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AJR 2006; 186:A66-A69
© American Roentgen Ray Society


ABSTRACT

26. Neuroradiology: Head and Neck, Brain White Matter Imaging

Scientific Session 26—Neuroradiology: Head and Neck, Brain White Matter Imaging

Thursday, May 4, 1:30 PM-3:30 PM

Abstracts 251-260

Moderators: Suresh K. Mukherji, MD and Zoran Rumboldt, MD

1:30 PM

Keynote Address: Reversible Encephalopathy Syndrome: What's New? Is It Time For a Change in Name?

Matthew F. Omojola, MB, BS, Creighton University, Omaha, NE

1:50 PM

251. Special Head and Arm Rest for Reduction of Radiation Dose and Contrast Dose in Patients Requiring Combined Neck and Chest CT

Sirineni G.K.*; Kalra M.; Hudgins P.; Tigges S.; Department of Radiology, Emory University School of Medicine, Atlanta, GA.

Address correspondence to G.K. Sirineni (sgkreddy{at}yahoo.com)

Objective: Most institutions acquire separate overlapping scan series for combined chest and neck CT protocol, which results in double exposure of lower neck and upper chest (Thyroid being highly radiosensitive). Therefore, we developed a special "head and arm" rest to allow combined scanning of neck and chest CT in a single series acquisition, with consequent radiation dose and contrast volume reduction. The purpose of our study is to evaluate the effect of "head and arm rest" on radiation dose and contrast dose reduction.

Materials and Methods: In an ongoing study, 5 patients on follow up for lymphoma were evaluated using the "head and arm rest". The "head and arm" rest allowed us to fix the arms of patients above head at an angle with spine such that both arms were above neck level in horizontal plane with patient lying supine. Scans were acquired on 64 slice (n = 4) and 16 slice (n = 1) MDCT scanner in a single run from upper pole of kidneys to frontal sinus in a caudo-cranial direction. A total of 75 cc of Non-ionic iodinated contrast (Omnipaque 350%) was injected in dual phase manner with first 35 cc at 1.4 ml/sec and rest at 3.5 cc/sec. A scan delay of 42 seconds and 37 seconds was used for 64 and 16 slice respectively. Two separate retrospective reconstruction of the neck was performed with a smaller field of view (to exclude the arms) for the neck at 2 mm/2 mm and 1 mm/1 mm (slice thickness/slice interval). The images for chest were reconstructed at 1.25 mm/1.25 mm. The scans were evaluated for presence of streak artifacts in neck and shoulder, overall image quality, and adequacy of contrast opacification of vascular tree.

Results: All 5 studies showed tolerable streak artifacts, acceptable image quality and good contrast opacification of vessels. On comparison with the prior combined chest and neck protocol that involved separate acquisitions of neck and chest, with a total contrast volume of 150cc-200cc, the present studies were satisfactory, and resulted in contrast dose reduction of 50%-62.5% and avoided double radiation exposure to the lower neck and upper chest. The need to retrospectively reconstruct separate image datasets for neck and chest resulted in increased work flow time for post processing for the technologist.

Conclusion: Single acquisition CT of the neck and chest is feasible in patients with lymphoma, with the "head and arm" rest. Our protocol allows reduction of radiation dose to thyroid and also reduction of contrast load by 50-62.5%.

* Will present paper

2:00 PM

252. Assessment of Pediatric Head and Neck Masses with Diffusion MR Imaging

Abdel Razek A.A.1*; Bilal M.1; Hafez M.2; Monier S.1; 1. Diagnostic Radiology, Mansoura University Hospital, Mansoura, DK, Egypt; 2. Pediatric, Mansoura University Hospital, Mansoura, DK, Egypt.

Address correspondence to A.A. Abdel Razek (arazek{at}mans.eun.eg)

Objective: To assess the clinical usefulness of diffusion MR imaging in pediatric patients with head and neck masses.

Materials and Methods: This study included 42 patients (28 boys and 14 girls aged 3ms-15 ys: mean 6ys) with head and neck mass. Routine MR imaging and diffusion weighted MR imaging were done on a 1.5tesla MR unit (Symphony-Siemens). Diffusion MR imaging was done using a single shot echo planar imaging (EPI) with a diffusion-weighted factor, factor b of 0,500 and 1000 sec/mm2. The apparent diffusion coefficient (ADC) map was reconstructed with calculation of ADC value in the suspected lesion. The final diagnosis proved by pathology, MR appearance and clinical examination.

