American Journal of Tropical Medicine and Hygiene, Dec 1, 2011
We describe a case of a woman who developed a peripheral polyneuropathy shortly after completing ... more We describe a case of a woman who developed a peripheral polyneuropathy shortly after completing 4 weekly doses of mefloquine hydrochloride (250 mg) malaria prophylaxis. Although mefloquine-related central nervous system neuropathy is well described in the literature, peripheral polyneuropathy similar to this case has been documented only once before, to our knowledge.
Tremor can range from imperceptible physiologic movements to severely handicapping shaking. It ca... more Tremor can range from imperceptible physiologic movements to severely handicapping shaking. It can be a single entity, or it can occur in association with a disease that affects the basal ganglia, brainstem, cerebellum or peripheral nervous system. Tremors can be classified according to their anatomic location, the circumstances under which they occur, their frequency and amplitude, and whether they are physiologic or pathologic. As an isolated disorder, tremor should be treated only when it impairs the patient's ability to perform activities. Successful treatment requires the identification of precipitating factors, as well as associated signs and symptoms.
We report a patient with rheumatoid arthritis (RA) on methotrexate (MTX) who presented with a pol... more We report a patient with rheumatoid arthritis (RA) on methotrexate (MTX) who presented with a polyradiculopathy due to an Epstein-Barr virus (EBV)associated lymphoproliferative disorder. The patient responded favorably to MTX discontinuation and rituximab immunotherapy. Case report. A 56-year-old woman with RA presented with a 4-day history of progressive left upperlimb weakness followed by proximal lower-limb weakness and hands and abdomen paresthesias. Her RA was controlled on 20 years therapy with MTX and hydroxychloroquine, and 4 years prednisone therapy. The examination showed asymmetric weakness that was more proximal than distal and involved the upper limbs significantly more than lower limbs. The reflexes were diminished. Decreased perception to touch and pinprick was noted below the umbilicus and above the inguinal region. Tandem gait was impaired. This progressed during the first 2 days of hospitalization to an inability to walk unaided. Percutaneous midcervical spinal nerve stimulation showed proximal conduction block consistent with a polyradiculopathy. An EMG and nerve conduction study done 9 days later demonstrated an acute, evolving polyradiculopathy with marked recruitment reduction. The relatively limited signs of denervation and their proximal predominance favored proximal denervation as opposed to axonal loss. A brain and spine MRI with contrast were unremarkable. Serum immunofixation showed no monoclonal spike. Bone marrow aspirate and biopsy demonstrated megaloblastoid maturation of myeloid series, compatible with the history of methotrexate therapy and numerous polyclonal lymphoid aggregates. CSF analysis revealed a white cell count of 60/L (normal: Ͻ4 cells/L) with increased lymphocytes. CSF flow cytometric immunophenotyping showed a population of CD 20-positive B-cells that had a monotypic staining pattern for kappa cytoplasmic immunoglobulin light-chains. The CSF protein was elevated (124 mg/dL; normal: 14-45 mg/dL) and
Endometriosis of the nerve often remains an elusive diagnosis. We report the first case of intran... more Endometriosis of the nerve often remains an elusive diagnosis. We report the first case of intraneural lumbosacral plexus endometriosis with sequential imaging at different phases of the menstrual cycle: during the luteal phase and menstruation. Compared to the first examination, the examination performed during the patient's period revealed the lumbosacral plexus larger and hyperintense on T2-weighted imaging. The intraneural endometriosis cyst was also larger and showed recent hemorrhage. Additionally, this case represents another example of perineural spread of endometriosis from the uterus to the lumbosacral plexus along the autonomic nerves and then distally to the sciatic nerve and proximally to the spinal nerves.
Individuals vary widely in their sensitivity to painful stimuli. Some exhibit heightened reaction... more Individuals vary widely in their sensitivity to painful stimuli. Some exhibit heightened reactions to pain (hyperpathia), while others show relative indifference. Although multiple factors may be responsible for these differences, varying sensitivities to pain also can be due to underlying differences in nociceptive neurophysiology. We present here the case of an individual with an apparent congenital inability to perceive pain. This patient appears to be different from other reported cases of insensitivity to pain described in the medical literature. He exhibited no evidence of an abnormality of the peripheral or autonomic nervous system and no apparent abnormality of the central nervous system other than isolated deficits in pain and temperature perception. Since pain is a subjective phenomenon, there is no definitive way to assess this patient's reported inability to perceive painful somatic stimulation, but available evidence suggests he has a defect in the supraspinal processing of nociceptive stimuli which renders him insensitive to pain. This raises the possibility of either deficient central nociceptive functioning or aberrant endogenous anti-nociceptive functioning.
