Name
AMYLOIDOSIS, PRIMARY
DESCRIPTION
DETAIL
CAUSES : 1. AUTONOMIC DISORDERS WITH BRAIN INVOLVEMENT : * MULTISYSTEM DEGENERATION : AUTONOMIC FAILURE CLINICALLY PROMINENT - MULTIPLE SYSTEM ATROPHY ( MSA ) - PRAKINSON DISEASE WITH AUTONOMIC FAILURE - DIFFUSE LEWY BODY DISEASE( SOME CASES) * MULTISYSTEM DEGENERATION: AUTONOMIC FAILURE CLINICALLY NOT USUALLY PROMINENT - PARKINSON DISEASE - OTHER EXTRAPYRAMIDAL DISORDERS( INHERITED SPINOCEREBELLAR ATROPHIES, PROGRESSIVE SUPRANUCLEAR PALSY, CORTICOBASAL DEGENERATION, MACHADO - JOSEPH DISEASE ) B. UNASSOCIATED WITH MULTISYSTEM DEGENERATION * DISOREDERS MAINLY DUE TO CEREBRAL CORTEX INVOLVEMENT - FRONTAL CORTEX LESIONS CAUSING URINARY/BOWEL INCONTINENCE - PARTIAL COMPLEX SEIZURES * DISORDERS OF THE LIMBIC & PARALIMBIC CIRCUITS - SHAPIRO SYNDROME( AGENESIS OF CORPUS CALLOSUM, HYPERHIDROSIS, HYPOTHERMIA) - AUTONOMIC SEIZURES * DISORDERS OF THE HYPOTHALAMUS - WERNICKE KORSAKOFF SYNDROME - DIENCEPHALIC SYNDROME - NEUROLEPTIC MALIGNANT SYNDROME - SEROTONIN SYNDROME - FATAL FAMILIAL INSOMNIA - ANTIDIURETIC HORMONE SYNDROME( DIABETES INSIPIDUS, INAPPROPRIATE ADH ) - DISTURBANCES OF TEMPRATURE REGULATION( HYPERTHERMIA, HYPOTHERMIA ) - DISTURBANCES OF SEXUAL FUNCTION - DISTURBANCES OF APPETITE - DISTURBANCES OF BP / HR & GASTRIC FUNCTION - HORNER SYNDROME * DISORDERS OF BRAIN STEM & CEREBELLUM - POSTERIOR FOSSA TUMORS - SYRINGOBULBIA & ARNOLD CHIARI MALFORMATION - DISORDERS OF BP CONTROL ( HYPERTENSION , HYPOTENSION ) - CARDIAC ARRHYTHMIAS - CENTRAL SLEEP APNEA - BAROREFLEX FAILURE - HORNER SYNDROME 2. AUTONOMIC DISORDERS WITH SPINAL CORD INVOLVEMENT * TRAUMATIC TETRAPLEGIA * SYRINGOMYELIA * SUBACUTE COMBINED DEGENERATION * MULTIPLE SCLEROSIS * AMYOTROPHIC LATERAL SCLEROSIS * TETANUS * STIFF MAN SYNDROME * SPINAL CORD TUMORS 3. AUTONOMIC NEUROPATHIES A. ACUTE/SUBACUTE NEUROPATHIES * SUBACUTE AUTOIMMUNE AUTONOMIC NEUROPATHY ( PANAUTONOMIC NEUROPATHY, PANDYSAUTONOMIA ) - SUBACUTE PARANEOPLASTIC AUTONOMIC NEUROPATHY - GUILLAIN-BARRE SYNDROME - BOTULISM - PORPHYRIA - DRUG INDUCED AUTONOMIC NEUROPATHIES - TOXIC AUTONOMIC NEUROPATHIES B. CHRONIC PERIPHERAL AUTONOMIC NEUROPATHIES * DISTAL SMALL FIBER NEUROPATHY * COMBINED SYMPATHETIC & PARASYMPATHETIC FAILURE - AMYLOID - DIABETIC AUTONOMIC NEUROPATHY - AUTOIMMUNE AUTONOMIC NEUROPATHY ( PARANEOPLASTIC & IDIOPATHIC ) - SENSORY NEUROPATHY WITH AUTONOMIC FAILURE - FAMILIAL DYSAUTONOMIA ( RILEY-DAY SYNDROME )* LABELED METAIODOBENZYLGUANIDINE UPTAKE ON SINGLE PHOTON EMISSION COMPUTED TOMOGRAPHY OR FLUORODOPAMINE ON PET (POSITRON EMISSION TOMOGRAPHY) IS MARKEDLY IMPAIRED IN DYSAUTONOMIA OF PARKINSON DISEASE BUT IS NORMAL IN MSA.
TYPENOTES
RELATED DISEASE
Not Available Disease
Disease
Remarks
AUTONOMIC DYSFUNCTION
DUE TO AUTONOMIC NEUROPATHY
DISEASE
INVESTIGATION
BLOOD SUGAR ( FASTING ), MRI BRAIN ( FUNCTIONAL ), BLOOD SUGAR ( AFTER MEALS ), COMPLETE BLOOD COUNT, MRI HEAD & SPINE, VITAMIN B 12