Results: The mean ADC value of the malignant tumor (0.98 ±0.12X 10-3 mm2/sec), benign tumor (1.47 ±0.08X 10-3 mm2/sec), vascular lesions (1.52±0.11X 10-3 mm2/sec), inflammatory lesions (1.77 ±0.12X 10-3 mm2/sec) and cystic lesions (2.05 ±0.09X 10-3 mm2/sec. There was statistically difference in ADC values of malignant neoplasm from benign pediatric head and neck lesions (p < 0.021).

Conclusion: Apparent diffusion coefficient value is a new non-invasive parameter than can be used for differentiation malignant tumors from benign pediatric head and neck lesions.

* Will present paper

2:10 PM

253. Basal Cell Adenoma versus Pleomorphic Adenoma of Parotid Gland: CT Findings

Nai Chi C.1*; Hsiu Mei W.1; Yi Hong C.1; Yi-You C.1; Yin Li W.2; Cheng Y C.1; 1. Radiology, Vghtpe, Taipei, 112, Taiwan; 2. Pathology, Vghtpe, Taipei, 112, Taiwan.

Address correspondence to C. Nai Chi (q28228410{at}yahoo.com.tw)

Objective: Basal cell adenoma (BCA) is a rare benign epithelial tumor of the salivary gland. The objective of this study is to present the CT findings of parotid BCA. We also compare CT findings of BCA with those of pleomorphic adenoma (PA), the most common parotid tumor, to determine whether any feature on CT can help differentiate these two entities.

Materials and Methods: Eleven patients (five women and six men; mean age, 61 years) with BCA and seventeen patients (five women and twelve men; mean age, 47 years)with PA were enrolled in this study. The CT examinations were evaluated by two radiologists. The following data for each tumor, namely, size, location, margin, density, and contrast enhancement pattern were analyzed and compared. Imaging features were correlated with gross and microscopic pathologic features of the tumors.

Results: The majority of BCAs were located in the superficial lobe. (n = 8; deep lobe, n = 3). The mean diameter was 2.0 x 1.9 cm (range: 1.0 x 0.8 to 3.0 x 3.0 cm). Tumors were round (n = 9), or lobulated (n = 2). Eight tumors (73%) were well circumscribed. Six tumors (55%) were enhanced homogeneously; the remaining five tumors showed heterogeneous enhancement including one with a prominent cystic component. However, PA showed no significant pattern difference in comparison with BCA in the basis of CT morphology. All tumors were enhanced more than the surrounding parotid gland. Pre-contrast and post-contrast densities on CT scan were significantly higher in BCA than in PA (pre-contrast, mean: 46.8 HU vs. 34.4 HU, p < 0.05; post-contrast, mean: 91.4HU vs. 66.1HU, p < 0.05, respectively). Patients with BCA were significantly older than in PA (mean: 63.9 years vs. 47.5 years, p < 0.05).

Conclusion: BCA of the parotid gland is chiefly located in the superficial lobe. They are generally round, well circumscribed tumor, showing homogeneous enhancement on CT. The age of the patient, the density on pre- and post-contrast CT scan may help in differentiating BCA from PA of parotid gland.

* Will present paper

2:20 PM

254. Radiographic Examination of the Lateral Skull Base with Digital Volume Tomography

Dalchow C.V.1*; Bien S.2; Werner J.A.1; 1. Department of Otorhinolaryngology, Philipps University, Marburg, Germany; 2. Department of Neuroradiology, Philipps University, Marburg, Germany.

Address correspondence to C.V. Dalchow (dalchow{at}med.uni-marburg.de)

Objective: The digital volume tomography (DVT) technique is an extension of panoramic tomography. With this diagnostic approach, characterized by high resolution, a minimal section thickness of 0.125 mm, and three-dimensional display, small pathological processes can be well visualized.

Materials and Methods: Since May 2003 five-hundred and thirty-one patients were routinely examined prior to temporal bone surgery with DVT (Accu-I-tomo, Morita, Japan) to evaluate the lateral skull base for pathologies such as unclear conductive hearing loss or chronic otitis media. We used the Accu-I-tomo (Morita, Kyoto, Japan) to perform our examinations. During the exposure the X-ray tube rotates along with the opposite sensor 360 degrees around the center of a conical shaped radiation beam visualizing a cylindrical format 3 cm high and 4 cm wide (transverse diameter). The acquired data is analyzed with special software on a PC where the section angle, intersection distance, and slice thickness can be freely changed to visualize small bony structures at a resolution of 0.125 mm.