York, NY) is used for erectile dysfunction. Little is known, however, about its effects on the CN... more York, NY) is used for erectile dysfunction. Little is known, however, about its effects on the CNS. A recent case report suggests that sildenafil, through unknown mechanisms, can cause stroke. 1 The authors describe a patient with a history of migraines who had a transient event of global amnesia after taking sildenafil. Patients with migraine may be more susceptible to transient global amnesia (TGA) if exposed to precipitants. Therefore, the authors recommend that sildenafil be used with caution in patients with a history of migraine. Case report. A 51-year-old, right-handed man with hypertension and a history of classic migraines had played 18 holes of golf in the early morning. When he returned home with his girlfriend, he ingested 25 mg of sildenafil. After 30 minutes, as he was about to engage in sexual intercourse, the patient reported that he "felt weird." He began pacing the floor and asked his girlfriend "who bought the shirt I am wearing?" She had purchased the shirt a week earlier. He could not remember that he had played golf that morning. He was immediately taken to a hospital, where he asked repeatedly what had happened to him and was unable to retain answers to his questions. He was treated with IV heparin for a presumed cerebrovascular event. Results of a cranial head CT were normal. Three hours later, he was transferred to our hospital, where he was found to have a blood pressure of 150/70 and a normal cardiovascular examination. He was alert, oriented, and attentive with normal language. He had good registration of information but recalled only one of five items at 1 and 5 minutes. He also could not recall where different denominations of money had been hidden in various locations around the room after he was told what was being concealed and where. He could not remember the events throughout the day and asked repeatedly "what happened?" and "why am I here?" Long-term memory was preserved. His cranial nerves were intact, his strength was full, and sensation was intact to light touch, pin prick, and vibration. His reflexes were ϩ2 and symmetric. Plantar responses were flexor. His gait was narrow based and steady. Heparin was discontinued. MRI and intracranial magnetic resonance angiography of the brain with diffusion-weighted imaging performed on arrival
Journal of Clinical Neuromuscular Disease, Dec 1, 1999
NPJJROPHYSIOLGGlC AND VASCULAR STUDIES IN ERYTHROMELALGIA: A RETRO.SPECTIVE ANALYSIS. ME' Davis. ... more NPJJROPHYSIOLGGlC AND VASCULAR STUDIES IN ERYTHROMELALGIA: A RETRO.SPECTIVE ANALYSIS. ME' Davis. P Sandroni. RmLc. Mayo Clinic and Mayo Foundation. Rochester. MN k.s&: During symptoms. mearuremcn~ in the tots during symptoms revealed a mean temperatures increase of I1.6"C @lo.aaOl) cowxxnitwd with a laser flow increasc from "mean of 6.8 mVmin/100g ds"e to 76.5 mVmin/lWg tissue @<o.WX). while baseline TcPOZ at the feet during sympmmr droppedby 6.65 mmHg&O.032). 21/54(39%)afWIGnewdingswensbnorrnal:alloflhcmfulfilled Ulccritcriaforluonalneumpathy. 37~27(63%)ofARS and I PASP showedseve~ponganglioniisudomomr impairnat, 507 (29%) additionally bad pcriphapl adnncrgic dysfunction. ZIi patients with AR.5 underwent vascular smdica wing skin tempcram~ and tcmpxawe increase after cxenion (pttmgnmnonic for erytbmmelalgia): 190.5 (16%) bad concordant result for the 2 twts No difference in clinical profile separated patients with normal fmm those with abnormal test results. No clinical parameter differentiated pa&no $at underwent vascular, ncurophysiologtc antior autonomic reflex screen from those who did not. &odwon: Our data suggest that during symptoms. an increase in flow and tempenturc is accompanied padoxically by a decrease in oxygenation of rhe affected area; a high proportion of prienu have a dirral small t&r neumparhy with selective involvement of cutmeow sympathetic fibers; in addition, targe fiber neuropalhy Is often prrwnt. Future Studio will attempt D better delineate and explain these vaxdar. neumphyriologic and sutonomtc abnormalitia
tachycardia syndrome (POTS) is characterized by excessive tachycardia without hypotension during ... more tachycardia syndrome (POTS) is characterized by excessive tachycardia without hypotension during orthostasis. Most POTS patients also report exercise intolerance. To assess cardiovascular regulation during exercise in POTS, patients (n ϭ 13) and healthy controls (n ϭ 10) performed graded cycle exercise at 25, 50, and 75 W in both supine and upright positions while arterial pressure (arterial catheter), heart rate (HR; measured by ECG), and cardiac output (open-circuit acetylene breathing) were measured. In both positions, mean arterial pressure, cardiac output, and total peripheral resistance at rest and during exercise were similar in patients and controls (P Ͼ 0.05). However, supine stroke volume (SV) tended to be lower in the patients than controls at rest (99 Ϯ 5 vs. 110 Ϯ 9 ml) and during 75-W exercise (97 Ϯ 5 vs. 111 Ϯ 7 ml) (P ϭ 0.07), and HR was higher in the patients than controls at rest (76 Ϯ 3 vs. 62 Ϯ 4 beats/min) and during 75-W exercise (127 Ϯ 3 vs. 114 Ϯ 5 beats/min) (both P Ͻ 0.01). Upright SV was significantly lower in the patients than controls at rest (57 Ϯ 3 vs. 81 Ϯ 6 ml) and during 75-W exercise (70 Ϯ 4 vs. 94 Ϯ 6 ml) (both P Ͻ 0.01), and HR was much higher in the patients than controls at rest (103 Ϯ 3 vs. 81 Ϯ 4 beats/min) and during 75-W exercise (164 Ϯ 3 vs. 131 Ϯ 7 beats/min) (both P Ͻ 0.001). The change (upright Ϫ supine) in SV was inversely correlated with the change in HR for all participants at rest (R 2 ϭ 0.32), at 25 W (R 2 ϭ 0.49), 50 W (R 2 ϭ 0.60), and 75 W (R 2 ϭ 0.32) (P Ͻ 0.01). These results suggest that greater elevation in HR in POTS patients during exercise, especially while upright, was secondary to reduced SV and associated with exercise intolerance.
