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.
Uses for Hydroxychloroquine Sulfate
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. 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. 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.
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A lymph node biopsy specimen showed noncaseating granulomas. Bronchial lavage failed to grow acid-fast bacilli or fungi. 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. There was no muscle atrophy or fasciculation. An unenhanced MR image of the brain and spinal cord was normal in February An electromyogram and a nerve conduction study of the right upper extremity were normal except for mild slowing for motor median conduction across the wrist.
The significance of the minimal stalling for median conduction was unclear. There was no evidence of radiculopathy. Treatment was restarted with prednisone, 60 mg daily. Within 6 months, there was a complete resolution of numbness, tingling, and muscle weakness. The patient, however, had become severely depressed and cushingoid.
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The prednisone dosage was gradually reduced. He remained obese and depressed. A few months later his right Achilles tendon ruptured. At this point, hydroxychloroquine sulfate, mg twice a day, was added. His muscle weakness and numbness abated. Hydroxychloroquine was continued for 18 months. When last seen in , the patient had no neurological symptoms.
He had lost weight and was no longer depressed.
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A chest radiograph and serum ACE level were normal. A year-old African American man with a history of pulmonary, cutaneous, and upper respiratory tract sarcoidosis, diagnosed in , developed marked fatigue, headaches, and eye pain. Examination of his eyes disclosed mild low-grade vitreitis.
Hydroxychloroquine
Serum ACE level was normal. An MR image of the brain showed diffuse leptomeningeal enhancement in the frontal lobes, interhemispheric fissure, and right temporal and occipital lobes Figure 2 , left, and Figure 3 , left. The patient stopped using prednisone because of depression but continued taking hydroxychloroquine.
After 8 months his headaches and weakness improved markedly. A repeated MR image after 11 months showed moderate improvement Figure 2 , right. Because of the residual MR imaging abnormality, the patient was given hydroxychloroquine for another 5 months. When the drug was discontinued after 1 year, the patient's symptoms recurred.
The MR imaging was not repeated, but hydroxychloroquine sulfate, mg twice a day, was reinstituted with improvement in symptoms. Left, Gadolinum-enhanced magnetic resonance image axial view of the brain showing multiple areas of diffuse leptomeningeal enhancement in the temporal and occipital lobes.
There is no evidence of optic nerve involvement patient 9; Table 1. Right, Much diminished leptomeningeal enhancement after treatment with hydroxychloroquine sulfate, mg, twice a day for 5 months. Left, Gadolinium-enhanced magnetic resonance image of the brain coronal view showing extensive leptomeningeal enhancement patient 9; Table 1. Right, Five months after hydroxychloroquine sulfate treatment, leptomeningeal enhancement is much diminished.
In March , a year-old African American man was examined because of increasing fatigue, polydipsia, nocturia, and decreased libido. A mediastinal lymph node biopsy showed noncaseating granuloma.
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A computed tomographic scan of the brain showed a 1-cm suprasellar enhancing mass. Serum ACE level was In August , computed tomography showed reduction in size of the pituitary mass. The patient did not tolerate prednisone; his weight and serum cholesterol level increased. Headaches reappeared and he developed uveitis. Optic atrophy secondary to a chiasmal mass appeared.
This was most likely related to his poor compliance with treatment. In , an exploration of the optic chiasma was performed. The arachnoid at the base of the skull was peppered with sarcoid granulomas. The optic chiasma was diffusely thickened and enlarged. The lesion was not surgically resectable.
His neurological status deteriorated. Because of the compliance problems and corticosteroid-induced side effects, the patient was given hydroxychloroquine sulfate, mg twice a day. The patient's condition stabilized initially, but the disease slowly progressed despite hydroxychloroquine therapy for 2 years.