Seventh Case : Hard Nut to Crack

Somehow you have managed to get out of that ED job and become a consultant in neuromedicine but before you could really start to enjoy it , you were referred an extemely tough case . It was a 51-year-old man whose misery started some eight months ago when he developed paresthesias of both feet. This was followed within weeks by early satiety, postprandial vomiting of undigested food, and reduced frequency of bowel movements. He became completely unable to eat and lost 60 Ib. A J-tube was placed. He had a 21 packyear cigarette habit, naturally Doctors suspected some carcinoma but extensive studies for malignancy were negative. In the subsequent weeks, your patient developed dry mouth, orthostatic hypotension, lower extremity loss of sweating, and impotence d. Medical therapy failed to improve the gastrointestinal dysmotility. Eleven months into this illness, refractory simple partial and secondary generalized seizures developed, with postictal aphasia, headaches, short-term memory loss, depression, and night sweats.These are the circumstances when he comes to you

On Examination you found :
The pupillary light reflex was normal. Other findings included a mild reduction in short-term memory and asymmetric loss of pin and temperature sensations in a stocking distribution. His feet were dry. Measurement of blood pressure with the patient standing revealed orthostatic hypotension.

After aa series of investigations you came to know that his Autonomic studies documented orthostatic hypotension and a blunted heart rate response to a Valsalva maneuver. Sweating was reduced in the legs. Gastroparesis and delayed small-bowel transit time were demonstrated. At presentation, serum type 1 antineuronal nuclear autoantibody (ANNA-1) was detected (1:15, 360). Cranial magnetic resonance imaging (MRI) showed mild atrophy and increased fluid attenuation inversion recovery (FLAIR) signal in the left mesial temporal, insular, and opercular cortices (Figure). The results of an exploratory mediastinoscopy and a video-assisted thoracic surgery procedure were negative. Computed tomographic (CT) scans of the chest at 3-month intervals and positron emission tomographic (PET) scans were uninformative.

Now you have his MRI and reports on your table and you have to diagnose and treat this poor fellow