Journal of The Peripheral Nervous System, Jun 1, 2004
Case Report: A 20-year-old man presented for evaluation following an episode of painless injury i... more Case Report: A 20-year-old man presented for evaluation following an episode of painless injury in which he almost amputated his thumb. Upon further questioning, the patient reported he rarely experienced physical (including visceral) pain and his mother provided support to his history recalling incidents occurring as a child. He does not experience itching either. His neurologic and general medical history was otherwise negative. His neurologic and general examination were unremarkable except for the inability to perceive painful (pinprick, pressure on Achilles' tendons), hot and cold stimuli over his entire body, with some relative sparing over his chest, abdomen and back. Nerve conduction studies and needle examinations were normal. Quantitative sensory testing revealed normal threshold to vibration, relatively elevated threshold to cold and insensitivity to heat and pain. Autonomic reflex screen and thermoregulatory sweat test were normal. Sural nerve biopsy showed normal myelinated and unmyelinated fibers. MRI of head and cervical spine, and extensive laboratory testing were unremarkable. Conclusion: This patient differs from any other case described in the literature of pain insensitivity or pain indifference. Suspecting a central pathology, we prescribed naltrexone and retested his heat-pain sensation after treatment. He perceived the probe as hotter. Unfortunately, he suffered unacceptable dysthymia from the medication and stopped it.
To report the clinical, laboratory, and radiographic features and the response to plasmapheresis ... more To report the clinical, laboratory, and radiographic features and the response to plasmapheresis in a patient with encephalopathy, opsoclonus, and myoclonus whose cerebrospinal fluid was positive for Nmethyl-D-aspartate receptor-IgG.
Pain is a subjective experience in which the patient’s emotional state has a major role, contribu... more Pain is a subjective experience in which the patient’s emotional state has a major role, contributing to the challenge pain clinicians have in quantifying and objectively evaluating this common complaint. The multiplicity and complexity of the neural mechanisms that produce chronic pain make the clinician’s task even more challenging. During the last 20 years, research in the assessment of the nociceptive and autonomic systems has provided useful tools for the diagnosis, treatment, and scientific investigation of neuropathic pain. QSTs provide an accurate, reproducible assessment of the sensory response to well-delineated controlled stimuli for evaluating the function of small myelinated and unmyelinated fibers. These tests can be useful for documenting sensory disturbances that may occur in patients with neuropathy who experience hypalgesia, hyperalgesia, or allodynia. The inclusion of autonomic tests in the evaluation of some patients with neuropathic pain may provide objective evidence of increased sympathetic tone, heightened somatosympathetic reflexes, or sympathetic denervation. This information may help the clinician to diagnose more accurately the particular type of pain syndrome the patient has so that the treatment may be better directed at the underlying mechanism. MCNG, primarily a research tool, is a powerful method for directly studying primary afferent and sympathetic efferent neural activity in patients with pain caused by lesions of the nervous system. LEPs allow clinicians and researchers to investigate objectively function of the peripheral and central nociceptive pathways in patients with neuropathic pain.
