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Villaret’s syndrome in a man with prostate carcinoma
Letters to the Editor
as intravenous antibiotics at home and about physicians providing house calls (1). This finding led us to conclude that a preference for a new system of care, such as acute home care, depends critically on patients’ beliefs in its feasibility. However, as we also demonstrated in our study, many older people hold positive perceptions of hospital care and negative perceptions of home care for other reasons. In addition, preferences for hospital care are influenced by fears of being alone among those who live alone (1), and fears of burdening family among those who live with others (2). As Leff and colleagues note, some older patients or their caregivers may prefer hospital care for reasons unrelated to its efficacy. Terri R. Fried, MD VA Connecticut Healthcare System and Yale University School of Medicine New Haven, Connecticut 1. Fried TR, van Doorn C, Tinetti ME, Drickamer MA. Older persons’ preferences for site of treatment in acute illness. J Gen Intern Med. 1998;13:522–527. 2. Fried TR, van Doorn C, O’Leary JR, et al. Older persons’ preferences for site of terminal care. Ann Intern Med. 1999;131:109 – 111.
VILLARET’S SYNDROME IN A MAN WITH PROSTATE CARCINOMA To the Editor: Villaret’s syndrome, which consists of palsy of the last four cranial nerves with ipsilateral Horner’s syndrome, was first described by Villaret in France in 1916 (1). We report a patient with Villaret’s syndrome and prostate carcinoma. A 64-year-old white man with metastatic prostate carcinoma, which was diagnosed in November 1997, presented in June 1998 with dysphagia, odynophagia, dysphonia, and back pain. The patient had an episode of dysphagia in March 1998 when a cyst was removed 516
April 15, 2000
Figure. Magnetic resonance image of the skull base with a soft tissue mass measuring 1.5 by 3 cm inferior and medial to the foramen jugulare (black arrow) and displacing the left carotid artery laterally (black pointer).
from his vallecula. Physical examination in June 1998 revealed left-sided partial ptosis with meiosis, tongue deviation to the left, an absent gag reflex, a weak voice, and a wasted left sternocleidomastoid muscle. Flexible laryngoscopy revealed a left vocal cord that was fixed in the midline. Computed tomographic and magnetic resonance imaging (MRI) scans of the brain were negative for an intracranial mass. However, MRI of the skull base (Figure) revealed a 1.5 by 3 cm soft tissue mass inferior and medial to the foramen jugulare that displaced the left carotid artery laterally. The patient was treated with palliative radiation therapy and the vocal cords were injected with Gelfoam with no significant improvement in his symptoms. Villaret’s syndrome, which was first described among injured soldiers (1), is part of the jugular foramen syndrome in which multiple lower cranial nerves are damaged. Fewer
THE AMERICAN JOURNAL OF MEDICINE威
Volume 108
than 30 cases have been reported. In a series of 29 patients from the Mayo Clinic with jugular foramen syndrome, only 2 patients have had Villaret’s syndrome (2,3). The most common causes are masses and foreign bodies in the retroparotid space, such as shrapnel, tumors (melanoma, renal cell carcinoma, meningioma, small cell carcinoma of the lung, and lymphoma), and aneurysms (1–5). Prostate cancer, which appears to be the cause in our patient, has not been reported. Patients present with dysphonia, dysphagia, hemiparesis of the posterior pharynx, soft palate, and vocal cord with ipsilateral deviation of the tongue and paresis of the sternocleidomastoid and trapezius muscles associated with ipsilateral partial ptosis, meiosis, and enophthalmos (3). Treatment consists of treating the cause of the compression either by surgery or radiation therapy. Toni Sicenica, MD, MSc Gobi Venkata Balaji, MD
Letters to the Editor
Albert Klein, MD Peter Berman, MD Usman Ahmad, MD Department of Internal Medicine and ENT Department UPMC McKeesport McKeesport, Pennsylvania 1. Villaret M. Le syndrome de l’espace retroparotidien posterieur. Rev Neurol. 1916; 29:188 –190. 2. Boon P, de Reuck J, van de Velde E. Villaret’s syndrome due to thrombosis of the jugular vein. Clin Neurol Neurosurg. 1990; 92:337–341. 3. Svien HJ, Baker LH, Rivers MH. Jugular foramen syndrome and allied syndromes. Neurology. 1963;13:797– 809. 4. Garrett D Jr, Lee VA, Story JL. Villaret’s syndrome: a report of two cases. Surg Neurol. 1993;39:282–285. 5. Tiliket C, Petiot P, Arpin D, et al. Clinical and radiological aspects of Villaret’s syndrome. Clin Neurol Neurosurg. 1996;98: 194 –196.
