Acute Thrombotic Thrombocytopenic Purpura: Rare and Life-Threatening Side Effect of Recent BNT-162b2 COVID-19 Vaccination.

Description Thrombotic thrombocytopenic purpura (TTP) is a rare, potentially life-threatening disorder characterized by uncontrolled and spontaneous clot formation throughout the body. Known secondary causes of TTP include malignancy, bone marrow transplantation, pregnancy, various medications, and HIV infection. TTP in the setting of COVID-19 vaccination is rare and not well reported. Reported cases have been confined primarily to the AstraZeneca and Johnson and Johnson COVID-19 Vaccines. TTP in the setting of Pfizer BNT-162b2 vaccination has only recently been reported. We present a patient with no obvious risk factors for TTP who presented with acute altered mental status and was found to have objective evidence of TTP. To our knowledge, there are very few reported cases of TTP in the setting of a recent Pfizer COVID-19 vaccination.

Intraprocedural Back Pain Associated with Awake Neuroendovascular Procedures.

BACKGROUND AND PURPOSE: The prevalence and characteristics of intraprocedural back pain is not well studied in awake patients undergoing neuroendovascular procedures. METHODS: We performed a prospective study as part of quality improvement initiative in which all patients who underwent neuroendovascular procedures in awake state were inquired regarding presence, severity (using a numeric rating scale score ranging from 0 [no pain] to 10 [worst pain possible]), and location (using anatomical chart) of back pain immediately after the procedure. The primary endpoint was the proportion of patients with moderate to severe pain (score of ≥3). RESULTS: A total of 100 (41.3%) of 242 patients reported intraprocedural back pain with a median severity of 5/10 (range 1-10). The mean age was 58.7 ± 16.2 years. The mean duration of the procedure was 82.3 minutes (range 15-410 minutes). The pain was classified as moderate to severe in 86 of 100 patients. The locations of pain were identified in lumbar (n = 77), thoracic (n = 6), cervical (n = 7), cervical and lumbar (n = 8), and cervical with thoracolumbar (n = 2) regions. There was a significant relationship between patients' history of the previous neck and/or back surgery and frequency of moderate to severe back pain (P = .02). No significant relationship was observed between frequency of none to mild and moderate to severe back pain among the strata by patients' age, body mass index, or duration of procedures. CONCLUSIONS: The relatively high prevalence of intraprocedural back pain in patients undergoing neuroendovascular procedures in awake state must be recognized, and strategies to reduce the occurrence need to be identified.

The Effect of Ticagrelor on Platelet Reactivity in Patients with Clopidogrel Resistance Undergoing Neuroendovascular Procedures.

BACKGROUND AND PURPOSE: Suboptimal platelet inhibition by clopidogrel (clopidogrel resistance) may be associated with high rates of stent thrombosis and ischemic events. Our objective was to determine if ticagrelor, a P2Y12 receptor inhibitor, can result in platelet inhibition in patients with clopidogrel resistance. METHODS: A thromboelastography-platelet mapping assay was used in all patients undergoing neuroendovascular procedures requiring oral clopidogrel. In patients with suboptimal platelet inhibition (<60%) on clopidogrel, ticagrelor was imitated after an oral bolus of 180 mg followed by 90 mg twice daily and the platelet mapping assay was repeated. The primary endpoint was hemorrhagic complications classified as major (hemoglobin decrease >5 g/dL or intracranial hemorrhage with deficits), minor (hemoglobin decrease 3-5 g/dL or intracranial hemorrhage without residual deficits), or insignificant. RESULTS: Suboptimal platelet inhibition on clopidogrel was seen in 70 of 106 patients undergoing neuroendovascular procedures. There was a significantly higher magnitude of platelet inhibition with ticagrelor compared with clopidogrel in patients with clopidogrel resistance (mean ± SD: 85.90 ± 10.74% vs. 29.26 ± 17.71%; P < .001); 50 of 70 patients showed optimal inhibition. Two patients had major (fatal) hemorrhagic events (both received either intravenous thrombolytics and/or eptifibatide infusion). Three patients had minor hemorrhagic events, and two patients had insignificant hemorrhagic events. Four of seven hemorrhagic events occurred in patients with optimal response to clopidogrel, two occurred in patients with suboptimal response to ticagrelor, and one occurred in a patient with optimal response to ticagrelor. CONCLUSIONS: Oral ticagrelor can augment platelet inhibition in patients who have clopidogrel resistance.

