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Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis

Souayah, Nizar; Pahwa, Ankit; Jaffry, Mustafa; Patel, Tejas; Nasar, Abu; Chong, Zhao Zhong; Sander, Howard W.
Objective: Since motor nerve conduction slowing can occur due to loss of large axons, we investigate the conduction slowing profile in amyotrophic lateral sclerosis (ALS) and identify the limits beyond which the diagnosis of exclusive axonal loss is unlikely. Methods: First, using linear regression analysis, we established the range of motor conduction slowing in 76 chronic inflammatory demyelinating polyneuropathy (CIDP) patients. Demyelinating range confidence intervals were defined by assessing conduction velocity (CV), distal latency (DML), and F-wave latency (F) in relation to distal compound muscle action potential (CMAP) amplitude of median, ulnar, fibular, and tibial nerves. Results were subsequently validated in 38 additional CIDP patients. Then, the newly established demyelination confidence intervals were used to investigate the profile of conduction slowing in 95 ALS patients. Results: CV slowing, prolonged DML, and abnormal F were observed in 22.2%, 19.6%, and 47.1% of the studied nerves respectively in ALS patients. When slowing occurred, it affected more than one segment of the motor nerve, suggesting that CMAP amplitude dependent conduction slowing caused by an exclusive loss of large axons is the main mechanism of slowing. No ALS patient had more than 2 nerves with CV slowing in the confidence interval defined by the regression equations or the American Academy of Neurology (AAN) research criteria for CIDP diagnosis. Conclusions: The presence of more than two motor nerves with CV slowing in the demyelinating range defined by the regression analysis or AAN criteria in ALS patients suggests the contribution of acquired demyelination or other additional mechanisms exist in the electrodiagnostic profile of ALS.
SCOPUS:85165307572
ISSN: 2405-8440
CID: 5548372

Differentiating Flail Limb Syndrome from Amyotrophic Lateral Sclerosis

Kornitzer, Jeffrey; Abdulrazeq, Hael F; Zaidi, Mohammad; Bach, John R; Kazi, Abdul; Feinstein, Erin; Sander, Howard W; Souayah, Nizar
OBJECTIVE:To investigate differences between flail limb syndrome (FLS) and amyotrophic lateral sclerosis (ALS). DESIGN/METHODS:Retrospective chart review identified 16 cases of ALS and 16 of FLS. Revised ALS Functional Rating Scale (ALSFRS-R), compound muscle action potential (CMAP) amplitudes, and rate of loss of vital capacity (ΔVC) were compared. RESULTS:Comparing ALS and FLS patients, ΔVC was 5.26% ± 0.33% vs. 0.54% ± 0.06%, respectively (p<0.05). No patient in FLS group had a ΔVC more that 0.65% per month. No patient in ALS group had a ΔVC less than 4.6% per month. Average ulnar nerve CMAP amplitudes were significantly lower in FLS (p<0.05). No significant difference was observed in rate of ALSFRS-R decline or average peroneal, tibial, and median nerve CMAP amplitudes. CONCLUSION/CONCLUSIONS:In FLS, an average monthly decrease in VC exceeding 0.65% may suggest a spread of motor neuron loss to higher cervical anterior horn areas and raise the possibility of progression to ALS. Larger prospective studies are needed to investigate the rate of VC decline in FLS and limb-onset ALS and to establish whether a cut-off score combining ΔVC and CMAP amplitude mainly of the ulnar nerve might predict progression of FLS to ALS, the knowledge of which can facilitate appropriate patient counseling.
PMID: 32251109
ISSN: 1537-7385
CID: 4377062

Radiologic abnormalities in patients with neuropathy

Zakin, Elina; Shin, Susan C; Sander, Howard W
ORIGINAL:0015372
ISSN: n/a
CID: 5053482

Multinodular and Vacuolating Neuronal Tumor - Are We Only Seeing the Tip of the Iceberg? [Meeting Abstract]

