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Background and purposeTo describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria as the first and predominant sign of a presumed neurodegenerative disease.MethodsParticipants were 25 patients with spastic dysarthria as the only or predominant speech disorder. Clinical features, pattern of MRI volume loss on voxel‐based morphometry and pattern of hypometabolism on F18‐fluorodeoxyglucose positron emission tomography (FDG‐PET) scan are described.ResultsAll patients demonstrated speech characteristics consistent with spastic dysarthria, including strained voice quality, slow speaking rate, monopitch and monoloudness, and slow and regular speech alternating motion rates. Eight patients did not have additional neurological findings on examination. Pseudobulbar affect, upper motor neuron pattern limb weakness, spasticity, Hoffman sign and positive Babinski reflexes were noted in some of the remaining patients. Twenty‐three patients had electromyographic assessment and none had diffuse motor neuron disease or met El Escorial criteria for amyotrophic lateral sclerosis. Voxel‐based morphometry revealed striking bilateral white matter volume loss affecting the motor cortex (BA 4), including the frontoparietal operculum (BA 43) with extension into the middle cerebral peduncle. FDG‐PET showed subtle hypometabolism affecting the premotor and motor cortices in some patients, particularly in those who had a disease duration longer than 2 years.ConclusionsA neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways is characterized. The descriptive label ‘progressive spastic dysarthria’ to best capture the dominant presenting feature of the syndrome is proposed.

Objectives: Peroneal nerve injuries are not uncommon with multiligament (ML) knee dislocations. However, there remains little information on outcome of these injuries. Therefore, the purpose of this study was to (1) report the clinical and functional outcomes after ML knee injury with concomitant peroneal nerve palsy, (2) compare recovery and outcome with partial versus complete peroneal nerve injury, (3) compare outcomes to a matched cohort of ML knee injuries without concomitant peroneal nerve palsy. Methods: Patients that underwent surgical reconstruction of a ML knee injury from 1993 to 2010 were reviewed and concomitant nerve injuries were identified, forming the peroneal nerve injury group (PI). Patient demographics, injury mechanism, modified Schenck classification, and peroneal nerve function, treatment, and recovery were documented. Patients were divided into two sub-groups: complete peroneal nerve palsy (CNP) and partial peroneal nerve palsy (PNP). A second group of patients with ML knee injury without peroneal nerve injury were identified, forming the no nerve injury group (NI). The PI and NI groups were matched based on Modified Schenck Classification, age, and gender. Peroneal nerve motor grade was assessed at last clinical follow-up and outcome scores were obtained using the International Knee Documentation Committee (IKDC) and Lysholm forms at a minimum of 2 years post-op. Data was analyzed using univariate and multivariate models to assess for predictive factors between the groups. Results: 27 patients (24 males, 3 females) met inclusion criteria for the PI group (9 CNP and 18 PNP). Treatment for CNP included an AFO for all patients, non-operative (1), neurolysis (2), tendon transfer (3), nerve transfer (1), and combined nerve/tendon transfer (1). One patient in the CNP group required additional surgery, undergoing below knee amputation. Treatment for PNP included non-op (12), neurolysis (4), nerve transfer (1), and combined nerve/tendon transfer (1). 2 patients with incomplete nerve palsy required additional surgery, undergoing an arthrodesis and a total knee arthroplasty. 3 CNP (37.5%) and 15 PNP (83.3%) regained 3/5 or greater tibialis anterior (TA) strength. PNP was associated with increased recovery of 3/5 extensor hallucis longus (EHL) strength (p=0.0093). Outcome scores were obtained for 17 of the remaining patients at a mean 6.6 (range 2-18) years. Univariate analysis revealed no difference between the CNP and PNP groups. Final motor grade of 3/5 did not predict improved outcomes. Not undergoing a tendon transfer procedure predicted better outcomes on the IKDC score (70.5 vs 45.0; p=0.027). All other peroneal nerve interventions were not associated with improved motor or functional outcome. 20 patients formed the NI group. The PI and NI groups were matched on gender (18 M, 2 F), age (mean 30.8 vs 29.5 years), and Modified Schenck Classification (2 KD-I, 10 KD-IIIL, 5 KD-IV, 3 KD-V vs 2, 7, 8, and 3 respectively). In univariate analysis, the PI group had inferior outcomes on both the IKDC (64.5 vs 77.3; p=0.036) and Lysholm (67.1 vs 83.0; p=0.023) scores. Conclusion: Patients with partial peroneal nerve palsy are more likely to regain antigravity EHL strength, but not antigravity TA strength. There is no significant difference in outcomes between partial and complete peroneal nerve palsy. In ML knee injuries, patients with peroneal nerve injury have significantly worse outcomes than similar patients without peroneal nerve injury.