Internal Carotid Artery Aplasia in a Patient With Nail-Patella Syndrome.

Nail-patella syndrome (NPS) is a rare disorder characterized by abnormal development of ectodermal and mesodermal tissues. Classically, NPS presents as a triad of nail dysplasia, dysplastic patellae, and bony exostoses of the ilia known as "iliac horns." Apart from dermatological and skeletal abnormalities, patients may also have involvement of ophthalmologic and renal systems. The underlying molecular etiology in NPS is the mutation of LMX1B homeobox gene which results in loss of function of its protein also called LMX1B, a DNA-binding protein belonging to the larger LIM-homeodomain transcription factor family. Normal LMX1B gene and protein function are essential for dorsalization of the vertebrate limb bud, development of anterior eye structures, skull formation, and differentiation and migration of neurons in the central nervous system. We report a case of confirmed NPS presenting with congenital aplasia of the internal carotid artery and believe this is the first report of cerebrovascular developmental abnormality associated with NPS.

Sleep-related Eating Disorder in a Patient with Parkinson's Disease.

Sleep disorders constitute a major aspect of the non-motor symptoms of Parkinson's disease (PD). Rapid eye movement (REM) behavior disorders are the most frequently experienced parasomnias in patients with PD. Non-REM sleep disorders like confusional arousals, sleep terrors, sleepwalking, and sleep-related eating disorder (SRED) are also associated with PD. Parasomnias can affect the quality of life of the patients as well as the night time sleep of their bed partners. Hence, it is important for physicians to recognize the occurrence of parasomnias in PD. We report an unusual case of PD with SRED along with obstructive sleep apnea (OSA) and REM behavior disorder. To our knowledge, only two cases have been reported in the literature highlighting the association of SRED with PD. We also explain the different night-time eating disorders like nocturnal eating syndrome and binge eating syndrome, which can be seen in PD, and differentiate them from SRED.

Lennox-Gastaut Syndrome: In a Nutshell.

Lennox-Gastaut syndrome is one of the rare childhood-onset epileptic encephalopathies, characterized by multiple type seizure disorder, the typical pattern on electroencephalogram and intellectual disability. Tonic-type seizures are most commonly seen in these patients. Behavioral disturbances and cognitive decline are gradual-onset and last long after the first episode of epileptiform activity. In most cases, there is some identifiable cause that has led to the clinical presentation of the patient. Various pharmacological and surgical procedures have been proposed for the treatment of Lennox-Gastaut syndrome and many more to come in the very near future to overcome the drug resistance and to avoid the patient forming a life-long dependency.

Acute Autonomic Symptoms with Anti-myelin-associated Glycoprotein Neuropathy as a Presentation of Small B Cell Lymphoma: A Case Report and Literature Review.

The association of symmetrical distal sensorimotor polyneuropathy with anti-myelin-associated glycoprotein antibodies (MAG) has been well established. Although autonomic symptoms are uncommon with anti-MAG antibody neuropathy (MAN). We are presenting an unusual case, who developed acute onset urinary retention, orthostatic hypotension, bradycardia and was found to have MAN. She was a 68-year-old lady, presented with progressive ascending numbness, weakness of her extremities and balance problems. On neurological examination, she had generalized muscle weakness, reduced perception to all modalities of sensation with marked impairment of vibration and position sense in the lower limbs. Reflexes were diminished throughout and Romberg sign was positive. Initial lab work including thyroid-stimulating hormone (TSH), vitamin B12, Hb1c, and routine lab reports was normal. The patient then developed acute urinary retention, severe orthostatic hypotension, and symptomatic bradycardia. Workup during this time revealed M spike on serum electrophoresis with IgM kappa on immunofixation. IgM titers were higher than normal. Initially, she was thought to have monoclonal gammopathy of undetermined significance (MGUS) related neuropathy but further workup showed very high levels of anti-MAG antibody titer. Further workup including a bone marrow biopsy revealed a small B cell lymphoma. Only a few cases have reported a small B cell lymphoma presenting with MAN-associated autonomic symptoms. She is currently being treated with rituximab with significant improvement in her neuropathic symptoms. Further case studies are needed to show whether autonomic symptoms are the feature of MAN or this atypical presentation is the paraneoplastic manifestation of the lymphoma.