NEUROLEPTIC MALIGNANT-LIKE SYNDROME IN AN ELDERLY PATIENT CAUSED BY ABRUPT WITHDRAWAL OF TOLCAPONE, A CATECHOL-O-METHYL TRANSFERASE INHIBITOR To the Editor: Neuroleptic malignant syndrome can occur after the use of potent neuroleptic agents (1,2) or after withdrawal of antiparkinsonian drugs (3). It is characterized by autonomic dysfunction, extrapyramidal signs, and hyperthermia (4). We report a patient with Parkinson’s disease who developed the syndrome after the withdrawal of tolcapone. An 88-year-old woman was admitted to the hospital for evaluation of worsening confusion and agitation. The patient’s medical history included Parkinson’s disease and mild dementia. She had been treated with carbidopa/levodopa 25/100 mg three times daily. Three weeks before pre-
sentation, tolcapone (100 mg three times daily) was added. Four days before admission, she was seen in the emergency department after a fall that resulted in a small left-sided head laceration. A computed tomographic (CT) scan of the head was unremarkable. It was suspected that she may have been experiencing a tolcaponeinduced side effect, and tolcapone was discontinued. Nursing home staff noted that she became agitated and confused, and began hallucinating. They also observed worsening rigidity, skin pallor, and diaphoresis. On admission, the blood pressure was 164/70 mm Hg, pulse was 91 beats per minute, respirations were 20 per minute, and the temperature was 37.6⬚C. She appeared confused, belligerent, agitated, and disoriented to time and place. Her speech was incoherent. Strength was 4-5/5 and symmetrical. An occasional rest tremor of the left hand was noted, along with a mild action tremor of the left upper extremity. She needed assistance with transfers, and constant support in the standing position to avoid falling. Admission laboratory tests revealed an increased serum creatine kinase level (278 u/L; normal range, 0 to 130 u/L). Other routine blood and urine tests were normal. Arterial blood gases were normal; blood cultures were negative. A head CT scan revealed mild increased ventricular size, unchanged from the previous study. A chest radiograph was normal. An electrocardiogram showed normal sinus rhythm and an old inferior infarct. Because of the concern that antiparkinsonian drugs were contributing to her confusion, carbidopa/levodopa was withheld. Forty-eight hours after admission, she developed a temperature of 39.7⬚C and was noted to have diaphoresis, skin pallor, severe rigidity, akinesia, and deterioration of consciousness. A repeat evaluation, including cultures of the urine, blood, and cerebrospinal fluid, was negative. She was treated with intravenous hyApril 15, 2000
dration. She was discharged to the nursing home on day 7, after improvement in her level of consciousness and resolution of the syndrome. During 2 months of follow-up, she has had no recurrent episodes of confusion or fever, despite reintroduction of carbidopa/levodopa therapy. Tolcapone has been developed as a selective and reversible inhibitor of cathechol-O-methyltransferase (5–9), which increases levels of levodopa in the central nervous system and prolongs the intrastriatal half-life of dopamine (6,7). Adverse side effects, which can occur in up to 7% of patients receiving doses of 300 mg to 600 mg daily (8), include postural hypotension, insomnia, confusion, hallucination, nausea, dyskinesia, anorexia, sleep disorders, diarrhea, dizziness, vomiting, and headache (9). Our patient developed neuroleptic malignant syndrome associated with tolcapone withdrawal. Cases of hyperpyrexia after carbidopa/levodopa withdrawal have been reported (3), and indeed, in our patient, withdrawal of carbidopa/levodopa led to fever and increased rigidity. A diagnostic work-up in our patient failed to identify other causes of hyperpyrexia and confusion. We postulate that the withdrawal of tolcapone caused confusion, increased rigidity, and autonomic problems (diaphoresis, pallor) typical of drug-induced neuroleptic malignant syndrome, as seen with other dopaminergic agents (10). Clinicians should be aware of this potential adverse effect. In addition to dose adjustment of carbidopa/levodopa, tolcapone should be tapered in a gradual manner to prevent rebound hyperpyrexia. Cletus U. Iwuagwu, MD David Riley, MD Robert A. Bonomo, MD Geriatric Care Center and Department of Neurology University Hospitals of Cleveland Case Western Reserve Univeristy Cleveland, Ohio
THE AMERICAN JOURNAL OF MEDICINE威
Volume 108 517
as intravenous antibiotics at home and about physicians providing house calls (1). This finding led us to conclude that a preference for a new system of care, such as acute home care, depends critically on patients’ beliefs in its feasibility. However, as we also demonstrated in our study, many older people hold positive perceptions of hospital care and negative perceptions of home care for other reasons. In addition, preferences for hospital care are influenced by fears of being alone among those who live alone (1), and fears of burdening family among those who live with others (2). As Leff and colleagues note, some older patients or their caregivers may prefer hospital care for reasons unrelated to its efficacy. Terri R. Fried, MD VA Connecticut Healthcare System and Yale University School of Medicine New Haven, Connecticut 1. Fried TR, van Doorn C, Tinetti ME, Drickamer MA. Older persons’ preferences for site of treatment in acute illness. J Gen Intern Med. 1998;13:522–527. 2. Fried TR, van Doorn C, O’Leary JR, et al. Older persons’ preferences for site of terminal care. Ann Intern Med. 1999;131:109 – 111.