Results: By using DVT small bony pathologies of the lateral skull base can be analyzed prior to surgery. Especially erosions of the ossicular chain, external ear canal, semicircular canals, mastoid, and the cochlea could be visualized in detail. With the help of DVT the precise planning of surgery is feasible, reducing the risk of injuring important structures.

Conclusion: Digital volume tomography (DVT) expands the application of diagnostic possibilities in the lateral skull base. Therefore we believe improved pre-op diagnosis can be achieved along with more accurate planning of the surgical procedure. DVT delivers a small radiation dose and a high resolution coupled with a lower purchase price for the equipment.

* Will present paper

2:30 PM

255. Sebaceous Carcinoma of the Ocular Adnexa: CT and MR Imaging Findings

Kim H.1*; Kim Y.D.2; Baek J.H.3; Ko Y.4; Kim S.T.1; Weon Y.C.1; Jeon P.1; Byeon H.S.1; 1. Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea; 2. Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea; 3. Otolaryngology and Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea; 4. Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

Address correspondence to H. Kim (hyungkim{at}smc.samsung.co.kr)

Objective: The purpose of this study was to describe the CT and MR imaging findings of sebaceous carcinoma (SC) of the ocular adnexa.

Materials and Methods: The review of the medical records during last 10 years revealed 26 patients (M:F = 7:19; mean age, 65 y; range, 41-81y) with histologically proved SC of the head and neck. Of these 26, 10 patients (M:F = 2:8; mean age, 60y; range, 43-80y) underwent CT (n = 6) and/or MR imaging (n = 5), who formed the basis of this study. At the initial presentation at our hospital, 6 had a primary tumor (3 on the upper eyelid, 1 on the lower eyelid, 2 at the medial canthal area) and 4 had a metastatic tumor (3 in the parotid gland and 1 in the infraauricular lymph node). In 1 patient presenting with a primary medial canthal tumor, a metastatic parotid tumor was developed 9 months after resection of primary tumor. All 4 patients presenting with a metastatic tumor had a history of surgery for SC on the ipsilateral upper eyelid 2-10 years before. We investigated the CT and MR imaging findings of the primary and metastatic SC with particular attention to the margin and invasiveness.

Results: In 6 patients with primary ocular adnexal SC, CT and MR imaging showed an oval to ellipsoid soft tissue mass with a well-defined (n = 3) or ill-defined (n = 3) margin (0.6-2.9 cm in greatest diameter). Two tumors invaded the orbit, one of which also involved the bony maxilla and paranasal sinuses. Various signal intensity was demonstrated on T1- and T2-weighted MR images. Five tumors showed significant enhancement, and necrosis was noted in 1. In 4 patients with metastatic parotid SC, CT showed an ill-defined mass (2.5-12 cm in greatest diameter). Three tumors involved both superficial and deep lobes and 1 tumor involved only superficial lobe. The extraparotid extension was noted in 2. All 4 tumors enhanced well and contained considerable necrosis. Intratumoral calcification was detected in 2. In 1 patient with metastatic infraauricular lymphadenopathy, the involved node was a 1.8 cm well-defined, ovoid mass showing minimal enhancement.

Conclusion: SC of the ocular adnexa most commonly involves the upper eyelid of the elderly women and metastasizes frequently to the ipsilateral parotid gland via lymphatic spread. Both primary and secondary tumors can behave aggressively infiltrating into the adjacent structures. CT and MR imaging can provide the valuable informations on the locoregional invasiveness and lymphangitic metastasis by this aggressive tumor, thus helping guide the clinicians to proper management.

* Will present paper

2:40 PM

256. Dirty-Appearing White Matter in Multiple Sclerosis: 8 Year Follow-Up Study

Vertinsky T.1; Zhao G.J.2; Miropolsky V.1; Traboulsee A.2; Li D.K.1,2*; 1. Radiology, University of British Columbia, Vancouver, BC, Canada; 2. Division of Neurology, Medicine, University of British Columbia, Vancouver, BC, Canada.

