Disulfiram Neuropathy: Two Case Reports

Anh Thu Tran; Richard A. Rison; Said R. Beydoun


J Med Case Reports. 2016;10(72) 

In This Article

Case Presentation

Case 1

Our first patient was a 25-year-old Caucasian woman who was referred to our tertiary neuromuscular clinic for a second opinion regarding her neuropathy. She had a history of heavy alcohol use from ages 18 to 23, during which she consumed as many as eight to ten drinks per day. She first had sensory symptoms at age 22, described as numbness of her feet as if they were "falling asleep." She initially did not pay attention to the symptoms. Gradually, her symptoms progressed and included burning, electric shock sensation, and allodynia to objects including bed sheets, socks, and the floor when walking barefoot. She was placed on disulfiram therapy (500 mg orally every morning) for 6 months, then discontinued for several months before it was reinstituted for an additional 2 months at the same dose. By the eighth month of therapy, she was unable to walk owing to severe symptoms of pain and stiffness in her legs. She also noted symptoms in her hands. She stopped taking the disulfiram and was able to walk within a few weeks, but she continued to have pain and numbness.

She had seen a community neurologist prior to seeking a second opinion in our clinic. Laboratory studies completed previously showed normal levels of HbA1c, vitamins B1 and B12, folate, Sjögren syndrome antigen A and B antibodies, antinuclear antibody, antineutrophil cytoplasmic antibodies, and antigliadin antibodies. She had monoclonal gammopathy, and results of a sensorimotor neuropathy antibody panel were within normal limits. Pertinent examination findings showed that she had normal motor function except for decreased bulk in her extensor digitorum brevis. A sensory examination showed a gradient distribution decrease in light touch, temperature, and pinprick distally, along with decreased vibration and position sense at her toes. Her gait and reflexes were normal. Electrodiagnostic studies showed a severe length-dependent motor neuropathy, as evidenced by a significantly decreased amplitude in the compound motor action potential (CMAP) in her lower extremities with a preserved CMAP in her upper extremities. There was sparing of her large fiber sensory nerves, as evidenced by normal sensory nerve action potentials (SNAPs). Both right and left posterior tibial CMAP waveforms showed temporal dispersion as well as prolonged duration. Electromyography (EMG) was performed on selected muscles in her bilateral lower extremities and revealed 2+ fibrillation potentials and positive sharp waves in her abductor hallucis muscles. Also seen were polyphasia, increased amplitude and increased duration with decreased recruitment, and increased firing rate to most muscles sampled.

Case 2

Our second patient was a 46-year-old Caucasian woman who was being followed by a community neurologist, to whom she reported sudden onset bilateral numbness of her feet when walking. She had progressively worsening symptoms, including freezing/burning pain, and allodynia to bed sheets and when walking barefoot. She was initially referred to our tertiary neuromuscular clinic; however, she was admitted to the Keck Medical Hospital owing to the acutely progressive nature of her symptoms. During her hospital stay she had an unremarkable workup, including lumbar puncture, cervical and thoracic MRI, as well as chest, abdominal, and pelvic CT. Results from an additional laboratory workup, including testing for vitamin B12, folate, TSH, rapid plasma reagin, heavy metals, HbA1c, and homocysteine, and a serum electrophoresis were normal.

She presented to our clinic a month after being discharged from the hospital. She reported a 3-month history of sudden-onset numbness in both feet that had worsened and which she described as a frostbite sensation of freezing and burning. She had allodynia to bed sheets and walking barefoot that she described as a sensation of "walking on pegs." Her social history showed heavy alcohol use of one bottle of wine daily. She had taken disulfiram at a dose of 500 mg daily but had stopped the medication a couple of weeks prior to her hospital admission when she developed symptoms of neuropathy. Since her discharge from hospital, she reported that she was no longer feeling pain and that the frostbite sensation had improved. Pertinent findings included normal results from a motor examination except for weaker bilateral ankle dorsiflexion. A sensory examination showed distal changes including absent position sense at the toes, significantly decreased vibration at the toes, and absent light touch and pinprick to the distal 5 cm of her feet. Electrodiagnostic testing was performed several weeks after her initial clinic visit and a nerve conduction study revealed severe motor axonal neuropathy in her lower extremity nerves with length-dependent characteristics. Her bilateral peroneal CMAPs were significantly decreased. By contrast, her upper extremity CMAP parameters were normal. There was sparing of her large fiber sensory nerves as evidenced by normal SNAPs. EMG showed 1+ positive sharp waves and fibrillation potentials in most of the sampled muscles of her lower extremities. There were slight to moderate increases in the amplitudes of the motor unit potentials as well as increased polyphasia.

Please see Table 1 and Table 2 for electrodiagnostic data for both patients.