In , a repeated chest x-ray film and computed tomograms disclosed the changes seen in His chest tightness, cough, and symptoms of neuropathy were still present but not severe enough for the patient to accept therapy. In , his condition worsened and he agreed to receive treatment. He was given prednisone, 60 mg daily, with instructions to continue treatment for 6 months.
After 2 weeks, the patient developed severe corticosteroid-induced side effects psychosis, insomnia, memory loss, muscle weakness, and weight gain. He discontinued the drug and did not come for a follow-up visit until At that time, he had been receiving no treatment for more than a year.
His ataxia had worsened to the degree that he was nearly totally incapacitated. Neurological examination showed marked distal wasting of muscles, loss of touch and pain sensation over the hands and feet, and impairment of vibration sense distally. An electromyogram demonstrated severe, diffuse axonal loss, and sensorimotor neuropathy, involving sensory more than motor nerves and lower extremities more than upper extremities.
The gradient distribution of abnormal findings in distal rather than proximal muscles was consistent with axonal polyneuropathy rather than mononeuritis multiplex. Magnetic resonance images of the brain, cervical spine, and thoracic spine and total-body gallium study were normal. He was given chloroquine phosphate, mg twice a day, for 6 months. He also received azathioprine, mg daily, for 3 months.
There was no response to either chloroquine or azathioprine. Subsequently, he received methotrexate, 20 mg orally, once a week for 3 months. No benefit occurred, and the patient's condition continued to deteriorate. In January , a year-old woman developed distal weakness involving both arms and the left leg. There was some tingling associated with paresthesias. These symptoms lasted for about 2 weeks and slowly resolved.
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Three months later, left facial palsy appeared. Once again, she complained of vague muscle spasms and weakness of her left leg. Parotid glands were not enlarged. Other notable findings included left upper- and lower-limb weakness, clonus, right lid ptosis, and up-going toes on both sides. A chest x-ray film showed hilar adenopathy and pulmonary infiltrate. Examinations of the CSF originally showed no white blood cells, but progressed to show 16 lymphocytes on the second and fourth taps. The CSF protein level ranged between 0.
Cultures of the CSF were negative for acid-fast bacilli and fungi. Results of serological studies including VDRL test, antinuclear antibody, anti—smooth muscle antibody, and anti—striated muscle antibody were normal. Magnetic resonance images of the brain and cervical, thoracic, and lumbar spine and bone scans were normal.
Electroencephalograms showed attenuation of alpha rhythm and occasional theta activity. Somatosensory evoked potentials of the left posterior tibial nerve were normal. Right posterior tibial nerve results were borderline nomal, indicating a possible disturbance at the cortex.
A transbronchial lung biopsy specimen showed noncaseating granulomas. An abnormal lung biopsy specimen, elevated ACE level, and CSF abnormality supported the diagnosis of neurosarcoidosis. She did not tolerate the drug and developed mood swings, acne, and weight gain. Chloroquine phosphate, mg twice a day, was started and continued for 6 months.
When last seen in , the patient was asymptomatic. There was no evidence of facial weakness. The weakness of the upper and lower extremities had subsided. She did not suffer from lower-leg numbing. Her chest x-ray film and ACE levels were normal. This previously healthy year-old woman developed focal motor seizures and transient loss of control of the left leg. She had no fever, rash, tachycardia, or influenzalike syndrome before the episode.
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Although results of a neurological examination were normal, an MR image of the brain demonstrated an intracranial mass in the right hemisphere. A chest radiograph showed bilateral hilar adenopathy, and a 67 Ga scan demonstrated uptake in hilar and parotid glands. The biopsy specimens of the brain mass showed noncaseating granulomas Figure 1. The patient's condition responded to corticosteroids, but she developed severe psychological side effects and depression.
The prednisone dosage was tapered and chloroquine phosphate, mg twice a day, was added. Finally, prednisone was discontinued. The patient's hair became bleached. Her condition remained stable for 2 years without seizures. At various times her medications included verapamil hydrochloride, sustained release Calan SR , mg twice a day; clonazepam Klonopin , 0.
Her seizures recurred after the discontinuation of chloroquine.
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Because mg chloroquine phosphate tablets were unavailable, hydroxychloroquine sulfate was started at mg twice a day. The patient became asymptomatic. This patient's neurosarcoidosis was characterized by remissions and exacerbations. She will continue to need treatment for a long period. In , a year-old man of Dutch ancestry developed acute iritis, fever, erythema nodosum, and joint pains.
About a year later he noticed weakness of the right hand grip. He could not twist caps off bottles with his right hand. Along with the progressive weakness, wasting of the right hand and forearm muscles became prominent. In early , the patient began to have pain in the left forearm muscles, atrophy of the left thenar eminence, and numbness of the left thumb, index, and middle fingers.
A chest radiograph showed diffuse pulmonary infiltrate. Noncaseating granulomas were present in lung biopsy specimens. In June , a neurological examination disclosed moderate atrophy of the right forearm and intrinsic hand muscles and selective atrophy of the left abductor pollicis brevis muscles. There were frequent twitches of the fingers on the right hand. Muscle strength was normal in the shoulders, upper arms, and wrist extensors. In the right arm, there was moderate weakness of wrist flexion and finger extension, slight weakness of hand grip, slight weakness of flexor pollicis longus and digitorum profundus, and severe weakness of intrinsic hand muscles.
In the left upper extremity, strength was normal except for slight weakness of the abductor pollicis brevis. There was no weakness in the lower extremities. Reflexes were normal except for absent right finger flexion reflex, reduced left finger flexion reflex, and absent left ankle reflex. The plantar reflexes were flexor. Sensation was normal. Results of a nerve conduction study and electromyography were consistent with diffuse axonal neuropathy, predominantly motor, with some mild demyelinating features and relative preservation of the sensory potentials.
His grip strength continued to deteriorate and was reduced to 8. He also developed cognitive side effects. Corticosteroids were discontinued and chloroquine phosphate, mg twice a day, was started. After 6 months his right hand grip improved and the left hand became almost normal.
When the patient's condition had stabilized, chloroquine was discontinued.
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In this patient chloroquine was effective despite the poor response to corticosteroids. In , a year-old man, otherwise asymptomatic, was found to have bilateral hilar adenopathy on a chest radiograph obtained during the workup for chronic sinusitis. Tuberculin, histoplasmin, and coccidioidin skin tests were negative. In , the patient became dyspneic. A chest radiograph at that time showed bilateral pulmonary infiltrates.
A lymph node biopsy specimen showed noncaseating granulomas. Bronchial lavage failed to grow acid-fast bacilli or fungi.
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Because of his symptoms, that patient was given prednisone, which he took for 6 months. In January , while not taking prednisone, he developed numbness of the right hand and tingling of both hands and forearms. All upper-extremity muscle groups were mildly weak, with a score of 3 to 4 on a scale of 0 to 5. Hope has emerged around two anti-malaria drugs: chloroquine, discovered in , and a derivative of it called hydroxychloroquine that is thought to have less severe side effects. And a small and preliminary clinical trial of hydroxychloroquine in France circulated widely and stirred excitement on social media including from the president — though its findings were hardly definitive about whether the drug would benefit coronavirus patients.
Doctors can already prescribe them off-label. I feel good about it. Just a feeling. Several other small studies of other antiviral drugs have also shown glimmers of hope.