A 55-year-old man with no significant past medical history presented to us with 1 month history of aimless wandering movements of his left hand. He said that he felt that his left hand was not under his control. It would act on its own will, in form of levitation of the arm, purposeless groping of objects in the surroundings. His left hand would hold objects in its vicinity and start using it, for example it would start to write if a pen was present. The left hand also showed interference in the activities of the right hand. Like when he would bring the food towards his mouth with the right hand, the left hand would try and stop it. There was also history of inter-manual conflict in form that whenever he would put his reading glasses with his right hand, the left hand would remove them. When he would button his shirt with the right hand the left hand would unbutton it. When he would wear his cap with the right hand the left hand would pull off the cap and throw it on the ground. Many a times the patient had to use his right hand to control the activities of his left hand.
Over the last 20 days the patient started feeling that even his left leg was not under his control. While sleeping in the night the left leg would move out of the blanket and would make purposeless levitation movements. Whenever he started to walk he noticed that the left leg would step backwards. Hence he had great difficulty in walking and after effort he would drag the left leg to move forward. The patient did not feel any weakness in the left leg. There was no history of cognitive decline, myoclonus, ataxia, behavior changes, weight loss. There was no history of surgical intervention, or any toxin exposure like bismuth. Examination revealed normal speech and higher mental function. The cranial nerve and motor system examination was normal. The sensory examination showed loss of cortical sensation in form of graphesthesia, loss of two point discrimination, sensory extinction on left side of body including the face. Rest of the examination was within normal limits. Over the course of stay in the hospital (next 15 days) the patient also developed truncal ataxia, cognitive impairment, left arm myoclonus. The case was discussed as progressive left alien hand and leg syndrome. The MRI brain revealed cortical hyperintensities in bilateral frontal, bilateral parietal cortex, bilateral occipital cortex and cingulate gyrus on diffusion weighted images (DWI) [Figure 1]. The corresponding areas were also hyperintense on fluid attenuated inversion recovery (FLAIR) sequences but less than that on DWI. The electroencephalography (EEG) done using 10-20 system showed generalized periodic triphasic sharp wave complexes of 100-150 milli second (ms) duration and inter-complex interval of 500 ms to 1 second [Figure 2]. The cerebrospinal fluid (CSF) analysis showed proteins 32 g/dl, sugar 73 mg/dl (blood sugar was 80 mg/dl), Chloride 102 mM/L, cells were 2/cubic millimetre (all lymphocytes). The CSF Neuron Specific Enolase (NSE) done by enhanced chemiluminescence immunoassay was raised -143.2 ng/ml (normal below 16.3 ng/ml). CSF 14-3-3 was not done due to unavailability at our center. The CSF veneral disease research laboratory (VDRL) and tuberculosis polymerase chain reaction (Tb-PCR) were negative. The thyroid profile (including T3, T4, TSH and anti-thyroid peroxidase antibodies) was normal. Computed tomography (CT) chest, abdomen was normal. Stool for occult blood was negative. Upper and lower gastrointestinal tract endoscopy was normal. Serum levels of vitamin B12 and folate were normal. Serum HIV and VDRL were non-reactive. Carcinoembryonic antigen, alpha-fetoprotein, and prostate specific antigen were within normal limits. According to the Magnetic Resonance Imaging-Creutzfeldt-Jakob disease (MRI-CJD) Consortium criteria for sCJD the patient was diagnosed as a case of probable sCJD [Table 1].
Two out of 1 and atleast one out of 2.
Two out of 1 and duration less than 2 years.
The patient was put on sodium valproate 500 mg twice a day for myoclonus and discharged. The patient and his relatives were not willing for a brain biopsy. The prognosis of the disease was explained to the relatives.