Introduction: Paraneoplastic lower motor neuronopathies have been reported rarely with Hodgkin ly... more Introduction: Paraneoplastic lower motor neuronopathies have been reported rarely with Hodgkin lymphoma. Methods: We report a case of rapidly progressive motor neuronopathy preceding the diagnosis of Hodgkin lymphoma. Results: A 31‐year‐old woman developed subacute rapidly progressive quadriparesis. Electrodiagnostic studies revealed a severe diffuse disorder of motor neurons and their axons. Symmetric enhancement of the cauda equina motor nerve roots was notable on magnetic resonance imaging scan. Further imaging demonstrated an enlarged supraclavicular lymph node, and biopsy revealed Hodgkin lymphoma. A final diagnosis of paraneoplastic motor neuronopathy was made after investigations for alternative causes of motor neuronopathy were unrevealing. Neurological improvement was seen with combined treatment of the underlying malignancy and intravenous immunoglobulin.Conclusions: Paraneoplastic causes should be considered in the differential diagnosis of subacute motor neuronopathy, as the neurological presentation may precede cancer detection. Combinations of lymphoma treatment and immunotherapy may result in a favorable outcome. Muscle Nerve 46: 823–827, 2012
Objective Multiple system atrophy (MSA) is characterized by urinary dysfunction, yet the influenc... more Objective Multiple system atrophy (MSA) is characterized by urinary dysfunction, yet the influence of sex and gender on urinary symptoms and treatment is unclear. We sought to characterize sex and gender differences in the symptomatology, evaluation, and management of urinary dysfunction in patients with MSA.Methods Patients with MSA evaluated at our institution were reviewed and stratified by sex.Results While the prevalence of urinary symptoms was similar in male and female patients, incontinence was more common in females. Despite this, males and females underwent postvoid residual (PVR) measurement at similar rates. While catheterization rates were similar when PVR was measured, males were more than twice as likely to be catheterized than females in the absence of PVR measurement.Conclusion Urinary symptoms are common in MSA, but their presentation differs between males and females. The difference in catheterization rates may be driven by a gender disparity in referrals for PVR,...
Objective To describe the natural history of afferent baroreflex failure (ABF) based on systemati... more Objective To describe the natural history of afferent baroreflex failure (ABF) based on systematic review of clinical and laboratory data in patients with a diagnosis of ABF at Mayo Clinic Rochester. Methods We performed a retrospective chart review of all patients who underwent standardized autonomic reflex testing between 2000 and 2020 and had confirmation of the diagnosis of ABF by an autonomic disorders specialist. Patients were identified using a data repository of medical records. Variables included demographic, all-cause mortality, medications, ABF manifestations, comorbidities, and laboratory (autonomic testing, blood pressure monitoring, echocardiogram, brain imaging, plasma catecholamines, serum sodium level, and kidney function tests). Results A total of 104 patients with ABF were identified. Head and neck radiation was the most common etiology (86.5%), followed by neck surgery (5.8%) and other causes (7.7%). The most common findings were hypertension (87.5%), fluctuating blood pressure (78.8%), orthostatic hypotension (91.3%), syncope (58.6%), headache (22.1%), and tachycardia (20.2%). Patients commonly received antihypertensives (66.3%), pressor agents (41.3%), or a combination of both (19.2%). The median latency from completion of radiation to ABF was longer compared to the latency in the surgery group (p < 0.0001). Comorbidities, including complications from neck radiation, were frequently seen and all-cause mortality was 39.4% over a 20-year period. Conclusions ABF should be suspected in patients with prior head and neck cancer treated by radiation or surgery who present with labile hypertension and orthostatic hypotension. Management may require both antihypertensive and pressor medications. The morbidity and mortality in ABF are high.
Background-Chronic symptoms of orthostatic intolerance occur in postural tachycardia syndrome (PO... more Background-Chronic symptoms of orthostatic intolerance occur in postural tachycardia syndrome (POTS) and patients with orthostatic intolerance (OI) without tachycardia. We recently reported that deconditioning is almost universal in both patient groups. In this study, we focussed on the question of how much dysautonomia, besides orthostatic tachycardia, is there in POTS vs. OI, and how the two groups compare in regards to clinical, autonomic, laboratory, and exercise variables. Methods-We retrospectively studied all patients referred for orthostatic intolerance at Mayo Clinic between January 2006 and June 2011, who underwent standardized autonomic and exercise testing. Results-Eighty-four POTS and 100 OI fulfilled inclusion criteria, 89 % were females. The mean age was 25 and 32 years, respectively. Clinical presentation, autonomic parameters, laboratory findings, and degree of deconditioning were overall similar between the two groups, except for the excessive orthostatic heart rate (HR) rise and mild vasomotor findings observed in POTS but not in OI (slightly larger Valsalva ratio and incomplete blood pressure recovery during Valsalva). Both groups responded poorly to various medications. Severely deconditioned patients were similar to non-deconditioned patients, except for 24 h urine volume (1,555 vs. 2,417 ml), sweat loss on thermoregulatory sweat test (1.5 vs. 0.5 %), and few respiratory parameters during exercise, which are likely clinically insignificant. Conclusion-Though similar in clinical presentation, POTS and OI are different entities with greater, albeit still mild, dysautonomia in POTS. The clinical and pathophysiological relevance of minimal dysautonomia in the absence of orthostatic tachycardia as seen in OI remain uncertain.