Objectives: Arthroscopic knee procedures are commonly performed in pediatric/adolescent patients. Reported complications following these procedures are low, however, no childhood specific data exists. Therefore, the purpose of this study is to determine the acute complications (within 6 months) of arthroscopic knee procedures in patients aged 17 years or less. Methods: This is a retrospective review of patients aged 17 years or less who underwent an arthroscopic knee procedure from 1997 to 2009 at a single institution. Demographic and surgical data was collected, in addition to specific data on intra-operative and post-operative complications. Minor complications included peripheral nerve block failure, regional anesthesia failure requiring conversion to general anesthesia, superficial wound infection/dehiscence, persistent knee effusion requiring aspiration, and sensory nerve dysesthesia. Major complications included death, major medical complication, septic arthritis, wound requiring repeat closure, arthrofibrosis, equipment failure, and revision surgery. Results: 1015 patients (555 males (54.7%), 460 females (45.3%)) with average age 15.3 (range 4-17) years were analyzed. The average operative time was 133.9 minutes (range 14-520). 467 (46%) underwent ACL reconstruction, 465 (45.8%) underwent synovectomy, treatment of an OCD lesion, meniscal treatment, or lateral release, 52 (5%) had a medial patellofemoral ligament reconstruction +/- tibial tubercle transfer, 17 (1.7%) arthroscopic fixation of a tibial eminence fracture, and 14 (1.4%) other ligament reconstruction. There were 147 (14.5%) total complications recorded. Major complications occurred in 21 (1.4%) of patients and minor complications in 126 (12.4%) of patients. Major complications included: intra-articular instrument breakage in 1(0.098%), septic arthritis in 4 patients (0.394%), 9 wounds requiring repeat closure (0.886%), arthrofibrosis in 5 patients (0.492%), unplanned subsequent surgical procedure in 3 patients (0.002%), and death in 1 patient (0.098%). 2 patients were readmitted to the hospital (1 DIC, 1 a-fib and syncope). There were no pulmonary emboli or DVTs, and no vascular injuries. No patients developed CRPS. Minor complications included:5 patients (0.492%) sensory nerve paresthesias, 10 (0.985%) patients with failed regional anesthetic, 16 (1.57%) patients with post-operative pain pump that required early discontinuation, 18(1.77%) patients with superficial wound infection/irritation, 60 (5.91%) patients with persistent effusion/hemarthrosis requiring arthrocentesis, and 17(1.67%) patients had medical problems that required intervention (asthma exacerbation, urinary retention). Conclusion: Major complications following knee arthroscopy in children and adolescents are relatively low 1.4%. Minor complications are more common (12.4%) but did not alter the post-operative course or recovery. DVT, PE, and CRPS did not occur in this patient cohort.

Objectives: TT-TG distance is a commonly used measurement for surgical decision-making in patients with patellofemoral malalignment and instability. This measurement has historically been performed utilizing axial CT scans. More recently, MRI has been proposed as an equivalent test for measurement of TT-TG distance. We sought to determine the reliability of TT-TG measurements on both MRI and CT, and to determine whether the measurements can be used interchangeably. Methods: All patients diagnosed with patellar instability who had received both CT and MRI of the knee between 2003 and 2011 were included (n=59 knees in 54 patients). Two fellowship trained musculoskeletal radiologists measured the TT-TG distances for each patient by CT and MRI in a randomized, blinded fashion for a total of 236 measurements. Inter-observer reliability was calculated between radiologists for both imaging modalities and inter-methods reliability was calculated between the two imagining modalities. The results are reported using intraclass correlation coefficients (ICC) and Bland Altman analysis (BAA). Results: The 59 knees had a mean TT-TG distance of 16.9 mm (range: 8.3-25.8) by CT and 14.7 mm (range: 1.5-25.1) by MRI. Eighteen patients (31%) had a TT-TG ≥ 20 mm by CT, and only 9 (15%) had a TT-TG ≥ 20 mm by MRI. Inter-observer reliability between the radiologists was considered excellent for both CT and MRI (ICC = 0.777 and 0.843 respectively). When comparing CT to MRI, however, the ICC was considered only fair for each of the two raters (0.532 and 0.539). A total of 11 patients (19%) had a TT-TG ≥ 20 mm on CT preoperatively and underwent distal realignment by tibial tubercle osteotomy (TTO). In this surgical subgroup, the mean TT-TG on CT was 22.5 mm (range 19.8-25.8) while the mean TT-TG on MRI was only 18.7 mm (range 14.4-22.8). Conclusion: TT-TG distance can be measured with excellent inter-rater reliability on both MRI and CT scans; however, the values derived from these two tests may not be interchangeable. This observation should be taken into consideration when MRI is used for surgical planning since MRI may underestimate TT-TG distance when compared to CT.

Purkinje cell cytoplasmic antibody type 1 (PCA-1), or anti-Yo, is the most frequently detected autoantibody in paraneoplastic cerebellar degeneration (PCD). The vast majority of cases of anti-Yo PCD, however, occur in females over 60 years old and are associated with gynecologic tumors. Only 10 cases have been reported in males, and only 2 were associated with cancer of the lung. Here we describe the youngest known case of PCA-1 positive PCD in a male, whose lung tumor was undetectable even on FDG-PET.