included GCA patients who underwent 3-T HR MRI from December 2014 to January 2020. Two radiologists, blinded to all data, individually assessed for the presence of enhancement of the 3rd, 4th, and/or 6th CN on post-contrast HR imaging and high signal intensity on HR T2-WI, for signal abnormalities of extraocular muscles and the brainstem, and for inflammatory changes of the ophthalmic and extracranial arteries. A Fisher's exact test was used to compare patients with or without diplopia. RESULTS: In total, 64 patients (42/64 (66%) women and 22/64 (34%) men, mean age 76.3 ± 8 years) were included. Of the 64 patients, 14 (21.9%) presented with diplopia. Third CN enhancement was detected in 7/8 (87.5%) patients with 3rd CN impairment, as compared to no patients with 4th or 6th CN impairment or to patients without diplopia (p < 0.001). Third CN abnormal high signal intensity on HR T2-WI was detected in 4/5 patients (80%) with 3rd CN impairment versus none of other patients (p < 0.001). Sensitivity, specificity, positive predictive value, and negative predictive value for detecting 3rd CN signal abnormalities were of 0.88, 1, 1, and 0.99 and 0.8, 1, 1, and 0.98 for post-contrast HR imaging and HR T2-WI, respectively. CONCLUSIONS: HR MRI had excellent diagnostic sensitivity and specificity when detecting signal abnormalities of the 3rd CN in GCA patients presenting with 3rd CN impairment. KEY POINTS: • Third cranial nerve enhancement was detected in all patients with 3rd cranial nerve impairment except for one with transient diplopia. • The "check mark sign" might be useful to identify 3rd cranial nerve signal abnormalities in the orbital apex. • No signal abnormalities of the 4th or 6th cranial nerves could be detected on high-resolution MRI.

Magnetic resonance features and cranial nerve involvement in pediatric head and neck rhabdomyosarcomas.

PURPOSE: Rhabdomyosarcoma (RMS) is a malignant tumor frequent in children. The frequency and characteristics of cranial nerve involvement in pediatric head and neck (H&N) RMS have been scarcely reported. The aim of this study is to review a large cohort of pediatric head and neck RMS with an emphasis on cranial nerve involvement. METHODS: We retrospectively reviewed H&N RMS cases from 3 tertiary hospitals over a 10-year period. Cranial nerve involvement was defined as radiologically apparent tumor extension along a nerve and/or the presence of secondary signs. Scans were reviewed by two pediatric neuroradiologists, blinded to clinical data. RESULTS: A total of 52 patients met the inclusion criteria. Histologically, 39/52 were embryonal RMS, while 13/52 were alveolar RMS. Regional lymph nodes metastases were present in 19.2%. Cranial nerve involvement was present in 36.5%. Nerves were mainly involved as a direct extension of the mass through skull base foramina or after invasion of cavernous sinus, Meckel's cave, orbital apex, or stylomastoid foramen. CONCLUSION: Cranial nerve involvement is frequent in pediatric head and neck RMS and occurs secondary to "geographic" invasion due to direct extension through skull base foramina or cavernous sinus. These tumors never showed distant perineural metastatic disease as is seen in cases of adult head and neck carcinomas. This implies a different biological interaction between the nerves and these tumors in comparison to adult H&N tumors.

Isolated primary neurolymphomatosis with cranial multineuritis: a case presentation.

BACKGROUND: Isolated primary neurolymphomatosis (NL) of cranial multineuritis is a very rare condition that refers to the lymphomatous invasion of cranial nerves only. There are sparse cases of isolated cranial nerves NL reported worldwide. CASE PRESENTATION: We present magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) findings of a 63-year-old female patient suffering from isolated neurolymphomatosis of cranial multineuritis with a wide constellation of syndromes including binocular diplopia, left facial paralysis and pain, syncope episodes, and progressive dysphagia. A contrasted MRI brain showed multiple cranial nerves enhancement. Extensive workup for infectious, autoimmune, neoplastic, paraneoplastic, or inflammatory etiologies had been unrevealing except CSF cytology revealed large atypical monotypic B cells that were suspicious for non-Hodgkin lymphoma on the third large volume tap. The decision of biopsy was deferred after the risks and benefits discussion. Following the four cycles of empiric methotrexate-based induction chemotherapy, the patient's symptoms resolved, and a complete radiographic response was achieved without whole-brain radiation or autologous hematopoietic cell transplantation. In the latest follow-up, she is independent with her daily activities and remains in clinical and radiographic remission more than 3 years since initial chemotherapy. CONCLUSION: Isolated NL of cranial nerves can present diagnostic and management pitfalls for the neurologist, neurosurgeons, and oncologists. Since current diagnostic modalities have modest sensitivity and a pathological diagnosis is often difficult, empiric treatment once other possibilities are ruled out can carry a good prognosis.

