Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review.

Hirayama disease is a rare neurological entity that is characterized by initial progressive muscular wasting and weakness of the distal upper limb in young men, followed by a spontaneous arrest within several years. The disease is believed to be a result of forward displacement of the cervical dural sac and spinal cord induced by neck flexion. It is commonly seen in Asia and rarely encountered in the Middle East countries. We report a rare case of a 20-year-old Kuwaiti patient presenting with a 10-month duration of gradual left upper limb weakness and wasting. We describe his electrophysiological and radiological findings that confirmed the diagnosis, and conducted a literature review. Hirayama disease is rarely encountered in clinical settings and should be suspected in male patients presenting with unilateral or asymmetrical bilateral lower motor weakness of hands and forearms. It is a benign entity, and cervical collar is usually the only treatment needed in most cases.

Radiological characteristics of neuromyelitis optica spectrum disorder in Kuwait.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating disorder of the central nervous system that predominantly targets optic nerves and spinal cord. Studies of NMOSD are scarce in the Middle East. OBJECTIVE: To evaluate the MRI characteristics of NMOSD patients in Kuwait. PATIENT AND METHODS: This is an observational, retrospective study on NMOSD patients who attended the MS clinic. Patients who fulfilled the 2015 diagnostic criteria of NMOSD were included. Patients` clinical, radiological and serological data were extracted from the medical records. The radiological variables were compared according to gender and AQP4 serostatus. RESULTS: Forty-two patients fulfilling the NMOSD diagnostic criteria. The mean age and mean age of onset were 32.6 ± 11.4 and 28.9 ± 9.8 years respectively. Females represented 83.3 % of the cohort with female-to-male ratio of 5:1. Thirty-one patients (73.8 %) tested positive for AQP4 antibody. Nineteen patients (45.2 %) had bilateral optic nerve involvement, while chiasmal involvement was seen in 8 (19.0 %) patients. Spinal cord was involved in 36 (85.7 %) patients; of whom 27 (64.3 %) had LETM. The most common spinal segment involved was the cervical (72.2 %) followed by the dorsal (25.0 %) regions. The brain was involved in 39 (92.8 %) patients and the periventricular region around fourth and lateral ventricles was the most commonly involved site (n = 35; 83.3 %), along with periaqueductal (n = 25; 61.9 %) and corpus callosal (n = 24; 57.1 %) regions. Isolated area postrema involvement was observed in 9 (21.4 %) patients. CONCLUSION: This is the first study describing the radiological characteristics of NMOSD in Kuwait. Although our data is comparable with the previous international studies, a higher percentage of bilateral optic nerve, brain, and callosal involvement was observed. Further multicenter studies with a larger cohort are needed to confirm our results.

Effect of Sphenoid Sinus Pneumatization on the Surgical Windows for Extended Endoscopic Endonasal Transsphenoidal Surgery.

BACKGROUND: Little is known on the impact of the pattern and extent of pneumatization of the sphenoid sinus (SS) on the dimensions of the surgical windows used in extended endoscopic endonasal transsphenoidal approaches. We therefore investigated whether the distances between the 2 optic canals and between the paired paraclival carotid arteries are influenced by the pattern and extent of pneumatization of the SS. METHODS: One hundred high-resolution computed tomography scans from 47 adult female and 53 adult male patients were analyzed. The pattern of SS pneumatization was classified into conchal, presellar, and sellar types. Sellar-type sinuses were then classified according to a newer detailed classification system. Maximal anteroposterior (AP), transverse (TR), and craniocaudal diameters of the SS, interoptic distance at the limbus sphenoidale (IODL) and at the entrance of the optic canal (IODE) and the intercarotid distance between the paraclival carotids (ICD) were measured. A 2-tailed Mann-Whitney U test and Pearson correlation coefficient (R) were used for statistical analysis. A P value <0.05 was considered statistically significant. RESULTS: Positive correlation was found between IODL and both AP and TR diameters of the SS; between IODE and both AP and TR diameters of the SS; and between ICD and all diameters of the SS. The highest correlation for each of the IODL, IODE, and ICD was noted with the TR diameter of the SS. CONCLUSIONS: During the development of the SS, pneumatization progress likely exerts quantitative and direction-specific forces, which gradually increase the interoptic and intercarotid distances.

Endoscopic Endonasal Excision of Large and Giant Pituitary Adenomas: Radiological and Intraoperative Correlates of the Extent of Resection.

BACKGROUND: Despite the superb visualization offered by the endoscopic endonasal transsphenoidal approach, the resection rates of large and giant pituitary adenomas have remained much lower than those of smaller macroadenomas. Various tumor characteristics can influence the extent of resection (EOR) and have been variably reported. Additional understanding of these factors is mandatory to improve the results. We analyzed the radiological and intraoperative tumor characteristics influencing the EOR in a cohort of patients with large and giant pituitary macroadenomas undergoing endoscopic endonasal transsphenoidal excision under our care. METHODS: Twenty-eight patients were included. Magnetic resonance images were retrospectively analyzed for pre- and postoperative tumor volumetric analysis; preoperative tumor volume calculation using the formula (A × B × C/2); preoperative radioanatomical characteristics, including tumor shape, radiological structure, contrast enhancement, and extension; and the EOR. Intraoperative data were retrieved and included. RESULTS: The preoperative calculated tumor volume was 38.14 ± 23.02 cm3 and the preoperative measured tumor volume was 50.345 ± 17.36 cm3. A statistically significant difference was found between the calculated and measured tumor volumes for the whole cohort and for tumors with a maximum diameter >3.9 cm. A statistically significant difference in the EOR was found at a volume threshold of 26.2 cm3. Large cysts, heterogeneous enhancement, Knosp grade ≤2, soft tumor consistency, and tumor hemorrhage were significantly associated with gross total resection. CONCLUSIONS: Volumetric analysis should replace 2-dimensional methods in determining the size of large and giant pituitary adenomas. Specific tumor characteristics were associated with the EOR and could help in predicting the EOR for these tumors.

