Extensive brain calcification and dementia in postsurgical hypoparathyroidism

Abstract

Abstract-The authors report four patients with a syndrome of painless bilateral isolated phrenic neuropathy. Electrophysiologic testing demonstrated active denervation restricted to the diaphragm. Long-term recovery was poor. The authors conclude that bilateral isolated phrenic neuropathy is a cause of painless diaphragmatic paralysis distinguishable from immune brachial plexus neuropathy and other neuromuscular disorders with similar clinical presentation. Bilateral phrenic neuropathy is a rare cause of unexplained dyspnea. Current literature generally describes this presentation in the setting of arm weakness with neck or shoulder pain, often occurring after infectious illness or surgery. [1][2][3] It follows that these factors have led authors to place this phenomenon within the spectrum of immune brachial plexus neuropathy. [1][2][3][4] In contrast, bilateral isolated phrenic neuropathy (BIPN) unaccompanied by pain, arm weakness, or antecedent events has not been emphasized as a distinct entity. In contrast to immune brachial plexus neuropathy, this painless involvement may be difficult to recognize and distinguish from other neuromuscular disorders with similar respiratory symptoms and signs.Methods. We reviewed the records of consecutive patients referred to our neuromuscular centers between 1995 and 2005 for evaluation of bilateral diaphragmatic paralysis. These patients were initially evaluated medically and referred after cardiac, pulmonary, and chest wall disorders were excluded. Presenting symptoms ranged from dyspnea, orthopnea, and exercise intolerance to frank respiratory failure. Pulmonary function testing revealed a restrictive process, with reduction of forced vital capacity with a proportional reduction of forced expiratory volume in one second suggestive of a neuromuscular disorder. Chest radiographs demonstrated bilateral diaphragmatic elevation, whereas fluoroscopy showed paradoxical upward movement of the diaphragm with sniff maneuver. Results. Illustrative case.A 43-year-old man noticed shortness of breath that developed over several days.There was no history of pain, prodromal illness, trauma, or vaccinations. A chest radiograph demonstrated an elevated left hemidiaphragm. Breathing became more difficult 4 months later and he reported that he could no longer walk 30 feet without becoming short of breath. Forced vital capacity (FVC) was 22% of predicted and the FEV1/FVC ratio was 78%. A repeat chest radiograph showed bilateral hemidiaphragm elevations and fluoroscopy confirmed diaphragmatic paralysis. Spinal fluid protein level was 61 mg/dL with a normal cell count. Antiacetylcholine receptor antibodies, anti-GM-1 antibodies, and serum creatine kinase level were normal.Electrophysiologic testing revealed unobtainable phrenic nerve conduction responses bilaterally. Nerve conduction testing of the right upper limb and low-frequency repetitive stimulation of the nasalis (facial nerve), trapezius (accessory nerve), and first dorsal interosseous (ulnar nerve) were normal. EMG of the right diaph...

Pseudohypoparathyroidism type I‐b with neurological involvement is associated with a homozygous PTH1R mutation

“…The phenomenology and severity of the neurological involvement deserves some further comment. Delirium and cognitive decline are manifestations of HP and PHP, being usually associated and maybe pathophysiologically related to the presence of intracranial calcification (Adorni et al ; Baptista et al ; El Otmani et al ; Kowdley et al ; Nicolai & Lazzarino ; Roca et al ). Hypocalcemia seems to be a key factor for calcifications as well as for common neurological symptoms like tetany, muscle cramping and seizures, which usually respond quickly to calcium replacement (Friedman et al ; Fujita ).…”

“…Chronic HP and, more rarely, PHP are associated with central neurologic manifestations like epileptic seizures and extrapyramidal signs (Bhadada et al ; Guberman & Jaworski ; Mitchell et al ). The classical radiological finding is calcification of the basal ganglia (Faissolle et al ; Goswami et al ; Kahloul et al ) and encephalopathy (Gupta et al ; Handa et al ; Oechsner et al ) or rapidly progressive dementia (Adorni et al ; Mateo & Gimenez‐Roldan ; Nicolai & Lazzarino ; Zambrana Garcia et al ) have been described, but are usually responsive to treatment and rarely progressive (Stuerenburg et al ).…”

Outcome of parathyroid function after total thyroidectomy when calcium supplementation is administered routinely versus exclusively to symptomatic patients: A prospective randomized clinical trial

“…One study reported the feasibility and safety of calcium supplementation treated only in symptomatic patients ("parathyroid stimulating"), and their management could also avoid unnecessary supplementation for most patients [12]. Some studies recommended preserving serum calcium at the low normal or mildly subnormal levels [4,11] because of the stimulation theory and to avoid side effects [4,[13][14][15].…”