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Neurological Sciences : Official... Aug 2023A 56-year-old man presented to the clinic with episodic headaches for several years which had been worsening over a few months prior to the presentation. He described...
A 56-year-old man presented to the clinic with episodic headaches for several years which had been worsening over a few months prior to the presentation. He described headache as sharp, stabbing pain around the left eye associated with nausea, vomiting, photophobia, and phonophobia lasting for hours associated with flushing on the left side of the face. The picture of his face during these episodes showed flushing of the left side of the face, ptosis of the right eyelid, and miosis (panel A). Flushing in his face would resolve with the abortion of the headache. At the time of presentation to the clinic, his neurological exam was only significant for mild left eye ptosis and miosis (panels B and C). Extensive workup including MRI brain, cervical spine, thoracic spine, lumbar spine, CTA head and neck, and CT maxillofacial was unremarkable. He had tried several medications in the past including valproic acid, nortriptyline, and verapamil without significant benefit. He was started on erenumab for migraine prophylaxis and was given sumatriptan for abortive therapy following which his headaches improved. The patient was diagnosed with idiopathic left Horner's syndrome and his migraines with autonomic dysfunction would present with unilateral flushing opposite to the site of Horner's presenting as Harlequin syndrome [1, 2].
Topics: Male; Humans; Middle Aged; Autonomic Nervous System Diseases; Horner Syndrome; Miosis; Headache
PubMed: 37199876
DOI: 10.1007/s10072-023-06849-y -
Muscle & Nerve Jan 2021Diabetic neuropathies are the most common type of neuropathies seen in clinical practice. These neuropathies can range clinically from asymptomatic to manifesting... (Review)
Review
Diabetic neuropathies are the most common type of neuropathies seen in clinical practice. These neuropathies can range clinically from asymptomatic to manifesting symptoms caused by motor, sensory, and autonomic nerve dysfunction. These neuropathies can affect the peripheral nervous system, pain receptors, cardiovascular, urogenital, and gastrointestinal systems. This monograph presents an overview of the different types of diabetic neuropathies, their presentations, diagnostic tools, and strategies for management.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Cardiovascular System; Diabetes Mellitus; Diabetic Neuropathies; Humans
PubMed: 32589300
DOI: 10.1002/mus.27014 -
Current Neurology and Neuroscience... Nov 2023Dysautonomia refers to the dysfunction of the autonomic nervous system and encompasses a wide variety of autonomic symptoms and disorders. The most common autonomic... (Review)
Review
PURPOSE OF REVIEW
Dysautonomia refers to the dysfunction of the autonomic nervous system and encompasses a wide variety of autonomic symptoms and disorders. The most common autonomic disorders are postural orthostatic tachycardia syndrome (POTS), neurocardiogenic syncope (NCS), and orthostatic hypotension (OH), which may be encountered in clinical practice as part of a triad of dysautonomia, hypermobility spectrum disorders (HSD), and mast cell activation syndrome (MCAS). Migraine is one of the most common comorbidities of POTS, HSD, and MCAS; conversely, these conditions are also prevalent in patients with migraine, especially in those with multiple systemic symptoms, such as chronic dizziness, lightheadedness, orthostatic intolerance, joint pain, and allergic symptoms. Diagnostic criteria, pathophysiologic mechanisms, and therapeutic considerations in patients with migraine and comorbid dysautonomia, HSD, and MCAS are reviewed.
RECENT FINDINGS
Numerous studies indicate a significant overlap and shared pathophysiology in migraine, dysautonomia, HSD, and MCAS. In clinical setting, dysautonomia, HSD, and MCAS may present a diagnostic and therapeutic challenge in patients with migraine and require a high index of suspicion on the part of the neurologist. Diagnosis and treatment of these complex disorders in patients with migraine is essential to comprehensive patient-centric care, reduced symptom burden, and improved functional impairment secondary to both migraine and comorbidities.