Efficacy and Safety of the Rotational Technique for Endoscopic Transforaminal Excision of Colloid Cysts of the Third Ventricle.

BACKGROUND: The main criticism of endoscopic excision of colloid cysts of the third ventricle is the decreased ability to completely resect the cyst wall, therefore increasing the risk of recurrence. The extent of resection varies widely across the endoscopic series and is largely influenced by the surgical technique. We report the results of the rotational technique for endoscopic transforaminal excision of colloid cysts in a series of consecutive patients. Our objective is to contribute to the current literature and to shed more light on an effective and safe yet less commonly used technique. METHODS: Retrospective analysis was performed on 19 patients. Preoperative magnetic resonance imaging (MRI) was evaluated for cyst characteristics. Postoperative MRI was evaluated for residual cyst membranes. Operative records were reviewed for residual cyst components. Excision grade was determined based on the Barrow Neurological Institute grading scale. Symptom resolution was documented clinically. RESULTS: The study included 13 men and 6 women, with a mean age of 35 years (range, 19-56 years) and mean follow-up of 27.74 months (range, 4-55 months). Total excision was achieved in 17 of 19 cysts (89.5%). A small residual was seen intraoperatively but not radiographically in 1 patient. In another patient, residual cyst membrane seen intraoperatively and radiologically led to recurrence 18 months postoperatively. No mortalities or permanent morbidities occurred. CONCLUSIONS: Our high total excision rate and low complication profile are in concordance with the recent reports of endoscopic resection of colloid cysts. The rotational technique for the endoscopic transforaminal approach is highly effective and a safe alternative to the bimanual dissection technique.

Endoscopic treatment of adult idiopathic obstruction of the foramen of Monro.

BACKGROUND: Adult idiopathic membranous obstruction of the foramen of Monro is an extremely rare condition that can be effectively treated with endoscopic foraminoplasty. A unilateral or bilateral foraminoplasty is performed if one or both of the foramina of Monro are obstructed, respectively. Endoscopic septum pellucidotomy is usually used in combination with the foraminoplasty. METHODS: The operative chart and video of one of our patients undergoing endoscopic treatment for adult idiopathic membranous obstruction of the foramen of Monro were retrieved from our database and reviewed. A description of the surgical technique was then formulated. CONCLUSIONS: The surgical technique of endoscopic foraminoplasty plus septum pellucidotomy for adult idiopathic membranous obstruction of the foramen of Monro is described.

Infundibular Recess Angle Reduction After Endoscopic Third Ventriculostomy: Does It Reflect Clinical Success?

BACKGROUND: Although the ventricular size is significantly reduced after endoscopic third ventriculostomy (ETV) in most successfully treated patients, ventricular size reduction is not always seen after a successful ETV. Practical and reliable radiologic parameters are still needed to assess the clinical success of an ETV. METHODS: We retrieved the clinical and radiologic data of patients who underwent an ETV. Patients with the following criteria were included: (1) preoperative magnetic resonance imaging studies available, (2) postoperative magnetic resonance imaging studies done within the first 2 postoperative weeks, and (3) the infundibular recess clearly visible on preoperative and postoperative sagittal magnetic resonance imaging. Preoperative and postoperative measurements of the angle of the infundibular recess of the third ventricle were performed on midsagittal T1-weighted, T2-weighted, fast imaging employing steady-state acquisition, or constructive interference in steady state images. RESULTS: The extent of reduction of the infundibular recess angle predicted the clinical outcome of ETV during the early postoperative period with a high degree of accuracy. The average reduction was about 48% in successful procedures versus only 15% in failed procedures. CONCLUSIONS: The degree of reduction of the angle of the infundibular recess of the third ventricle correlated with the amount of third ventricular decompression after ETV. Most importantly, such a reduction was noted to occur during the early postoperative period when radiologic changes are less pronounced. Assessment of change in infundibular recess angle measurement is easy to perform and may prove helpful in cases with no clear-cut clinical evidence of success of ETV.

Endoscopic management of a choroid plexus cyst of the third ventricle: case report and documentation of dynamic behavior.

PURPOSE: The aim of this study is to document the dynamic behavior of a choroid plexus cyst of the third ventricle. Although these lesions may float freely within the ventricle leading to intermittent obstruction of the cerebrospinal fluid (CSF) circulation at variable points in a single patient, such a phenomenon has only been documented using cranial ultrasonography and was never observed intraoperatively. METHODS: We endoscopically treated a case of third ventricular choroid plexus cyst in a 9-year-old boy who presented with headaches and disturbed conscious level. He underwent a transventricular approach through a single burr hole. RESULTS: During the procedure, the cyst was noted to intermittently herniate into the lateral ventricle and recede back through the foramen of Monro. Endoscopic ablation of the cyst was achieved and followed by endoscopic third ventriculostomy (ETV). The patient made an excellent recovery after the procedure. CONCLUSIONS: We were able to endoscopically observe the dynamic behavior displayed by a choroid plexus cyst of the third ventricle. To the best of our knowledge, intraoperative documentation of the obstruction of the CSF pathway by a single choroid plexus cyst that intermittently herniates through the foramen of Monro and back into the third ventricular cavity has not been previously demonstrated neither microsurgically nor endoscopically.