Address correspondence to D.K. Li (david.li{at}ubc.ca)

Objective: Dirty-appearing white matter (DWM) has been described in patients with multiple sclerosis (MS) as diffuse areas of slightly increased signal intensity in the white matter distinct from the typical focal high signal intensity MS lesion. Previous studies have shown that patients with DWM had shorter disease duration and progressed faster clinically. The objective of the present study was to determine the prevalence and incidence of DWM in a group of MS patients followed for 8 years.

Materials and Methods: 348 relapsing-remitting MS patients originally enrolled in a randomized placebo-controlled treatment trial (PRISMS) were studied at baseline and 8 years later. For the first 2 years, patients were randomized to placebo, low dose and high dose interferon beta-1a treatment. Placebo patients were subsequently re-randomized to receive low or high dose treatment while treated patients continued with their blinded treatment for the next 4 years. For the final 2 years treatment was not controlled. Non contrast 5-mm thick transverse proton density and T2-weighted conventional spin echo and matching pre- and post-gadolinium enhanced T1 scans were obtained according to a protocol with strict repositioning criteria. 2 radiologists reviewed the scans initially independently and then together (by consensus with disagreements settled by a third radiologist) to identify the presence of DWM and the change over time. DWM was defined as areas of white matter, with a signal intensity that is slightly higher than normal white matter but much lower than typical MS lesions, measuring at least 10 mm in diameter and extending over at least 2 slices. The scans of 67 control subjects were also reviewed.

Results: DWM was seen in 3 of 67 (4%) control subjects and in 86 of 348 (25%) of the MS patients at baseline. At 8 year follow-up only 1 patient developed a new DWM. In the patients with DWM on the initial scan, DWM completely disappeared in 3 (4%) patients, decreased in 18 (21%) and increased in extent in 2 patients (2%). The changes in DWM over time may precede typical MS lesion formation and the development of brain atrophy.

Conclusion: The 25% prevalence of DWM did not change over an 8 year follow-up with the extent of DWM involvement being more likely to decrease or even disappear than show an increase over time.

* Will present paper

2:50 PM

257. Probabilistic Assessment of Progression of Signal Intensity Changes Reflecting Myelination in Normal Infants

Provenzale J.M.1*; Liang L.1; York G.1; DeLong D.1; 1. Radiology, Duke University Medical Center, Durham, NC.

Address correspondence to J.M. Provenzale (prove001{at}mc.duke.edu)

Objective: To provide normative data for MR signal intensity (SI) changes reflecting myelination in infants.

Materials and Methods: The study population consisted of 54 infants who were relatively equally distributed through first 12 months of life who underwent clinical axial T1WI and T2WI imaging (1.5 T scanner). All had normal scans and no neurological abnormality at 6 month clinical follow-up. Two blinded radiologists scored SI of 9 regions: frontal white matter (FWM), occipital WM (OWM), genu (GENU) and splenium (SPLEN) of corpus callosum, posterior limb (PLIC) anterior limb (ALIC) of internal capsule, coronal radiata (CR), cerebral peduncle (CP) and middle cerebellar peduncle (MCP). Scoring criteria for T1WI: -1 (mildly hypointense to cortex), 0 (isointense), 1 (mildly hyperintense), 2 (moderately hyperintense), 3 (markedly hyperintense); for T2WI: -1 (mildly hyperintense to cortex), 0 (isointense), 1 (mildly hypointense), 2 (moderately hypointense), 3 (markedly hypointense). We used ordinal logistic regression models to estimate ages at which probability of attaining Sl scores was 90% (90prob) with 95% confidence intervals [CI] (occasionally not determinable [nd]).

Results: At birth, on T1WI, PLIC, MCP and CR in all infants had a score of +1. By 6 months, SPLEN (4.9 [4.0-6.3]), ALIC (5.8 [4.5-8.3] and OWM (5.9 [5.1-7.7]) had a 90prob of +1 score. At 9 months, GENU (6.4 [5.6-7.7]), CP (7.2 [4.9-12.9]) and FWM (9.1 [8.0-11.3]) had a 90prob of +1 score. Ages of 90prob of score of +2 (markedly hyperintense) were 6.9 months [6.0-8.5] for SPLEN, 9.2 months [8.3-10.7] for GENU, 10.8 months [8.1-nd] for PLIC, and 12.4 months for CR [10.2-nd]. At birth, on T2WI, PLIC and MCP in all infants had a score of +1. By 6 months, SPLEN (6.0 [5.2-7.4]) had a 90prob of score of +1. By 9 months, GENU (7.4 [6.6-9.1]), CP (7.9 [6.5-10.7]) and ALIC (8.0 [6.9-11.0) had a 90prob of +1 score. By 12 months, CR (9.8 [8.2/nd] and OWM (11.1 [10.0-nd]) had a 90prob of score of +1 score; FWM remained isointense to cortex. Age of attaining 90prob of score of +2 (moderately hypointense) were 4.2 months [3.5-9.1] for MCP, 4.3 months [3.3-7.6] for PLIC, and 8.0 months [7.1-9.4] for SPLEN.