Topics: Humans; Mast Cell Activation Syndrome; Postural Orthostatic Tachycardia Syndrome; Primary Dysautonomias; Comorbidity; Migraine Disorders
PubMed: 37847487
DOI: 10.1007/s11910-023-01307-w -
Lakartidningen Dec 2021Paroxysmal sympathetic hyperactivity (PSH) is a condition mainly described in patients after traumatic brain injury and it is also known under the terms "autonomic...
Paroxysmal sympathetic hyperactivity (PSH) is a condition mainly described in patients after traumatic brain injury and it is also known under the terms "autonomic storm" and "dysautonomia". It affects between 8-10% of patients after traumatic brain injury and can also affect patients after other neurological diseases, such as anoxic brain injury, stroke, tumors or infections. PSH manifests with six main symptoms: tachycardia, tachypnea, hypertension, hyperthermia, hyperhidrosis and increased muscle tonus. It is of outmost importance to exclude other causes for the symptoms and there are diagnostic criteria established to identify and diagnose PSH. The treatment is pharmacological and non-pharmacological and often multimodal. PSH is probably underdiagnosed and increased awareness is needed.
Topics: Autonomic Nervous System Diseases; Brain Injuries; Brain Injuries, Traumatic; Humans; Hypertension; Tachycardia
PubMed: 34914088
DOI: No ID Found -
Journal of the European Academy of... Sep 2021
Topics: Autonomic Nervous System Diseases; Flushing; Humans; Hypohidrosis
PubMed: 33974302
DOI: 10.1111/jdv.17337 -
Continuum (Minneapolis, Minn.) Feb 2020This article reviews disorders of sweating, including hyperhidrosis and anhidrosis due to central or peripheral autonomic nervous system causes. (Review)
Review
PURPOSE OF REVIEW
This article reviews disorders of sweating, including hyperhidrosis and anhidrosis due to central or peripheral autonomic nervous system causes.
RECENT FINDINGS
Disorders of thermoregulation and sweating may manifest with hyperhidrosis or hypohidrosis/anhidrosis. Primary disorders of hyperhidrosis may significantly impact quality of life yet tend to be benign. Many sweating disorders present with compensatory hyperhidrosis due to areas of anhidrosis. Anhidrosis may occur due to either central or peripheral damage to the autonomic nervous system. The thermoregulatory control of sweating involves central pathways from the hypothalamus to the brainstem and then spinal cord as well as projections to peripheral structures, including the sympathetic chain ganglia, peripheral nerves, and eccrine sweat glands. Disruption at any point of this pathway may lead to impaired sweating. Characterization of sweating dysfunction helps localize different autonomic disorders to guide diagnosis and may allow for evaluation of treatment effect.
SUMMARY
Sweating dysfunction manifests in myriad ways, including essential hyperhidrosis, complete anhidrosis with heat intolerance, and compensatory hyperhidrosis due to anhidrosis, and often indicates involvement of underlying central or peripheral autonomic dysfunction.
Topics: Aged; Autonomic Nervous System Diseases; Body Temperature Regulation; Female; Humans; Hyperhidrosis; Hypohidrosis; Male; Middle Aged; Sweating; Young Adult
PubMed: 31996625
DOI: 10.1212/CON.0000000000000813 -
Headache Oct 2019Dysautonomia and headache are 2 common diagnoses within pediatric neurology; in the case of dysautonomia, a lack of consideration may lead to misdiagnosis. Despite being... (Review)
Review
Dysautonomia and headache are 2 common diagnoses within pediatric neurology; in the case of dysautonomia, a lack of consideration may lead to misdiagnosis. Despite being common conditions, there is a lot to learn about each individually as well as collectively. Many of the symptoms between headache and dysautonomia patients overlap making the diagnosis difficult. Migraine patients often exhibit symptoms of dysautonomia, namely postural orthostatic tachycardia syndrome (POTS); yet these symptoms are overlooked or lumped in as a part of their migraine diagnosis. The distinction or coexistence between dysautonomia and headache is identified through a thorough history, a full exam, and an open mind. This is crucial for the treatment and outcomes of these patients. Struggles arise when critical treatment differences are overlooked because dysautonomia is not considered. In this review, we will look at the epidemiology of dysautonomia and headache with focus on POTS and migraine. We will then compare the clinical features of both conditions as well as some hypothesized pathophysiology overlaps. We will conclude by summarizing the diagnostic approach and multitiered treatment options for POTS and migraine.