Surgery for posterior fossa meningioma: elevated postoperative cranial nerve morbidity discards aggressive tumor resection policy.

Radical excision of meningioma is suggested to provide for the best tumor control rates. However, aggressive surgery for meningiomas located at the posterior cranial fossa may lead to elevated postoperative morbidity of adjacent cranial nerves which in turn worsens patients' postoperative quality of life. Therefore, we analyzed our institutional database with regard to new cranial nerve dysfunction as well as postoperative cerebrospinal fluid (CSF) leakage depending on the extent of tumor resection. Between 2009 and 2017, 89 patients were surgically treated for posterior fossa meningioma at the authors' institution. Postoperative new cranial nerve dysfunction as well as CSF leakage were stratified into Simpson grade I resections with excision of the adjacent dura as an aggressive resection regime versus Simpson grade II-IV tumor removal. Simpson grade I resections revealed a significantly higher percentage of new cranial nerve dysfunction immediately after surgery (39%) compared with Simpson grade II (11%, p = 0.01) and Simpson grade II-IV resections (14%, p = 0.02). These observed differences were also present for the 12-month follow-up (27% Simpson grade I, 3% Simpson grade II (p = 0.004), 7% Simpson grades II-IV (p = 0.01)). Postoperative CSF leakage was present in 21% of Simpson grade I and 3% of Simpson grade II resections (p = 0.04). Retreatment rates did not significantly differ between these two groups (6% versus 8% (p = 1.0)). Elevated levels of postoperative new cranial nerve deficits as well as CSF leakage following radical tumor removal strongly suggest a less aggressive resection policy to constitute the surgical modality of choice for posterior cranial fossa meningiomas.

Clinical signs, MRI findings and outcome in dogs with peripheral vestibular disease: a retrospective study.

BACKGROUND: Vestibular dysfunction is relatively common in dogs, with a prevalence of 0.08% reported in primary veterinary care in the UK. There are several studies investigating how to differentiate between peripheral and central vestibular disease but only limited information regarding the possible underlying causes for peripheral vestibular dysfunction in dogs. This study therefore aimed to describe the clinical signs, magnetic resonance imaging findings (MRI), underlying causes and outcome in a large population of dogs diagnosed with peripheral vestibular disease. RESULTS: One hundred eighty-eight patients were included in the study with a median age of 6.9 years (range 3 months to 14.6 years). Neurological abnormalities included head tilt (n = 185), ataxia (n = 123), facial paralysis (n = 103), nystagmus (n = 97), positional strabismus (n = 93) and Horner syndrome (n = 7). The most prevalent diagnosis was idiopathic vestibular disease (n = 128), followed by otitis media and/or interna (n = 49), hypothyroidism (n = 7), suspected congenital vestibular disease (n = 2), neoplasia (n = 1) and cholesteatoma (n = 1). Long-term follow-up revealed persistence of head tilt (n = 50), facial paresis (n = 41) and ataxia (n = 6) in some cases. Recurrence of clinical signs was observed in 26 dogs. Increasing age was associated with a mild increased chance of diagnosis of idiopathic vestibular syndrome rather than otitis media and/or interna (P = 0.022, OR = 0.866; CI 0.765-0.980). History of previous vestibular episodes (P = 0.017, OR = 3.533; CI 1.251-9.981) was associated with an increased likelihood of resolution of the clinical signs whilst contrast enhancement of cranial nerves VII and/or VIII on MRI (P = 0.018, OR = 0.432; CI 0.251-0.868) was associated with a decreased chance of resolution of the clinical signs. CONCLUSIONS: Idiopathic vestibular disease is the most common cause of peripheral vestibular dysfunction in dogs and it is associated with advanced age. Incomplete recovery from peripheral vestibular disease is common, especially in dogs presenting with cranial nerve enhancement on MRI but less so if there is previous history of vestibular episodes.