Conclusion: Our data correlated well with those reported in a non-quantitative manner by other authors [1] but offer the advantage of more specific dates and probability estimates for SI changes.

1. Barkovich AJ, et al. Normal maturation of the neonatal and infant brain: MR imaging at 1.5 T. Radiology 1988; 166:173-180

* Will present paper

3:00 PM

258. Correlation of Diffusion Tensor Imaging Measurements with Conventional MR Imaging and Clinical Scoring in Krabbe Patients

Peddi S.1; Escolar M.2; Poe M.2; Kurtzberg J.3; Provenzale J.M.1*; 1. Radiology, Duke University Medical Center, Durham, NC; 2. University of North Carolina, Chapel Hill, NC; 3. Pediatrics, Duke University Medical Center, Durham, NC.

Address correspondence to J.M. Provenzale (prove001{at}mc.duke.edu)

Objective: To compare (a) an established MR imaging scoring system [1] and (b) fractional anisotropy (FA) values with (c) neurological function scores in Krabbe disease children.

Materials and Methods: A retrospective analysis of 19 MR studies in 9 Krabbe infants was performed by 2 blinded neuroradiologists. Readers assessed disease severity using the Loes score (0 = normal, 32 points = markedly abnormal) for frontal WM (periventricular, subcortical and central WM), pyramidal tract (corona radiata, PLIC and brainstem) and whole brain. DTI parameters were TR 12,000 ms, TE 101 ms, b value 1,000 s/mm2 (6 directions, 1.5T). A third reader measured FA in 5 regions in frontal white matter [WM] and posterior limb of internal capsule [IC]. FA values were expressed as ratio of values in patients to 5 age-matched normal infants. Clinical scoring within 30 days of MR included age-adjusted developmental scores (developmental age/calendar age) for cognition, gross and fine motor function and overall development. Comparisons included: (1) Loes score in frontal WM and FA of frontal WM vs. cognition score, (2) Loes score in pyramidal tract and FA of PLIC vs. (2) gross motor score and (3) fine motor score, (4) total Loes score for whole brain and average FA for whole brain vs. overall developmental score.

Results: Mean Loes score for frontal WM was 1.53; mean FA for frontal WM was 0.77. Mean cognition score was 0.653. Correlation between Loes score and cognition score was -0.46 and for FA vs. cognition score was 0.43. Mean Loes score for pyramidal WM was 1.37; mean FA for PLIC was 0.67. Mean gross motor score was 0.495 and fine motor score was 0.680. Correlation between Loes score and gross motor score was -0.34 and for FA vs. cognition score was 0.38. Correlation between Loes score and fine motor score was -0.50 and that for FA vs. fine motor score was 0.44. Mean total Loes score was 8.47; mean FA for entire brain was 0.75. Mean composite score was 68.9. Correlation between total Loes score vs. overall developmental was -0.85 and for FA vs. overall developmental was 0.82.

Conclusion: DTI performed comparably to Loes scoring, which has the disadvantages of being labor-intensive and subjective. DTI provides a more objective, reproducible quantitative measure of WM integrity which are valuable for assessment of WM disorders. 1. Loes D, et al. Globoid cell leukodystrophy: distinguishing early-onset from late-onset disease using a brain MR imaging scoring method. AJNR 1999; 20:316-323

* Will present paper

3:10 PM

259. Correlation Between Swine Brain NAA Concentration Using PRESS Sequence with External Standard and HPLC Measurement

Wu R.H.1*; Lin R.1; Li H.1; Luo W.H.1; Rao H.B.1; Lang Z.J.2; 1. Medical Imaging, Shantou University Medical College, Shantou, Guangdong, China; 2. Radiology, Dalian Medical University First Hospital, Dalian, Liaoning, China.

