A case of immune reconstitution syndrome complicating progressive multifocal leukoencephalopathy after kidney transplant: Clinical, pathological, and radiographic features.

Progressive multifocal leukoencephalopathy (PML) is a life-threatening central nervous system (CNS) disorder, most commonly described in patients infected with the human immunodeficiency virus (HIV). Limited data exist on its natural history and treatment in solid organ transplant (SOT) recipients. A complication of PML is the immune reconstitution inflammatory syndrome (IRIS), which develops after T cell reconstitution and can have severe consequences when it occurs in the CNS. While well described in HIV-infected individuals, its clinical features, diagnosis, and treatment after SOT are largely unknown. We report a case of a kidney transplant recipient who was diagnosed with PML and developed significant worsening of her symptoms upon reduction of immunosuppression. Thallium SPECT showed avid uptake suggestive of lymphoma, but the diagnosis of PML-IRIS was ultimately established by brain biopsy. She survived with nearly complete restoration of her functional status after a prolonged steroid taper.

Electrodiagnostic studies in the intensive care unit: A comparison study 2 decades later.

INTRODUCTION: Since the late 1980s, critical illness myopathy (CIM) and critical illness polyneuropathy (CIP) have been increasingly recognized in the intensive care unit (ICU). We explored whether these causes of ICU weakness were now more likely to lead to electrodiagnostic studies (EDX) at our institution than they were 19-20 years earlier. METHODS: We reviewed 100 consecutive ICU patients who underwent EDX from 2009 to 2015 and compared them to a previously reported study population from 1990-1995. RESULTS: Thirty-seven (39%) had CIM, CIP, or both versus 55% in the previous study (P = 0.04). Thirty-four (36%) were diagnosed with "traditional" pre-ICU causes of weakness, such as motor neuron disease or Guillain-Barre syndrome, versus 29% in the earlier study (P = 0.3). DISCUSSION: CIM and CIP continue to be common disorders that lead to ICU EDX, but their proportion declined compared with 19-20 years earlier, possibly due to the perceived role and selective use of EDX in the ICU. Muscle Nerve 57: 772-776, 2018.

Comorbidities in pediatric patients with postural orthostatic tachycardia syndrome.

OBJECTIVE: To investigate the frequency of various nonorthostatic complaints in children with postural orthostatic tachycardia syndrome (POTS). STUDY DESIGN: We used our autonomic laboratory database to identify all pediatric patients with traditionally defined POTS who had completed the Ohio Dysautonomia Survey (ODYSA). The responses of the patients to questions targeting various autonomic complaints, including syncope, gastrointestinal symptoms, sleep disturbances, headaches, urinary symptoms, chronic pain, and Raynaud-like symptoms, were collected and analyzed. RESULTS: A total of 53 pediatric patients completed the ODYSA questionnaire. The participants reported high frequencies of sleep abnormalities, chronic pain, Raynaud-like symptoms, and gastrointestinal abnormalities, with lower rates of headaches, syncope, and urinary complaints. CONCLUSIONS: Our findings demonstrate the occurrence of various nonorthostatic symptoms in pediatric patients with POTS. These findings suggest the need for an interdisciplinary approach to the treatment and management of POTS, as well as for further investigation into the mechanisms that lead to the concurrent presentation of both orthostatic and nonorthostatic symptoms in these patients.

Third time's a charm: diagnosis of herpes simplex encephalitis after two negative polymerase chain reaction results.

INTRODUCTION: Polymerase chain reaction (PCR) testing of cerebrospinal fluid (CSF) is a sensitive and specific method in diagnosing herpes simplex virus (HSV) encephalitis. However there are increasing reports of false negative HSV PCR. CASE DESCRIPTION: We present a patient in the 9th decade of life with abnormal behavior and focal seizures with MRI showing a right temporal T2 hyperintense non-enhancing lesion with electrographic evidence of right lateralized periodic discharges. CSF analysis and PCR for HSV-1 and 2 yielded negative results twice, and therefore acyclovir was discontinued. Patient initially improved following correction of hyponatremia. Patient however deteriorated and imaging revealed a new right parietal lesion. Third CSF sample showed lymphocytic pleocytosis with positive HSV-1 PCR. Patient improved following antiviral treatment. DISCUSSION: Acyclovir treatment should continue in high clinical suspicion scenarios despite negative HSV PCR. We further discuss causes of PCR false negatives and challenges it poses for patient care.

Orthostatic syndromes differ in syncope frequency.

BACKGROUND: There are conflicting opinions on whether postural tachycardia syndrome predisposes to syncope. We investigated this relationship by comparing the frequency of syncope in postural tachycardia syndrome and orthostatic hypotension. METHODS: We queried our autonomic laboratory database of 3700 patients. Orthostatic hypotension and postural tachycardia syndrome were defined in standard fashion, except that postural tachycardia syndrome required the presence of orthostatic symptoms and a further increase in heart rate beyond 10 minutes. Syncope was defined as an abrupt decrease in blood pressure and often, heart rate, requiring termination of the tilt study. Statistical analysis utilized Fisher's exact test and Student's t test, as appropriate. RESULTS: Of 810 patients referred for postural tachycardia syndrome, 185 met criteria while another 328 patients had orthostatic hypotension. Of the postural tachycardia syndrome patients, 38% had syncope on head-up tilt, compared with only 22% of those with orthostatic hypotension (P<.0001). In the postural tachycardia group, syncope on head-up tilt was associated with a clinical history of syncope in 90%, whereas absence of syncope on head-up tilt was associated with a clinical history of syncope in 30% (P<.0001). In contrast, syncope on head-up tilt did not bear any relationship to clinical history of syncope in the orthostatic hypotension group (41% vs 36%; P=.49). CONCLUSION: Our results demonstrate that syncope (both tilt table and clinical) occurs far more commonly in patients who have postural tachycardia syndrome than in patients with orthostatic hypotension. These findings suggest that one should be clinically aware of the high risk of syncope in patients with postural tachycardia syndrome, and the low-pressure baroreceptor system that is implicated in postural tachycardia syndrome might confer more sensitivity to syncope than the high pressure system implicated in orthostatic hypotension.