Comparative Outcomes of Respiratory Failure Associated with Common Neuromuscular Emergencies: Myasthenia Gravis versus Guillain-Barré Syndrome.

Background and objectives: Myasthenia gravis (MG) and Guillain-Barré Syndrome (GBS) are autoimmune neuromuscular disorders that may present as neuromuscular emergencies requiring mechanical ventilation and critical care. Comparative outcomes of these disease processes, once severe enough to require mechanical ventilation, are not known. In this study, we compared the patients requiring mechanical ventilation in terms of in-hospital complications, length of stay, disability, and mortality between these two disease entities at a national level. Materials and Methods: Mechanically ventilated patients with primary diagnosis of MG (n = 6684) and GBS (n = 5834) were identified through retrospective analysis of Nationwide Inpatient Sample (NIS) database for the years 2006 to 2014. Results: Even though mechanically ventilated MG patients were older (61.0 ± 19.1 versus 54.9 ± 20.1 years) and presented with more medical comorbidities, they had lower disease severity on admission, as well as lower in-hospital complications sepsis, pneumonia, and urinary tract infections as compared with GBS patients. In the multivariate analysis, after adjusting for confounders including treatment, GBS patients had significantly higher disability (odds ratio (OR) 15.6, 95% confidence interval (CI) 10.9-22.2) and a longer length of stay (OR 3.48, 95% CI 2.22-5.48). There was no significant difference in mortality between the groups (8.45% MG vs. 10.0% GBS, p = 0.16). Conclusion: Mechanically ventilated GBS patients have higher disease severity at admission along with more in-hospital complications, length of stay, and disability compared with MG patients. Potential explanations for these findings include delay in the diagnosis, poor response to immunotherapy particularly in patients with axonal GBS variant, or longer recovery time after nerve damage.

Racial/ethnic disparities in hospital utilization in intracerebral hemorrhage.

BACKGROUND AND PURPOSE: There is evidence that racial and ethnic differences among intracerebral hemorrhage (ICH) patients exist. We sought to establish the occurrence of disparities in hospital utilization in the United States. METHODS: We identified ICH patients from United States Nationwide Inpatient Sample database for years 2006-2014 using codes (DX1 = 431, 432.0) from the International Classification of Diseases, 9th edition. We compared five race/ethnic categories: White, Black, Hispanic, Asian or Pacific Islander, and Others ( Native American and other) with regard to demographics, comorbidities, disease severity, in-hospital complications, in-hospital procedures, length of stay (LOS), total hospital charges, in-hospital mortality, palliative care, (PC) and do not resuscitate (DNR). We categorized procedures as lifesaving (i.e. ventriculostomy, craniotomy, craniectomy, and ventriculoperitoneal (VP) shunt), life sustaining (i.e. mechanical ventilation, tracheostomy, transfusions, and gastrostomy). White race/ethnicity was set as the reference group. RESULTS: Out of 710,293 hospitalized patients with ICH 470,539 (66.2%), 114,821 (16.2%), 66,451 (9.3%), 30,297 (4.3%) and 28,185 (3.9%) were White, Black, Hispanic, Asian or Pacific Islander, and Others, respectively. Minorities (Black, Hispanic, Asian or Pacific Islander, and Others) had a higher rate of in-hospital complications, in-hospital procedures, mean LOS, and hospital charges compared to Whites. In contrast, Whites had a higher rate of in-hospital mortality, PC, and DNR. In multivariable analysis, all minorities had higher rate of MV, tracheostomy, transfusions, and gastrostomy compared to Whites, while Hispanics had higher rate of craniectomy and VP shunt; and Asian or Pacific Islander and Others had higher rate of craniectomy. Whites had a higher rate of in-hospital mortality, palliative care, and DNR compared to minorities. In mediation analysis, in-hospital mortality for whites remained high after adjusting with PC and DNR. CONCLUSION: Minorities had greater utilization of lifesaving and life sustaining procedures, and longer LOS. Whites had greater utilization of palliative care, hospice, and higher in-hospital mortality. These results may reflect differences in culture or access to care and deserve further study.

Is black tar heroin use associated with wound botulism? A report of two Hispanic patients.

Wound botulism is a potentially lethal condition that can cause paralysis. Its association with black tar heroin is a well-established fact. It is essential to alert clinicians in recognizing the patients with history of injection drug abuse presenting with clinical features of botulism early on admission for prompt diagnosis and treatment.