Address correspondence to R.H. Wu (rhwu{at}stu.edu.cn)

Objective: Metabolite ratio is a useful method to express measurement results in the field of MR spectroscopy (MRS). However, a major disadvantage of the metabolite ratio is that ratio results are not comparable with absolute metabolite concentrations in vivo. The purpose of this study was to examine the accuracy of noninvasive quantification of brain N-Acetylaspartate (NAA) concentration using a MRS external standard method.

Materials and Methods: Eight swine were scanned on a GE 1.5 T scanner with a standard head coil. The external standard method was utilized with a sphere filled with NAA, GABA, glutamine, glutamate, creatine, choline chloride, and myo-inositol. The position resolved spectroscopy (PRESS) sequence was used with TE = 135 msec, TR = 1,500 msec, and 128 scan averages. The analysis of MRS was done with SAGE/IDL program. In vivo NAA concentration was obtained using the equation S = N * e(-TE/T2) * [1-e(-TR/T1). In vitro NAA concentration was measured by high performance liquid chromatography (HPLC).

Results: In the MRS group, the mean concentration of NAA was 10.03 ± 0.74 mmol/kg. In the HPLC group, the mean concentration of NAA was 9.22 ± 0.55 mmol/kg. There was no significant difference between the two groups (p = 0.46). However, a slightly higher value was observed in the MRS group (7/8 swine), compared with HPLC group. The range of differences was between 0.02~2.05 mmol/kg.

Conclusion: MRS external reference method is an accurate method to measure metabolite concentrations. 1H MRS does not easily distinguish between N-acetyl resonance frequencies and other N-acetylated amino acids.

* Will present paper

3:20 PM

260. Imaging Findings in a New Autosomal Recessive Syndrome Caused by Mutation in HOXA1 Gene

Alorainy I.A.1*; Bosley T.M.2,3; Salih M.A.4; Tischfield M.A.5,7; Sener E.C.8; Oystreck D.T.2; Chan W.M.5; Andrews C.5; Engle E.C.5,6,7; 1. Department of Radiology and Diagnostic Imaging, College of Medicine, King Saud University, Riyadh, Saudi Arabia; 2. Neuro-ophthalmology Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 3. Neuroscience Department, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia; 4. Division of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia; 5. Department of Medicine Program in Genomics, Children's Hospital Boston, Boston, MA; 6. Department of Neurology, Children's Hospital Boston, Boston, MA; 7. Program in Neuroscience, Division of Medical Sciences, Harvard Medical School, Boston, MA; 8. Department of Ophthalmology, Hacettepe University Hospital, Ankara, Turkey.

Address correspondence to I.A. Alorainy (alorainy{at}ksu.edu.sa)

Objective: To describe the imaging findings in head and neck region in patients with Bosley-Salih-Alorainy syndrome (BSAS), a newly described autosomal recessive syndrome caused by mutation in HOXA1 gene on chromosome 7p.

Materials and Methods: Nine patients with genetically proven mutation in HOXA1 gene manifested clinically as congenital horizontal eye movement abnormality (bilateral Duane syndrome), congenital sensorineural hearing loss, and absent or hypoplasic internal carotid arteries were evaluated radiologically and the head and neck CT and MR results analyzed.

Results: Imaging was available for inner ear in seven patients, five of them had bilateral common cavity deformity and two had cochlear aplasia. In these patients, the internal auditory canal was absent or severely narrowed. In one patient, the inner ear and IAC were normal, bilaterally. In six patients, the internal carotid arteries were abnormal. The left internal carotid artery (ICA) was hypoplastic in two and absent in three. In two of the three patients with absent left ICA, the cavernous portion of the contralateral right ICA was hypoplastic. Only one patient had absent ICA on both sides. This particular patient had also anomalous arterial connection between the two enlarged vertebral arteries anterior to the brainstem. In four patients with absent/hypoplastic left ICA, the right common carotid artery had very low bifurcation in the neckat about C7 level. In one patient, the right vertebral artery was duplicated. None of the patients had congenital brain anomalies or signs of ischemia.

Conclusion: In patients with horizontal eye movement disorder, the presence of inner ear malformation and internal carotid artery maldevelopment should raise the possibility of Bosley-Salih-Alorainy syndrome.

* Will present paper


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