American Journal of Tropical Medicine and Hygiene, Dec 1, 2011
We describe a case of a woman who developed a peripheral polyneuropathy shortly after completing ... more We describe a case of a woman who developed a peripheral polyneuropathy shortly after completing 4 weekly doses of mefloquine hydrochloride (250 mg) malaria prophylaxis. Although mefloquine-related central nervous system neuropathy is well described in the literature, peripheral polyneuropathy similar to this case has been documented only once before, to our knowledge.
Tremor can range from imperceptible physiologic movements to severely handicapping shaking. It ca... more Tremor can range from imperceptible physiologic movements to severely handicapping shaking. It can be a single entity, or it can occur in association with a disease that affects the basal ganglia, brainstem, cerebellum or peripheral nervous system. Tremors can be classified according to their anatomic location, the circumstances under which they occur, their frequency and amplitude, and whether they are physiologic or pathologic. As an isolated disorder, tremor should be treated only when it impairs the patient's ability to perform activities. Successful treatment requires the identification of precipitating factors, as well as associated signs and symptoms.
We report a patient with rheumatoid arthritis (RA) on methotrexate (MTX) who presented with a pol... more We report a patient with rheumatoid arthritis (RA) on methotrexate (MTX) who presented with a polyradiculopathy due to an Epstein-Barr virus (EBV)associated lymphoproliferative disorder. The patient responded favorably to MTX discontinuation and rituximab immunotherapy. Case report. A 56-year-old woman with RA presented with a 4-day history of progressive left upperlimb weakness followed by proximal lower-limb weakness and hands and abdomen paresthesias. Her RA was controlled on 20 years therapy with MTX and hydroxychloroquine, and 4 years prednisone therapy. The examination showed asymmetric weakness that was more proximal than distal and involved the upper limbs significantly more than lower limbs. The reflexes were diminished. Decreased perception to touch and pinprick was noted below the umbilicus and above the inguinal region. Tandem gait was impaired. This progressed during the first 2 days of hospitalization to an inability to walk unaided. Percutaneous midcervical spinal nerve stimulation showed proximal conduction block consistent with a polyradiculopathy. An EMG and nerve conduction study done 9 days later demonstrated an acute, evolving polyradiculopathy with marked recruitment reduction. The relatively limited signs of denervation and their proximal predominance favored proximal denervation as opposed to axonal loss. A brain and spine MRI with contrast were unremarkable. Serum immunofixation showed no monoclonal spike. Bone marrow aspirate and biopsy demonstrated megaloblastoid maturation of myeloid series, compatible with the history of methotrexate therapy and numerous polyclonal lymphoid aggregates. CSF analysis revealed a white cell count of 60/L (normal: Ͻ4 cells/L) with increased lymphocytes. CSF flow cytometric immunophenotyping showed a population of CD 20-positive B-cells that had a monotypic staining pattern for kappa cytoplasmic immunoglobulin light-chains. The CSF protein was elevated (124 mg/dL; normal: 14-45 mg/dL) and
Endometriosis of the nerve often remains an elusive diagnosis. We report the first case of intran... more Endometriosis of the nerve often remains an elusive diagnosis. We report the first case of intraneural lumbosacral plexus endometriosis with sequential imaging at different phases of the menstrual cycle: during the luteal phase and menstruation. Compared to the first examination, the examination performed during the patient&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s period revealed the lumbosacral plexus larger and hyperintense on T2-weighted imaging. The intraneural endometriosis cyst was also larger and showed recent hemorrhage. Additionally, this case represents another example of perineural spread of endometriosis from the uterus to the lumbosacral plexus along the autonomic nerves and then distally to the sciatic nerve and proximally to the spinal nerves.
Individuals vary widely in their sensitivity to painful stimuli. Some exhibit heightened reaction... more Individuals vary widely in their sensitivity to painful stimuli. Some exhibit heightened reactions to pain (hyperpathia), while others show relative indifference. Although multiple factors may be responsible for these differences, varying sensitivities to pain also can be due to underlying differences in nociceptive neurophysiology. We present here the case of an individual with an apparent congenital inability to perceive pain. This patient appears to be different from other reported cases of insensitivity to pain described in the medical literature. He exhibited no evidence of an abnormality of the peripheral or autonomic nervous system and no apparent abnormality of the central nervous system other than isolated deficits in pain and temperature perception. Since pain is a subjective phenomenon, there is no definitive way to assess this patient&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s reported inability to perceive painful somatic stimulation, but available evidence suggests he has a defect in the supraspinal processing of nociceptive stimuli which renders him insensitive to pain. This raises the possibility of either deficient central nociceptive functioning or aberrant endogenous anti-nociceptive functioning.