Huang, Hao; Croll, Leah; Sander, Howard; Bansal, Neil; Zan, Elcin; Kurzweil, Arielle
ISI:000475965903082
ISSN: 0028-3878
CID: 4029092

A Retrospective Analysis of the Safety Profile of Intravenous Immunoglobulin in 1176 Patients Receiving Home Infusion Therapy

Souayah, Nizar; Pahwa, Ankit; Burawski, Lauren; Opila, Tamara; Sander, Howard W
OBJECTIVES/OBJECTIVE:This analysis assessed the safety of intravenous immunoglobulin (IVIg) in the treatment of patients with neuroimmunological and immunological disorders in a home-based setting. METHODS:Adverse reactions (ARs) were assessed in a retrospective review of 1176 patients receiving 28,677 home-based IVIg infusions between 1996 and 2013. RESULTS:Of 1176 patients, 648 (55.1%) experienced IVIg-related ARs; 536 (45.6%) were mild, 78 (6.6%) moderate, and 34 (2.9%) severe. Thirty-seven (3.1%) patients were hospitalized because of ARs; of these, headache was most common (51.4%). Mean number of ARs per patient increased from 1.4 (low dose) to 3.6 (high dose). Incidence of ARs increased from 41% in the first 5-year moving average in 2003 to 65% in 2008. The number of ARs correlated with the number of infusions (ρ = 0.24; P < 0.001) and the average IVIg dose (ρ = 0.10; P < 0.001). CONCLUSIONS:Low- and high-dose IVIg were safe and well tolerated with a few serious ARs in patients with neuroimmunological and immunological disorders.
PMID: 29794573
ISSN: 1537-1611
CID: 3129472

Trends in outcomes and hospitalization costs among pediatric myasthenia gravis patients in the United States. Data from kids' inpatient database (KID) 1997-2009 [Meeting Abstract]

Sander, H; Elenein, R; He, W; Sheikh, Z; Al, Qudah Z; Souayah, N
OBJECTIVE: To assess the impact of new therapeutic strategies on the outcome and cost of hospitalization among pediatric patients with myasthenia gravis (MG) in the United States. BACKGROUND: Advances in understanding of MG has led to the development of more effective therapeutic strategies including pulmonary support and mechanical ventilation. DESIGN/METHODS: We determined the rates of occurrence, in-hospital outcomes, and mean hospital charges for pediatric patients hospitalized with MG from 1997 to 2009 using Kids' Inpatient Database (KID). RESULTS: There was a steady increase in the number of MG patients from 1997 (144) to 2009 (319). The average patient age varied significantly, with a minimum of 11.9+/-5.7 years in 1997 and a maximum of 14.1+/-5.8 years in 2000 (p=0.014). Female predominance was observed in all time periods. The average length of stay was not significantly different from 1997 to 2009. The maximum average length of stay was 6.3+/-6.5 days in 1997 and the minimum was 4.7+/-5.4 days in 2003 (p=0.22). The vast majority of patients were discharged to home in all studied time periods. During the studied time periods there was only one death, which occurred in 2006. There was a significant increase in average hospital charges over time: $24,382+/-42,678 in 1997 and $51,109+/-68,370 in 2009 (p<0.001). This difference persisted after adjustment for inflation. CONCLUSIONS: There was a steady increase in the number of pediatric MG patients from 1997 to 2009. There was a significant increase in in-hospital charges of patients admitted for MG from 1997 to 2009, but without any significant variation in average length of stay, death rate, or disposition status
EMBASE:71129782
ISSN: 0028-3878
CID: 509752

Trends in outcome and hospitalization charges of pediatric patients admitted with guillain barre syndrome in the United States. Data from kids' inpatient database (KID) 1997-2009 [Meeting Abstract]

