MR of Acute Autonomic and Sensory Neuropathy Takeshi Yasuda, Gen Sobue, Yoshiki yo Hirose, Mikio Mimura , and Tsutomu Yanagi Summary: Th e case of acute autonomic and sensory neuropathy presented here demonstrated high MR signal intensity in the posterior columns of th e spinal cord. Index terms: Neuropathy ; Spinal cord , magnetic resonance; Nervous sys t em , di seases Acut e aut ono mic and sensory neuropathy was fir st described by Colan and colleagues in 1980 (1 ). Th e disease is characterized by autonomic dysfun c tion and severe sensory deficit with acute onset (2, 3). The disease is rare and is thought to be a variant fo rm of acute pandysautonomia ( 4, 5) and Guillain- Bar re syndrome (6) . Pathologi- cally, the abnormality in this disease as found in two autopsied cases was located in the dorsal root ganglia with the secondary degeneration in th e post erior column of the spinal cord (7 , 8). In th e present st udy we show the magnetic reso- nance ( MR) findings in the spinal cord of a patient wi th acute autonomic and sensory neuropathy. Case Rep ort A 44-year-old wo man had an upper respiratory tra ct infection 2 weeks before the onset of general fatigue and gait disturbance. She was adm itted to a local hospital with dif ficulty in walki ng and numbness of the hand s. Because of severe vo miting, abdomi nal distension, and numbness throughout her entire body, includi ng th e face, th e patient was transferred to our hospital. Physical exa mina t ion on admission revealed co mpl ete anhid rosis, paretic ileus, and diminished bowe l sounds. Urinary retention was noted, and catheterization was perfo rmed. Neurologically, light reflex was bilaterally sl uggish, corneal reflexes were absent, and there was a mild facial dip legia. She had mild muscular weakness of the upper limbs on both sides which was worse on the left , and she could not keep her kn ees flexed while in bed. Sensation was completely lost for all modali- ties over the entire body . Deep tendon reflexes were gen- erally absent. In the ensuing weeks, she developed postural hypotension wi th syncope. Cerebrospin al fluid pro tein was 158 mg / dL, and the cell count was normal. Neurophysio- logic studies showed that sensory nerve action potentials were not evoked in the four extremities, but motor con- duction velocities and compound muscle action potentials were normal. Sensory evoked potentials were not elicited by median nerve stimulation . Sural nerve biopsy demon- strated severe axonal degeneration of myelinated and un- myelinated fibers. Within 6 months after the onset of sy mptoms , th e motor weakness recovered, and the urinary disturbance and orthostatic hypotension markedly im- prov ed without any drugs. However , sensory loss in pain, tou ch, vibration , and position persisted in the trunk and four extremities. The patient could not walk without as- sistance because of severe sensory ataxia. MR performed on a 1 .5-T unit 6 months after the onset demonstrated a high- intensity area in the posterior column of the cervical, thoracic, and lumber spinal cord , including both the fas- ciculi cuneatus and gracilis on T2 *-weighted gradient-echo images (Fig 1). These lesions were not enhanced by gado- linium. Di scussion Our patient experienced acute onset of urinary retention , paretic ileus, postural hypotension , and severe · sensory loss. Her symptoms and signs were compatible with those of acute autonomic and sensory neuropath y (1). Other autonomic and sensory neuropathies such as diabetic neuropa- thy; alcoholic neuropathy ; neuropathies caused by botulism , amyloidosis, and malignant disease; and hereditary sensory and autonomic neuropa- th y including Reiley-Day syndrome could be ruled out by this patient's clinical features and hi story . In our case , ort hostatic hypotension , paret ic ileus, anhidrosis, and uri nary dist urbance began to re- solve within 6 months after the onset of symp- tom s. However, sensory Joss in all modali t ies over the en tire body did not recover. The absence of sensory evo ked potentials , severe axona l invo lve- men t on sural nerve biops y, and persi stent sen- Received August 10, 1992; accept ed pending revision October 16: revision received December 15. From the Departments of Neurology (T.Yas., Y. H.• T.Ya n .) and Radiology (M.M.), Nagoya Daini Red Cross Hospital; and the Fourth Depart ment of Internal Medicine (G.S.), Aic hi Medical University, Aic hi-ken, Japan. Address reprint requests to Gen Sobue, Division of Neurology, Four th Department of Internal Medicine, Ai chi Medi cal University, Nagk ute, Aich i 480-11 . Japan. JNR 15:114- 115. Jan 1994 0195-6 1 08/ 94/ 1501-0114 © American Society of Neuroradiology 114