York, NY) is used for erectile dysfunction. Little is known, however, about its effects on the CN... more York, NY) is used for erectile dysfunction. Little is known, however, about its effects on the CNS. A recent case report suggests that sildenafil, through unknown mechanisms, can cause stroke. 1 The authors describe a patient with a history of migraines who had a transient event of global amnesia after taking sildenafil. Patients with migraine may be more susceptible to transient global amnesia (TGA) if exposed to precipitants. Therefore, the authors recommend that sildenafil be used with caution in patients with a history of migraine. Case report. A 51-year-old, right-handed man with hypertension and a history of classic migraines had played 18 holes of golf in the early morning. When he returned home with his girlfriend, he ingested 25 mg of sildenafil. After 30 minutes, as he was about to engage in sexual intercourse, the patient reported that he "felt weird." He began pacing the floor and asked his girlfriend "who bought the shirt I am wearing?" She had purchased the shirt a week earlier. He could not remember that he had played golf that morning. He was immediately taken to a hospital, where he asked repeatedly what had happened to him and was unable to retain answers to his questions. He was treated with IV heparin for a presumed cerebrovascular event. Results of a cranial head CT were normal. Three hours later, he was transferred to our hospital, where he was found to have a blood pressure of 150/70 and a normal cardiovascular examination. He was alert, oriented, and attentive with normal language. He had good registration of information but recalled only one of five items at 1 and 5 minutes. He also could not recall where different denominations of money had been hidden in various locations around the room after he was told what was being concealed and where. He could not remember the events throughout the day and asked repeatedly "what happened?" and "why am I here?" Long-term memory was preserved. His cranial nerves were intact, his strength was full, and sensation was intact to light touch, pin prick, and vibration. His reflexes were ϩ2 and symmetric. Plantar responses were flexor. His gait was narrow based and steady. Heparin was discontinued. MRI and intracranial magnetic resonance angiography of the brain with diffusion-weighted imaging performed on arrival
Journal of Clinical Neuromuscular Disease, Dec 1, 1999
NPJJROPHYSIOLGGlC AND VASCULAR STUDIES IN ERYTHROMELALGIA: A RETRO.SPECTIVE ANALYSIS. ME' Davis. ... more NPJJROPHYSIOLGGlC AND VASCULAR STUDIES IN ERYTHROMELALGIA: A RETRO.SPECTIVE ANALYSIS. ME' Davis. P Sandroni. RmLc. Mayo Clinic and Mayo Foundation. Rochester. MN k.s&: During symptoms. mearuremcn~ in the tots during symptoms revealed a mean temperatures increase of I1.6"C @lo.aaOl) cowxxnitwd with a laser flow increasc from "mean of 6.8 mVmin/100g ds"e to 76.5 mVmin/lWg tissue @<o.WX). while baseline TcPOZ at the feet during sympmmr droppedby 6.65 mmHg&O.032). 21/54(39%)afWIGnewdingswensbnorrnal:alloflhcmfulfilled Ulccritcriaforluonalneumpathy. 37~27(63%)ofARS and I PASP showedseve~ponganglioniisudomomr impairnat, 507 (29%) additionally bad pcriphapl adnncrgic dysfunction. ZIi patients with AR.5 underwent vascular smdica wing skin tempcram~ and tcmpxawe increase after cxenion (pttmgnmnonic for erytbmmelalgia): 190.5 (16%) bad concordant result for the 2 twts No difference in clinical profile separated patients with normal fmm those with abnormal test results. No clinical parameter differentiated pa&no $at underwent vascular, ncurophysiologtc antior autonomic reflex screen from those who did not. &odwon: Our data suggest that during symptoms. an increase in flow and tempenturc is accompanied padoxically by a decrease in oxygenation of rhe affected area; a high proportion of prienu have a dirral small t&r neumparhy with selective involvement of cutmeow sympathetic fibers; in addition, targe fiber neuropalhy Is often prrwnt. Future Studio will attempt D better delineate and explain these vaxdar. neumphyriologic and sutonomtc abnormalitia
tachycardia syndrome (POTS) is characterized by excessive tachycardia without hypotension during ... more tachycardia syndrome (POTS) is characterized by excessive tachycardia without hypotension during orthostasis. Most POTS patients also report exercise intolerance. To assess cardiovascular regulation during exercise in POTS, patients (n ϭ 13) and healthy controls (n ϭ 10) performed graded cycle exercise at 25, 50, and 75 W in both supine and upright positions while arterial pressure (arterial catheter), heart rate (HR; measured by ECG), and cardiac output (open-circuit acetylene breathing) were measured. In both positions, mean arterial pressure, cardiac output, and total peripheral resistance at rest and during exercise were similar in patients and controls (P Ͼ 0.05). However, supine stroke volume (SV) tended to be lower in the patients than controls at rest (99 Ϯ 5 vs. 110 Ϯ 9 ml) and during 75-W exercise (97 Ϯ 5 vs. 111 Ϯ 7 ml) (P ϭ 0.07), and HR was higher in the patients than controls at rest (76 Ϯ 3 vs. 62 Ϯ 4 beats/min) and during 75-W exercise (127 Ϯ 3 vs. 114 Ϯ 5 beats/min) (both P Ͻ 0.01). Upright SV was significantly lower in the patients than controls at rest (57 Ϯ 3 vs. 81 Ϯ 6 ml) and during 75-W exercise (70 Ϯ 4 vs. 94 Ϯ 6 ml) (both P Ͻ 0.01), and HR was much higher in the patients than controls at rest (103 Ϯ 3 vs. 81 Ϯ 4 beats/min) and during 75-W exercise (164 Ϯ 3 vs. 131 Ϯ 7 beats/min) (both P Ͻ 0.001). The change (upright Ϫ supine) in SV was inversely correlated with the change in HR for all participants at rest (R 2 ϭ 0.32), at 25 W (R 2 ϭ 0.49), 50 W (R 2 ϭ 0.60), and 75 W (R 2 ϭ 0.32) (P Ͻ 0.01). These results suggest that greater elevation in HR in POTS patients during exercise, especially while upright, was secondary to reduced SV and associated with exercise intolerance.