Topics: Adolescent; Age of Onset; Analgesics; Child; Child, Preschool; Comorbidity; Female; Headache; Humans; Infant; Male; Migraine Disorders; Neurologic Examination; Neurotransmitter Agents; Neurovascular Coupling; Postural Orthostatic Tachycardia Syndrome; Prevalence; Primary Dysautonomias; Symptom Assessment; Vasodilation
PubMed: 31549738
DOI: 10.1111/head.13659 -
Seminars in Neurology Oct 2020Autonomic dysfunction is a characteristic feature in the synucleinopathies. Differences in cellular deposition and neuronal populations affected by α-synuclein... (Review)
Review
Autonomic dysfunction is a characteristic feature in the synucleinopathies. Differences in cellular deposition and neuronal populations affected by α-synuclein aggregation influence the manifestations and severity of autonomic failure in the different synucleinopathy disorders. The Lewy body disorders (Parkinson's disease, dementia with Lewy bodies, and pure autonomic failure) have predominantly peripheral involvement, whereas multiple system atrophy chiefly manifests as central autonomic failure. Clinical and laboratory features may be useful in distinguishing the different synucleinopathies based on the pattern and severity of autonomic failure. Treatment recommendations are aimed at the underlying pathophysiology and utilize non-pharmacologic and pharmacologic approaches. This review will focus on pathophysiology, clinical manifestations, and management recommendations for autonomic failure including neurogenic orthostatic hypotension, thermoregulatory dysfunction, genitourinary dysfunction, and gastrointestinal dysfunction in the synucleinopathies.
Topics: Autonomic Nervous System Diseases; Humans; Lewy Body Disease; Multiple System Atrophy; Parkinson Disease
PubMed: 32906169
DOI: 10.1055/s-0040-1713844 -
Zhurnal Nevrologii I Psikhiatrii Imeni... 2023The article presents a progressive neurodegenerative disease - multisystem atrophy, characterized by a combination of autonomic failure and various motor disorders,...
The article presents a progressive neurodegenerative disease - multisystem atrophy, characterized by a combination of autonomic failure and various motor disorders, including parkinsonism and/or cerebellar ataxia; etiopathogenetic factors and variants of the clinical picture are described. We describe own clinical observation of a 59-old patient with cerebellar and bulbar syndromes, parkinsonism, pyramidal insufficiency, cognitive deficits, and autonomic dysfunction. The differential diagnosis included a whole range of neurodegenerative and hereditary diseases: Parkinson's disease, vascular parkinsonism, progressive supranuclear palsy, spinocerebellar ataxia, FXTAS, mitochondrial encephalopathies. The moderate severity of parkinsonism and the significant predominance of cerebellar symptoms and autonomic dysfunction make this clinical case difficult to diagnose. However, based on the life and disease history, clinical picture and research methods, a diagnosis of multiple system atrophy, cerebellar type (cerebellar, autonomic, bulbar syndrome, parkinsonism, pyramidal insufficiency and moderate cognitive impairment) was established. Differential search in such patients is a difficult task and includes a whole range of neurodegenerative and hereditary diseases due to the similarity of individual clinical and neuroimaging features and, unfortunately, the limited availability of molecular genetic diagnostic methods. However, earlier diagnosis is necessary to focus in time on the development of a personalized approach to the management of each such patient, taking into account the rate of symptoms development and steady progression, in order to ensure the longest possible survival time with an acceptable level of quality of life.
Topics: Humans; Multiple System Atrophy; Quality of Life; Parkinsonian Disorders; Ataxia; Parkinson Disease; Autonomic Nervous System Diseases
PubMed: 36843472
DOI: 10.17116/jnevro2023123021144 -
Tierarztliche Praxis. Ausgabe G,... Dec 2021
Topics: Animals; Autonomic Nervous System Diseases; Colic; Horse Diseases; Horses; Italy
PubMed: 34861738
DOI: 10.1055/a-1647-9687