A 31-year-old with idiopathic reversible cerebral vasoconstriction syndrome.

In our patient with reversible cerebral vasoconstriction syndrome (RCVS) syndrome, presenting with thunderclap-like headache, there is a possibility to be readily confused with migraine. Initiating treatment with selective serotonin reuptake inhibitors (SSRIs) and triptans can further aggravate the condition. Therefore, it is essential to understand the nature and type of headache and correlate the clinical findings with imaging studies.

A 72-year-old with eyelid opening apraxia in Steele-Richardson-Olszewski syndrome.

In our patient with Steele-Richardson-Olszewski syndrome presenting with apraxia of eyelid opening, there is a possibility to be readily confused with conditions presenting in similar pattern such as blepharospasm and eyelid ptosis. Therefore, it is essential to correlate the clinical findings with electromyographic recordings and dopamine transporter scan (DaT).

Black Tar Heroin Skin Popping as a Cause of Wound Botulism.

BACKGROUND: Botulism is a rare potentially fatal and treatable disorder caused by a bacteria-produced toxin that affects the presynaptic synaptic membrane resulting in a characteristic neuromuscular dysfunction. It is caused by either the ingestion of the toxin or the bacteria, inhalation, or wound infection. We present our observations with a descriptive case series of wound botulism secondary to black tar heroin (BTH) injection. METHODS: We report a retrospective single-center case series of 15 consecutive cases of wound botulism presenting to University Medical Center of El Paso. Medical records where reviewed to obtain demographic information, clinical presentation, treatment, and outcome. RESULTS: We identified fifteen patients with mean age of 47 years: twelve men, and three women. All had administered BTH through skin popping and had abscesses in the administration areas. By history, the most common symptoms were dysphagia (66%), proximal muscle weakness of upper and lower extremity (60%), neck flexor muscle weakness (33%), ophthalmoplegia (53%), bilateral ptosis (46%), dysarthria (53%), double vision (40%), blurred vision (33%), and dry mouth (20%). During the examination, the most common features noted were: proximal muscle weakness of upper and lower extremities (73%), ophthalmoplegia (53%), ptosis (46%). In patients with documented wound botulism, the pupils were reactive in 46%. All patients required mechanical ventilation and were treated with the trivalent antitoxin. Eleven patients (73.3%) were discharged home, two were transferred to a skill nursing facility, and two were transferred to long-term acute care facility. CONCLUSION: In our patients, BTH injection, involving the action of injecting under the skin acetylated morphine derivatives (mostly 6-monoacetylmorphine and 3-monoacetylmorphine), was associated with the development of botulism. The availability of BTH at the US-Mexican border is not surprising since it is frequently produced in Latin America. Its association with the development of botulism should be recognized early to allow a prompt diagnosis and treatment with the antitoxin. A clinical feature worth noting is the presence of normal pupillary light reflex in nearly half of patients. Therefore, the presence of a normal pupillary response does not exclude the presence of wound botulism.

Case Report: A case report of Moyamoya disease in a 36 year old African American woman.

Moyamoya is a rare idiopathic progressive vaso-occlusive disease characterized by irreversible condition of main blood vessels to the brain as they enter into the skull. We present a case of 36 year old African American female presenting to the Out Patient Clinic with headache which were on and off for 4-6 months and did not relieve on routine medical therapy. It was associated with weakness on right side for last few days. The patient was investigated with CT Angiogram, diagnosed as Moyamoya disease and operated. She has been followed up for the last 5 years and the patient has not complained of any headaches or focal neurological symptoms.

Case Report: Gollop-Wolfgang Complex in a 5 month old baby.

Skeletal dysplasias are disorders associated with a generalized abnormality in the skeleton. The Gollop-Wolfgang complex (GWC) is a limb deficiency disorder and an unusual limb malformation with highly variable manifestations. Here we report an interesting case of a 5-month old male baby from India with Gollop-Wolfgang Complex showing bifurcation of the right femur, ectrodactyly of both feet, ectrodactyly of left hand, syndactyly of right hand and unusual presentation of bilateral fibular agenesis and caudal (Sacrococcygeal) agenesis. The etiology of GWC in this 5 month old male baby could possibly be attributed to spontaneous gene mutation. The clinical, radiographic findings and the unusual presentation are presented in detail.