Journal of The Peripheral Nervous System, Jun 1, 2004
Case Report: A 20-year-old man presented for evaluation following an episode of painless injury i... more Case Report: A 20-year-old man presented for evaluation following an episode of painless injury in which he almost amputated his thumb. Upon further questioning, the patient reported he rarely experienced physical (including visceral) pain and his mother provided support to his history recalling incidents occurring as a child. He does not experience itching either. His neurologic and general medical history was otherwise negative. His neurologic and general examination were unremarkable except for the inability to perceive painful (pinprick, pressure on Achilles' tendons), hot and cold stimuli over his entire body, with some relative sparing over his chest, abdomen and back. Nerve conduction studies and needle examinations were normal. Quantitative sensory testing revealed normal threshold to vibration, relatively elevated threshold to cold and insensitivity to heat and pain. Autonomic reflex screen and thermoregulatory sweat test were normal. Sural nerve biopsy showed normal myelinated and unmyelinated fibers. MRI of head and cervical spine, and extensive laboratory testing were unremarkable. Conclusion: This patient differs from any other case described in the literature of pain insensitivity or pain indifference. Suspecting a central pathology, we prescribed naltrexone and retested his heat-pain sensation after treatment. He perceived the probe as hotter. Unfortunately, he suffered unacceptable dysthymia from the medication and stopped it.
To report the clinical, laboratory, and radiographic features and the response to plasmapheresis ... more To report the clinical, laboratory, and radiographic features and the response to plasmapheresis in a patient with encephalopathy, opsoclonus, and myoclonus whose cerebrospinal fluid was positive for Nmethyl-D-aspartate receptor-IgG.
Pain is a subjective experience in which the patient’s emotional state has a major role, contribu... more Pain is a subjective experience in which the patient’s emotional state has a major role, contributing to the challenge pain clinicians have in quantifying and objectively evaluating this common complaint. The multiplicity and complexity of the neural mechanisms that produce chronic pain make the clinician’s task even more challenging. During the last 20 years, research in the assessment of the nociceptive and autonomic systems has provided useful tools for the diagnosis, treatment, and scientific investigation of neuropathic pain. QSTs provide an accurate, reproducible assessment of the sensory response to well-delineated controlled stimuli for evaluating the function of small myelinated and unmyelinated fibers. These tests can be useful for documenting sensory disturbances that may occur in patients with neuropathy who experience hypalgesia, hyperalgesia, or allodynia. The inclusion of autonomic tests in the evaluation of some patients with neuropathic pain may provide objective evidence of increased sympathetic tone, heightened somatosympathetic reflexes, or sympathetic denervation. This information may help the clinician to diagnose more accurately the particular type of pain syndrome the patient has so that the treatment may be better directed at the underlying mechanism. MCNG, primarily a research tool, is a powerful method for directly studying primary afferent and sympathetic efferent neural activity in patients with pain caused by lesions of the nervous system. LEPs allow clinicians and researchers to investigate objectively function of the peripheral and central nociceptive pathways in patients with neuropathic pain.
