Hereditary neuropathy with liability to pressure palsies presenting with sciatic neuropathy.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant disorder associated with recurrent mononeuropathies following compression or trivial trauma. Reports on sciatic neuropathy as the presenting manifestation of HNPP are very scarce. We report on a 21-year-old previously healthy man who was admitted with sensorimotor deficits in his left leg. He had no history of preceding transient episodes of weakness or sensory loss. Clinical and electrophysiological examinations were consistent with sciatic neuropathy. Cerebrospinal fluid investigation and MRI of the nerve roots, plexus, and sciatic nerve did not indicate the underlying aetiology. When extended electrophysiological tests revealed multiple subclinical compression neuropathies in the upper limbs, HNPP was contemplated and eventually confirmed by genetic testing.

[SPECT and FDG-PET in diagnostics of neurolues]. / SPECT und FDG-PET in der Diagnostik der Neurolues.

Syphilis is a recurrent treponematosis of acute and chronic evolution. In general it is either sexually or congenitally transmitted. Primary syphilis appears as a single and painless lesion. Secondary syphilis may manifest years later, the secondary bacteremic stage is accompanied by generalized mucocutaneous lesions. Tertiary disease can be disseminated to bones and virtually any organ, involving principally the ascending aorta and the central nervous system. Nuclear medicine provides diagnostic methods in case of skeletal manifestations by bone scan - identifying periostitis and osteomyelitis. Hepatic gummas can be imaged by 99m-Tc-colloid liver scintigraphy. In neurosyphilis brain perfusion SPECT enables imaging of cerebral involvement by small vessel endarteritis resulting from syphilitic vascular disease. 18-FDG PET is also useful to evaluate neurosyphilis, a reduction of brain glucose consumption is observed. The technique adequately enables imaging of therapeutic response and might be superior to morphologic imaging. We present our experiences with these nuclear medicine methods in patients with neurolues. The incidence of neurolues is estimated at 2 per 100.000 inhabitants worldwide, migration processes might bring a re-emergence of this disease to Austria and other developed countries of the EU. Scintigraphic methods should be kept in mind for diagnostic evaluation of neurosyphilis.