High-Resolution Ultrasound of Small Clinically Relevant Nerves Running Across the Posterior Triangle of the Neck.

With the advent of high-frequency ultrasound (US) transducers, new perspectives have been opened in evaluating millimetric and submillimetric nerves that, despite their dimensions, can be considered relevant in clinical practice. In the posterior triangle of the neck, the suprascapular, long thoracic, phrenic, supraclavicular, great auricular, lesser occipital, and transverse cervical nerves are amenable to US examination and the object of special interest because they may be involved in many pathologic processes or have a value as targets of advanced therapeutic procedures. The correct identification of these nerves requires a deep knowledge of local neck anatomy and the use of a complex landmarks-based approach with US. This article describes the anatomy and US technique to examine small but clinically relevant nerves of the posterior triangle of the neck (excluding the brachial plexus), reviewing the main pathologic conditions in which they may be involved.

Atypical clinical manifestations of herpes zoster oticus: diagnostic usefulness of magnetic resonance imaging.

Typical symptoms of Ramsay Hunt syndrome (RHS) consist of painful vesicular eruptions in the external ear, unilateral facial palsy, and/or vestibulocochlear deficit. When RHS patients show atypical clinical manifestations, correct diagnosis can be delayed, and ideal treatment timing for antiviral therapy may be missed. The aim of this study is to describe RHS patients with atypical clinical manifestations and evaluate the usefulness of magnetic resonance imaging (MRI) for early differential diagnosis. We retrospectively reviewed the clinical data and investigated the findings of internal auditory canal (IAC) MRI of seven patients diagnosed with RHS presenting "atypical" clinical manifestations between January 2013 and December 2016. "Typical" symptoms of RHS consist of herpetic vesicular eruption and facial palsy with or without vestibulocochlear deficit. Regardless of symptomatic presentations, IAC MRI demonstrated post-contrast enhancement of cranial nerve (CN) VII, CN VIII, and IAC dura in patients with atypical clinical manifestations. In cases with multiple lower CN palsy, enhancement along the involved nerve was observed on IAC MRI. When RHS was complicated by acute parotiditis, diffuse enhancement of the parotid gland was demonstrated. The present study shows that in IAC MRI of RHS patients with atypical clinical manifestations, post-contrast enhancement was not confined to the facial nerve but also observed in CN VIII and IAC dura regardless of the symptoms, which may facilitate early diagnosis of RHS.

MRI-guided definition of cerebrospinal fluid distribution around cranial and sacral nerves: implications for brain tumors and craniospinal irradiation.

Background: The SIOPE-Brain Tumor Group recently published a guideline on craniospinal target volume delineation for highly conformal radiotherapy. In order to spare critical structures like e.g., the lens or cochlea, highly conformal techniques can underdose the cerebrospinal fluid (CSF) in the dural reflections around cranial and sacral nerves. The purpose of this study is to generate evidence for CSF extension within the dural sheaths of the cranial and sacral nerves in order to improve accuracy in target volume delineation.Material and methods: Ten healthy volunteers, age 21 till 41 years, underwent an MRI-scan of the skull-base and sacral plexus. To evaluate CSF extension, cT2-weighted images with fat suppression, low signal to noise ratio and little to no motion-related artifacts were used. Two observers measured the extension of CSF from the inner table of the skull for the cranial nerves, and outside the spinal canal for the sacral nerves.Results: CSF extension (mean distance [95% CI]) was visible within the dural sheaths surrounding the majority of the cranial nerves: optic nerve (40 mm [38-42]), trigeminal nerve (16 mm [15-19]), facial-vestibulocochlear nerve (11 mm [11-12]), glossopharyngeal-vagus-accessory nerve (7 mm [7-9]) and hypoglossal nerve (8 mm [7-9]). No CSF was observed outside the spinal canal at sacral level. No significant difference between both observers was measured.Conclusion: This study generates evidence for significant CSF extension outside the inner table of the skull. Despite the vicinity of the lens and cochlea, we therefore recommend the inclusion of both optic nerves and internal auditory canals in the clinical target volume for craniospinal irradiation when using highly conformal delivery techniques.