VILLARET’S SYNDROME IN A MAN WITH PROSTATE CARCINOMA To the Editor: Villaret’s syndrome, which consists of palsy of the last four cranial nerves with ipsilateral Horner’s syndrome, was first described by Villaret in France in 1916 (1). We report a patient with Villaret’s syndrome and prostate carcinoma. A 64-year-old white man with metastatic prostate carcinoma, which was diagnosed in November 1997, presented in June 1998 with dysphagia, odynophagia, dysphonia, and back pain. The patient had an episode of dysphagia in March 1998 when a cyst was removed 516
April 15, 2000
Figure. Magnetic resonance image of the skull base with a soft tissue mass measuring 1.5 by 3 cm inferior and medial to the foramen jugulare (black arrow) and displacing the left carotid artery laterally (black pointer).
from his vallecula. Physical examination in June 1998 revealed left-sided partial ptosis with meiosis, tongue deviation to the left, an absent gag reflex, a weak voice, and a wasted left sternocleidomastoid muscle. Flexible laryngoscopy revealed a left vocal cord that was fixed in the midline. Computed tomographic and magnetic resonance imaging (MRI) scans of the brain were negative for an intracranial mass. However, MRI of the skull base (Figure) revealed a 1.5 by 3 cm soft tissue mass inferior and medial to the foramen jugulare that displaced the left carotid artery laterally. The patient was treated with palliative radiation therapy and the vocal cords were injected with Gelfoam with no significant improvement in his symptoms. Villaret’s syndrome, which was first described among injured soldiers (1), is part of the jugular foramen syndrome in which multiple lower cranial nerves are damaged. Fewer
THE AMERICAN JOURNAL OF MEDICINE威
Volume 108
than 30 cases have been reported. In a series of 29 patients from the Mayo Clinic with jugular foramen syndrome, only 2 patients have had Villaret’s syndrome (2,3). The most common causes are masses and foreign bodies in the retroparotid space, such as shrapnel, tumors (melanoma, renal cell carcinoma, meningioma, small cell carcinoma of the lung, and lymphoma), and aneurysms (1–5). Prostate cancer, which appears to be the cause in our patient, has not been reported. Patients present with dysphonia, dysphagia, hemiparesis of the posterior pharynx, soft palate, and vocal cord with ipsilateral deviation of the tongue and paresis of the sternocleidomastoid and trapezius muscles associated with ipsilateral partial ptosis, meiosis, and enophthalmos (3). Treatment consists of treating the cause of the compression either by surgery or radiation therapy. Toni Sicenica, MD, MSc Gobi Venkata Balaji, MD
Letters to the Editor
Albert Klein, MD Peter Berman, MD Usman Ahmad, MD Department of Internal Medicine and ENT Department UPMC McKeesport McKeesport, Pennsylvania 1. Villaret M. Le syndrome de l’espace retroparotidien posterieur. Rev Neurol. 1916; 29:188 –190. 2. Boon P, de Reuck J, van de Velde E. Villaret’s syndrome due to thrombosis of the jugular vein. Clin Neurol Neurosurg. 1990; 92:337–341. 3. Svien HJ, Baker LH, Rivers MH. Jugular foramen syndrome and allied syndromes. Neurology. 1963;13:797– 809. 4. Garrett D Jr, Lee VA, Story JL. Villaret’s syndrome: a report of two cases. Surg Neurol. 1993;39:282–285. 5. Tiliket C, Petiot P, Arpin D, et al. Clinical and radiological aspects of Villaret’s syndrome. Clin Neurol Neurosurg. 1996;98: 194 –196.