Sander, H; Elenein, R; He, W; Sheikh, Z; Al, Qudah Z; Souayah, N
OBJECTIVE: To assess the impact of new therapeutic strategies on outcome and cost of hospitalization among pediatric patients with Guillain Barre Syndrome (GBS) in the United States. BACKGROUND: The advance in understanding of GBS has led to the development of more effective therapeutic strategies including pulmonary support and mechanical ventilation. DESIGN/METHODS: We determined the rates of occurrence, in-hospital outcomes, and mean hospital charges for pediatric patients hospitalized with GBS from 1997 to 2009 using the Kids inpatient Database (KID). RESULTS: There was a steady increase in the number of GBS patients from 1997 to 2009: 333 and 585 patients in 1997 and 2009 respectively. The average patient's age was significantly different in the studied time periods (minimum average age was 9.5+/-5.6 years in 1997 and maximum average age was 11.1+/-6.1 years in 2009, p<0.001). There was a non-significant reduction in the length of stay from 1997 (10.2+/-14.0 days) to 2009 (8.3+/-9.1 days). During the study time period, there were two deaths reported, both in 2003. There was a significant and steady increase in average hospital charges over time: $30,010+/-46,391 in 1997 and 73,538+/- 98,536 in 2009, p<0.001. This difference persisted after adjustment for inflation. CONCLUSIONS: There was a steady increase in the number of pediatric GBS patients from 1997 to 2009. There was a significant and steady increase in in-hospital charges of patients admitted for GBS from 1997 to 2009 that was paralleled by a significant increase in average age and a non significant reduction in the length of hospitalization
EMBASE:71129555
ISSN: 0028-3878
CID: 509762

Sleep disordered breathing and other sleep dysfunction in myotonic dystrophy type 2 [Letter]

Bhat, Sushanth; Sander, Howard W; Grewal, Raji P; Chokroverty, Sudhansu
PMID: 22959494
ISSN: 1389-9457
CID: 182942

Clinical approach to peripheral neuropathy: anatomic localization and diagnostic testing [Case Report]

Alport, Adina R; Sander, Howard W
PURPOSE OF REVIEW: This article provides a clinical approach to peripheral neuropathy based on anatomic localization and diagnostic testing. RECENT FINDINGS: Advances have been made in the evaluation of small fiber neuropathy and in the known genetic causes of neuropathy. SUMMARY: History and physical examination remain the most useful tools for evaluating peripheral neuropathy. Characterization of a neuropathy aids in limiting the differential diagnosis and includes consideration of temporal profile (tempo of onset and duration), heredity, and anatomic classification. Anatomic classification involves (1) fiber type (motor versus sensory, large versus small, somatic versus autonomic), (2) portion of fiber affected (axon versus myelin), and (3) gross distribution of nerves affected (eg, length-dependent, length-independent, multifocal). Diagnostic testing may include serologic and CSF evaluation, electrodiagnosis, skin biopsy, quantitative sensory testing, autonomic testing, nerve biopsy, confocal corneal microscopy, and laser Doppler imager flare.
PMID: 22810068
ISSN: 1080-2371
CID: 955492

Demyelinating neuropathy in patients with hepatitis C virus infection [Case Report]

Chin, Russell L; Sander, Howard W; Brannagan, Thomas H 3rd; De Sousa, Eduardo; Latov, Norman
Infection with hepatitis C virus (HCV) can be associated with demyelinating polyneuropathy that may be responsive to immunomodulatory therapy. In this case report series, we review four patients (all male, ages 47-60 years) with HCV and demyelinating polyneuropathy. Two of the four patients were diagnosed with HCV during the course of initial neuropathy evaluation. All patients had sensory loss, absent/diminished reflexes, lower extremity weakness (except for one patient), and demyelinating electrodiagnostic features. Three patients had polyclonal hypergammaglobulinemia and one patient had IgM monoclonal gammopathy. Intravenous immunoglobulin resulted in improvement in three patients; one patient had no benefit from rituximab therapy, but his symptoms have been stable. Demyelinating neuropathy may develop in patients with HCV unrelated to antiviral therapy. Immunomodulatory therapy may be beneficial in some cases. Testing for HCV should be considered, especially in patients with hypergammaglobulinemia or IgM monoclonal gammopathy
PMID: 20516810
ISSN: 1537-1611
CID: 112105