ABSTRACT
A painful worsening of known difficulties in walking led us to investigate a man who presented a spastic paraparesis. Radiological investigations had to be repeated three times before making a diagnosis of a right C6 spinal dural arteriovenous fistula after a 22-month follow-up. Knowing the mechanisms leading to spinal venous hypertension may explain the low yield of the early radiological investigations that should be repeated. The efficiency of the treatment depends on the severity of the presurgical neurologic manifestations.
Subject(s)
Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/pathology , Central Nervous System Vascular Malformations/surgery , Cerebral Angiography , Chronic Disease , Disease Progression , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Reflex, Abnormal , Treatment OutcomeABSTRACT
The percentage and cause of neuromuscular (NM) diseases have been analysed during a 15-year period of time. A NM disorder was found in 10,852 patients (or 14.4% of all neurological patients seen in our hospital). Mononeuropathies have been the most common causes, followed by polyneuropathies and radiculopathies, but with a variable percentage during time for the most frequent causes, that were carpal tunnel syndrome and diabetic polyneuropathies. Myopathies, diseases of the neuromuscular junction and anterior horn cell disorders counted for 5% among all NM disorders during the first 10 years (1% of all neurological patients) and for 12% during the last 5 years (2.4% of all neurological patients), this period corresponds to the opening of our outpatient clinic dedicated to NM diseases. Among polyneuropathies, the most common causes were diabetes mellitus, chronic inflammatory demyelinating polyneuropathies and the inherited forms of peripheral neuropathies. Among myopathies, the most frequently reported symptom was exercise-induced myalgia. The 2 most common muscular dystrophies (MD) were Steinert myotonic MD, and facio-scapulo-humeral MD. Other myopathies were rare, but of diverse causes, all of them corresponding to the newly introduced definition of Rare or Orphan Diseases.
Subject(s)
Neuromuscular Diseases/epidemiology , Peripheral Nervous System Diseases/epidemiology , Adult , Humans , Switzerland/epidemiologyABSTRACT
We report a case of myositis presenting as an apparently unique progressive facial weakness. The only biological abnormality after an 8-year follow-up was an immunocytoma. Molecular analyses excluded facioscapulohumeral muscular dystrophy. Based on this single case and a review of the literature, we suggest a continuum between focal myositis and generalized myositis.