NEUROLEPTIC MALIGNANT-LIKE SYNDROME IN AN ELDERLY PATIENT CAUSED BY ABRUPT WITHDRAWAL OF TOLCAPONE, A CATECHOL-O-METHYL TRANSFERASE INHIBITOR To the Editor: Neuroleptic malignant syndrome can occur after the use of potent neuroleptic agents (1,2) or after withdrawal of antiparkinsonian drugs (3). It is characterized by autonomic dysfunction, extrapyramidal signs, and hyperthermia (4). We report a patient with Parkinson’s disease who developed the syndrome after the withdrawal of tolcapone. An 88-year-old woman was admitted to the hospital for evaluation of worsening confusion and agitation. The patient’s medical history included Parkinson’s disease and mild dementia. She had been treated with carbidopa/levodopa 25/100 mg three times daily. Three weeks before pre-
sentation, tolcapone (100 mg three times daily) was added. Four days before admission, she was seen in the emergency department after a fall that resulted in a small left-sided head laceration. A computed tomographic (CT) scan of the head was unremarkable. It was suspected that she may have been experiencing a tolcaponeinduced side effect, and tolcapone was discontinued. Nursing home staff noted that she became agitated and confused, and began hallucinating. They also observed worsening rigidity, skin pallor, and diaphoresis. On admission, the blood pressure was 164/70 mm Hg, pulse was 91 beats per minute, respirations were 20 per minute, and the temperature was 37.6⬚C. She appeared confused, belligerent, agitated, and disoriented to time and place. Her speech was incoherent. Strength was 4-5/5 and symmetrical. An occasional rest tremor of the left hand was noted, along with a mild action tremor of the left upper extremity. She needed assistance with transfers, and constant support in the standing position to avoid falling. Admission laboratory tests revealed an increased serum creatine kinase level (278 u/L; normal range, 0 to 130 u/L). Other routine blood and urine tests were normal. Arterial blood gases were normal; blood cultures were negative. A head CT scan revealed mild increased ventricular size, unchanged from the previous study. A chest radiograph was normal. An electrocardiogram showed normal sinus rhythm and an old inferior infarct. Because of the concern that antiparkinsonian drugs were contributing to her confusion, carbidopa/levodopa was withheld. Forty-eight hours after admission, she developed a temperature of 39.7⬚C and was noted to have diaphoresis, skin pallor, severe rigidity, akinesia, and deterioration of consciousness. A repeat evaluation, including cultures of the urine, blood, and cerebrospinal fluid, was negative. She was treated with intravenous hyApril 15, 2000
dration. She was discharged to the nursing home on day 7, after improvement in her level of consciousness and resolution of the syndrome. During 2 months of follow-up, she has had no recurrent episodes of confusion or fever, despite reintroduction of carbidopa/levodopa therapy. Tolcapone has been developed as a selective and reversible inhibitor of cathechol-O-methyltransferase (5–9), which increases levels of levodopa in the central nervous system and prolongs the intrastriatal half-life of dopamine (6,7). Adverse side effects, which can occur in up to 7% of patients receiving doses of 300 mg to 600 mg daily (8), include postural hypotension, insomnia, confusion, hallucination, nausea, dyskinesia, anorexia, sleep disorders, diarrhea, dizziness, vomiting, and headache (9). Our patient developed neuroleptic malignant syndrome associated with tolcapone withdrawal. Cases of hyperpyrexia after carbidopa/levodopa withdrawal have been reported (3), and indeed, in our patient, withdrawal of carbidopa/levodopa led to fever and increased rigidity. A diagnostic work-up in our patient failed to identify other causes of hyperpyrexia and confusion. We postulate that the withdrawal of tolcapone caused confusion, increased rigidity, and autonomic problems (diaphoresis, pallor) typical of drug-induced neuroleptic malignant syndrome, as seen with other dopaminergic agents (10). Clinicians should be aware of this potential adverse effect. In addition to dose adjustment of carbidopa/levodopa, tolcapone should be tapered in a gradual manner to prevent rebound hyperpyrexia. Cletus U. Iwuagwu, MD David Riley, MD Robert A. Bonomo, MD Geriatric Care Center and Department of Neurology University Hospitals of Cleveland Case Western Reserve Univeristy Cleveland, Ohio
THE AMERICAN JOURNAL OF MEDICINE威
Volume 108 517