High-resolution MRI using compressed sensing-sensitivity encoding (CS-SENSE) for patients with suspected neurovascular compression syndrome: comparison with the conventional SENSE parallel acquisition technique.

AIM: To retrospectively compare sensitivity encoding (SENSE) and compressed sensing-sensitivity encoding (CS-SENSE) for high resolution (HR) cranial nerve magnetic resonance imaging (MRI) in a clinical population. MATERIAL AND METHODS: Twenty consecutive patients who were clinically suspected of neurovascular compression syndrome (NVCS) were enrolled in this study. HR three-dimensional isotropic T2-weighted fast spin-echo (T2 VISTA) sequences with SENSE or CS-SENSE, and contrast-enhanced three-dimensional T1-turbo field-echo (CE 3D T1 TFE) with SENSE or CS-SENSE, were compared using quantitative and qualitative methods by two board-certified neuroradiologists. RESULTS: For the T2 VISTA, CS-SENSE was significantly superior to SENSE in terms of cerebrospinal fluid homogeneity. For CE 3D T1 TFE, CS-SENSE was significantly superior to SENSE in terms of the existence of ghost artefact and the signal-to-noise ratio (SNR) of the pontine parenchyma. There was no significant difference in overall image quality between the two techniques. Compared with SENSE, CS-SENSE reduced the scan time to 44.2% of that with SENSE on T2 VISTA, and to 66.1% of that with SENSE of the CE 3D T1 TFE, with the differences being statistically significant (p<0.01, both). CONCLUSION: For T2 VISTA and CE 3D T1 TFE imaging of patients with suspected NVCS, CS-SENSE appears to offer superior reductions in motion artefact and scan time relative to SENSE, without a loss of overall image quality.

Congenital Cranial Dysinnervation Disorder: An Unusual Phenotype With Multiple Cranial Neuropathies and Novel Neuroimaging Findings.

Congenital cranial dysinnervation disorders result from a maldevelopment of brainstem nuclei and/or cranial nerves. In some cases, specific genetic abnormalities have been identified. We expand the clinical phenotype of these disorders with the report of a 28-month-old girl who was initially evaluated for seizures and was found to have right sixth nerve palsy, small optic discs with reduced vision in her right eye. Her development was delayed. Brain MRI showed multiple abnormalities involving other cranial nerves, the optic chiasm and brainstem. Her developmental delay and seizure disorder suggest additional cortical involvement.

Gridding Microsurgical Anatomy of Far Lateral Approach in the Three-Dimensional Model.

OBJECTIVE: The far lateral craniotomy involves osteotomy of various portions of occipital condyle. Intracranial operation exposing clivus encounters complicated neurovascular anatomy. The aim of the present study was to make refinement for the anatomy of far lateral approach by gridding route in the 3-dimensional model. METHODS: Computed tomography and magnetic resonance imaging data were used to construct 3-dimensional model containing osseous and neurovascular structures of skull base. Then, far lateral approach was simulated by triangular prism and divided into gridding surgical route. The relationship of surgical route and osseous and neurovascular structures was observed. Measurement of volume was performed to evaluate surgical exposure. RESULTS: Observation of 3-dimensional model showed bony drilling of far lateral approach started with the occipital condyle and passed through the lateral edge of foramen magnum. The cerebellum and medulla oblongata were exempted from the surgical route exposing clivus. The anatomy variances of operative space, osseous, and neurovascular structures in the gridding route were displayed clearly and compared objectively. CONCLUSION: The gridding operative spaces for the far lateral approach are useful to disclose the detailed discrepancy in the different surgical region. The volumetric measurement provides quantified information to facilitate a better understanding of the anatomy variance.

Enhancement of multiple cranial and spinal nerves in vanishing white matter: expanding the differential diagnosis.

Abnormal cranial or spinal nerve contrast enhancement on MRI in cases of suspected pediatric leukodystrophy is recognized as an important clue to the diagnosis of either metachromatic leukodystrophy or globoid cell leukodystrophy (Krabbe disease). We report a case of genetically confirmed childhood vanishing white matter with enhancement of multiple cranial and spinal nerves in addition to the more typical intracranial findings. This case expands the limited differential diagnosis of cranial nerve or spinal nerve enhancement in cases of suspected leukodystrophy and may aid in more efficient work-up and earlier diagnosis of vanishing white matter.