title: Neurological manifestations of phaeochromocytomas and secretory paragangliomas: a reappraisal.
abstract: OBJECTIVE
  To determine the frequency and range of neurological manifestations of phaeochromocytomas and secretory paragangliomas.
  
  
  METHODS
  A retrospective review of case notes of patients admitted to Auckland Hospital from 1985 to 2011 with a discharge diagnosis of phaeochromocytoma or secretory paraganglioma.
  
  
  RESULTS
  Ninety-three patients were admitted with a phaeochromocytoma or secretory paraganglioma. Sixty-eight patients (73%) had neurological symptoms, but only 15 patients (16%) received a neurological consultation. Neurological manifestations occurred in three main clinical contexts. First, paroxysmal symptoms occurred in 66 of 93 patients (71%). Neurological symptoms were common features of these attacks and included headache (47 patients), anxiety (24 patients), tremulousness (15 patients) and dizziness (12 patients). The headaches typically had an explosive onset. Delay in diagnosis was common. Second, 28 patients (30%) had an acute crisis, which was associated with neurological symptoms in 11 (39%) of the episodes: headache (10 patients); seizures (five patients); strokes (three patients); delirium (three patients) and subarachnoid haemorrhage (one patient). Third, five of six patients with a head and neck secretory paraganglioma had neurological symptoms related to infiltration of the middle ear or compression of cranial nerves. Reversible cerebral vasoconstriction syndrome (RCVS) was documented in three patients.
  
  
  CONCLUSIONS
  Neurological manifestations of phaeochromocytomas and secretory paragangliomas were common, and these tumours can present with various neurological manifestations. The paroxysmal symptoms can be incorrectly attributed to other headache syndromes, panic attacks or cerebral vasculitis. RCVS may play a role in the pathogenesis of the neurological symptoms associated with acute crises and paroxysmal attacks.
authors.affiliation: Department of Neurology, Auckland City Hospital, Private Bag 92024, Auckland 1, New Zealand. neila@adhb.govt.nz
authors.forename: Neil E
authors.initials: NE
authors.lastname: Anderson
authors.forename: Kong
authors.initials: K
authors.lastname: Chung
authors.forename: Ernie
authors.initials: E
authors.lastname: Willoughby
authors.forename: Michael S
authors.initials: MS
authors.lastname: Croxson
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000473:pathology* / Q000523:psychology*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002318:Cardiovascular Diseases / Q000209:etiology; D002533:Cerebral Angiography; D002648:Child; D004830:Epilepsy, Tonic-Clonic / Q000209:etiology; D005260:Female; D006258:Head and Neck Neoplasms / Q000150:complications / Q000473:pathology / Q000523:psychology; D006261:Headache / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000209:etiology; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000209:etiology / Q000473:pathology* / Q000523:psychology*; D010235:Paraganglioma / Q000150:complications / Q000473:pathology* / Q000523:psychology*; D010673:Pheochromocytoma / Q000150:complications / Q000473:pathology* / Q000523:psychology*; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000473:pathology / Q000517:prevention & control; D012186:Retroperitoneal Neoplasms / Q000150:complications / Q000473:pathology / Q000523:psychology; D012189:Retrospective Studies; D055815:Young Adult