Introduction: Paraneoplastic lower motor neuronopathies have been reported rarely with Hodgkin ly... more Introduction: Paraneoplastic lower motor neuronopathies have been reported rarely with Hodgkin lymphoma. Methods: We report a case of rapidly progressive motor neuronopathy preceding the diagnosis of Hodgkin lymphoma. Results: A 31‐year‐old woman developed subacute rapidly progressive quadriparesis. Electrodiagnostic studies revealed a severe diffuse disorder of motor neurons and their axons. Symmetric enhancement of the cauda equina motor nerve roots was notable on magnetic resonance imaging scan. Further imaging demonstrated an enlarged supraclavicular lymph node, and biopsy revealed Hodgkin lymphoma. A final diagnosis of paraneoplastic motor neuronopathy was made after investigations for alternative causes of motor neuronopathy were unrevealing. Neurological improvement was seen with combined treatment of the underlying malignancy and intravenous immunoglobulin.Conclusions: Paraneoplastic causes should be considered in the differential diagnosis of subacute motor neuronopathy, as the neurological presentation may precede cancer detection. Combinations of lymphoma treatment and immunotherapy may result in a favorable outcome. Muscle Nerve 46: 823–827, 2012
Objective Multiple system atrophy (MSA) is characterized by urinary dysfunction, yet the influenc... more Objective Multiple system atrophy (MSA) is characterized by urinary dysfunction, yet the influence of sex and gender on urinary symptoms and treatment is unclear. We sought to characterize sex and gender differences in the symptomatology, evaluation, and management of urinary dysfunction in patients with MSA.Methods Patients with MSA evaluated at our institution were reviewed and stratified by sex.Results While the prevalence of urinary symptoms was similar in male and female patients, incontinence was more common in females. Despite this, males and females underwent postvoid residual (PVR) measurement at similar rates. While catheterization rates were similar when PVR was measured, males were more than twice as likely to be catheterized than females in the absence of PVR measurement.Conclusion Urinary symptoms are common in MSA, but their presentation differs between males and females. The difference in catheterization rates may be driven by a gender disparity in referrals for PVR,...
Objective To describe the natural history of afferent baroreflex failure (ABF) based on systemati... more Objective To describe the natural history of afferent baroreflex failure (ABF) based on systematic review of clinical and laboratory data in patients with a diagnosis of ABF at Mayo Clinic Rochester. Methods We performed a retrospective chart review of all patients who underwent standardized autonomic reflex testing between 2000 and 2020 and had confirmation of the diagnosis of ABF by an autonomic disorders specialist. Patients were identified using a data repository of medical records. Variables included demographic, all-cause mortality, medications, ABF manifestations, comorbidities, and laboratory (autonomic testing, blood pressure monitoring, echocardiogram, brain imaging, plasma catecholamines, serum sodium level, and kidney function tests). Results A total of 104 patients with ABF were identified. Head and neck radiation was the most common etiology (86.5%), followed by neck surgery (5.8%) and other causes (7.7%). The most common findings were hypertension (87.5%), fluctuating blood pressure (78.8%), orthostatic hypotension (91.3%), syncope (58.6%), headache (22.1%), and tachycardia (20.2%). Patients commonly received antihypertensives (66.3%), pressor agents (41.3%), or a combination of both (19.2%). The median latency from completion of radiation to ABF was longer compared to the latency in the surgery group (p < 0.0001). Comorbidities, including complications from neck radiation, were frequently seen and all-cause mortality was 39.4% over a 20-year period. Conclusions ABF should be suspected in patients with prior head and neck cancer treated by radiation or surgery who present with labile hypertension and orthostatic hypotension. Management may require both antihypertensive and pressor medications. The morbidity and mortality in ABF are high.
Background-Chronic symptoms of orthostatic intolerance occur in postural tachycardia syndrome (PO... more Background-Chronic symptoms of orthostatic intolerance occur in postural tachycardia syndrome (POTS) and patients with orthostatic intolerance (OI) without tachycardia. We recently reported that deconditioning is almost universal in both patient groups. In this study, we focussed on the question of how much dysautonomia, besides orthostatic tachycardia, is there in POTS vs. OI, and how the two groups compare in regards to clinical, autonomic, laboratory, and exercise variables. Methods-We retrospectively studied all patients referred for orthostatic intolerance at Mayo Clinic between January 2006 and June 2011, who underwent standardized autonomic and exercise testing. Results-Eighty-four POTS and 100 OI fulfilled inclusion criteria, 89 % were females. The mean age was 25 and 32 years, respectively. Clinical presentation, autonomic parameters, laboratory findings, and degree of deconditioning were overall similar between the two groups, except for the excessive orthostatic heart rate (HR) rise and mild vasomotor findings observed in POTS but not in OI (slightly larger Valsalva ratio and incomplete blood pressure recovery during Valsalva). Both groups responded poorly to various medications. Severely deconditioned patients were similar to non-deconditioned patients, except for 24 h urine volume (1,555 vs. 2,417 ml), sweat loss on thermoregulatory sweat test (1.5 vs. 0.5 %), and few respiratory parameters during exercise, which are likely clinically insignificant. Conclusion-Though similar in clinical presentation, POTS and OI are different entities with greater, albeit still mild, dysautonomia in POTS. The clinical and pathophysiological relevance of minimal dysautonomia in the absence of orthostatic tachycardia as seen in OI remain uncertain.
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Papers by Paola Sandroni