A 31-year-old male patient was admitted to the department of neurology for progressive spastic paraparesis and sen-sory deficit in bilateral lower legs. On review of system, he had decreased libido and mild urinary frequency. On neuro-logic examination, he showed paraparesis, lower limb hypesthesia, bilateral hyperactive knee jerk and bilateral positive Babinski sign. The nerve conduction studies were sensori-motor demyelinating polyneuropathy. For spastic paraparesis and peripheral neuropathy, we approached adrenomyeloneuropathy and ascertained elevated serum very long chain fatty acids (VLCFA) level. (J Korean Neurol Assoc 19(4):427~430, 2001)
Adrenoleukodystrophy* ; Adult ; Fatty Acids* ; Humans ; Hypesthesia ; Knee ; Leg ; Libido ; Lower Extremity ; Male ; Neural Conduction ; Neurology ; Paraparesis ; Paraparesis, Spastic ; Peripheral Nervous System Diseases ; Polyneuropathies ; Reflex, Babinski

Subacute sensory neuropathies associated with primary Sjogren's syndrome have been reported rarely. We describe a woman with primary Sjogren's syndrome who developed a widespread, pure sensory neuropathy with a subacute onset. An electrophysiological study showed the typical absence or decreased amplitude of sensory nerve action potentials (SNAPs). A sural nerve biopsy showed a loss of large myelinated fibers and axonal degeneration without inflammation. The clinical course of long-standing subacute sensory neuropathy, the biopsy-documented axonal degeneration, and the neurophysiological findings suggest involvement of the dorsal root ganglia.
Action Potentials ; Axons ; Biopsy ; Female ; Ganglia, Spinal ; Humans ; Inflammation ; Myelin Sheath ; Peripheral Nervous System Diseases ; Sjogren's Syndrome* ; Sural Nerve

Myasthenia gravis has been associated with a variety of autoimmune diseases, but the association of myasthenia gravis with idiopathic thrombocytopenic purpura has been rarely reported. A previously healthy 33-year-old female presented with a 10-month history of easy fatigability, motor weakness, diplopia and menorrhage. Clinical and labora-tory findings supported the diagnosis of myasthenia gravis and idiopathic thrombocytopenic purpura. After a thymecto-my, there were surprising improvements not only in muscle strength, but also of thrombocytopenia. Though this associ ?ation may be incidental, the patient's progress suggests that idiopathic thrombocytopenic purpura may be closely relat-ed to myasthenia gravis immunologically because both diseases developed simultaneously and showed good therapeu-tic responses after a thymectomy.
Adult ; Autoimmune Diseases ; Diagnosis ; Diplopia ; Female ; Humans ; Muscle Strength ; Myasthenia Gravis* ; Purpura, Thrombocytopenic, Idiopathic* ; Thrombocytopenia ; Thymectomy