(ed): Current Concepts in Ophthalmology. St. Louis, Mosby, 1972, V O ~ 3, pp 76-91

5. Trobe JD, Glaser JS, Post JD: Meningiomas and aneurysms of the cavernous sinus: neuro-ophchalmologic features. Arch Ophthalmol 96457-467, 1978

motor neuron disease in childhood. The similarity of onset and course in siblings suggests that it may be a genetically distinct variety.

Children’s Hospital of Michigan Detroit, M I 48201

Progressive Bulbar Palsy of Childhood in Siblings David Benjamins, M D

In 1962, Gomez, Clermont, and Bernstein [2] reported on the autopsy findings in a child with progressive bulbar paresis (Fazio-Londe disease). A similarly affected sibling of that patient recently died at Children’s Hospital of Michigan .

A 29-month-old boy was first seen because of progres- sive inspiratory stridor. A sibling had died at 44 months of age of a similar illness. Twin female siblings were well at age 7 years. The parents had no other children. The boy had been born six weeks prematurely weighing 2,386 gm. His neonatal course was uncomplicated, and early motor development was normal. He sat alone at 7 months and walked alone at 10 months.

On initial examination the child had mild bilateral ptosis. The pupils and extraocular movements were normal. Fasciculations of the tongue were seen by one observer. Deep tendon reflexes were exaggerated, but no Babinski response was elicited. Direct laryngoscopy demonstrated no movement of the right vocal cord and slight movement of the left. A tracheostomy was done. Electromyography of facial muscles (orbicularis oculi and oris) demonstrated in- creased numbers of positive sharp waves. No fasciculations or fibrillations were seen.

The child was readmitted at age 32 months because of difficulty swallowing with episodes of choking and cough- ing. On examination he had bilateral ptosis (right more than left), bilateral facial weakness, no gag reflex, and hyperreflexia in all extremities. One month later, chest fluoroscopy demonstrated diminished diaphragm move- ment bilaterally. Subsequently the child had increasing re- spiratory problems, with poor respiratory effort and poor cough. A diffuse decrease in peripheral strength developed along with tremulousness on movement and generalized muscle wasting. He died at 36 months of age, eight months after the onset of the disease. Permission for autopsy was denied.

The onset and course of disease in this patient were re- markably similar to that of his sibling; the course of an au- topsied patient reported by Alexander et al [ 11 was also similar, The presence of this disease in siblings has been reported twice before [2 ] . Our report is further evidence for an autosomal recessive form of inheritance for the trait. The clinical and pathological findings in these two siblings as well as in Alexander’s patient demonstrate that progres- sive bulbar palsy may be regarded as a bulbar variant of

References 1. Alexander M, Emery E, Koerner F: Progressive bulbar paresis

in childhood. Arch Neurol 33:66-68, 1976 2. Gomez M, Clermont V, Bernstein J: Progressive bulbar

paralysis in childhood (Fazio-Londe’s disease). Arch Neurol 6:317-323, 1962

Phenytoin-Induced Cerebellar Degeneration William C. Koller, MD, PhD, Sander L. Glatt, MD, and Jacob H. Fox, M D

McLain and associates [l] recently reported five cases of irreversible cerebellar symptoms occurring in patients on long-term phenytoin therapy for seizure disorders. Cere- bellar degeneration was documented by computed tomog- raphy (CT). Because of the absence of hypoxia associated with multiple major motor seizures, phenytoin was thought to be responsible for the cerebellar degeneration.

We wish to report preliminary data from an ongoing study of cerebellar degeneration. We have prospectively examined all patients at our institution with the findings of cerebellar degeneration on CT scan. In a one-year period, eight such patients o n long-term phenytoin therapy were identified; all were under good seizure control and had taken phenytoin for an average of fourteen years. Atrophy on CT scan consisted of enlargement of the cisterna magna and of the superior cerebellar and cerebellopontine angle cisterns. These observations implicate phenytoin as a cause of cerebellar degeneration; however, none of our patients had signs of cerebellar dysfunction on neurological exam- ination.

McLain and co-workers noted that a prolonged period of exposure to phenytoin is required to cause clinical symp- toms. They suggested that degeneration may begin with initial exposure to the drug but must progress considerably before clinical manifestations appear. Our observations suggest that phenytoin may cause cerebellar atrophy ob- servable on CT scan when patients are asymptomatic. Cerebellar degeneration has been demonstrated histo- pathologically in patients who exhibited no signs of cere- bellar dysfunction on clinical examination [2, 31.

The determinant of symptoms in patients with cerebellar degeneration is unknown. If phenytoin-induced cerebellar degeneration is a progressive disorder leading to an iat- rogenic, irreversible cerebellar syndrome, CT scanning may be helpful in detecting it before symptoms appear. However, our patients had already been on chronic

Notes and Letters 203

phenytoin for many years and may never develop a clinical correlate of their radiological abnormality.

Rush-Presbyterian -St. Luke’s Medical Center Chicago, IL 60612

Rde ren ces McLain LW Jr, Martin JT, Allen JH: Cerebellar degeneration due to chronic phenytoin therapy. Ann Neurol7:18-23, 1980 Salcman M, Defendini R, Correll J, Gilman G: Neuro- pathological changes in cerebellar biopsies of epileptic pa- tients. Ann Neurol 3:lO-19, 1978 Victor M, Adams RD, Mancall E L A restricted form of cere- bellar cortical degeneration occurring in alcoholic patients. Arch Neurol 1:579-688, 1959

r r r . Incontinence ot u rine with Long-Term Bromocriptine Therapy Govindan Gopinathan, MD, FRCP(C), and Donald B. Calne, DM, FRCP

We report a patient with Parkinson disease who developed incontinence of urine during long-term bromocriptine therapy. The patient was a 47-year-old man who had had Parkinson disease since 197 1 and was taking six tablets of Sinemet-25/250 daily. Bromocriptine was started in De- cember, 1976, and the dosage had remained at 50 mg daily since then. He had not undergone any surgical procedures on the genitourinary tract. Six months before this writing the patient developed constant dribbling of urine. He ex- perienced several on-off swings daily and also had episodes of confusion and hallucinations. The incontinence was present throughout the day, even when he was lucid and ambulatory.

He was admitted for evaluation of his incontinence on September 9, 1979. Physical examination revealed nothing other than features of parkinsonism. The prostate was not enlarged. Urinalysis was normal and cultures were nega- tive. Plain roentgenograms of the kidney, ureter, and blad- der and an intravenous pyelogram were normal. Spine roentgenograms did not reveal any abnormalities. Cerebro- spinal fluid was normal. Because of severe dyskinesias and frequent on-off phenomena, bromocriptine was stopped on the fifth hospital day. Within 24 hours the incontinence started to improve, and it disappeared in three more days. The patient was discharged from the hospital and has been seen in follow-up twice. The incontinence has not recurred since withdrawal of bromocriptine.

The pathophysiology of this patient’s incontinence is un- certain. Incontinence of urine with long-term bromocrip- tine therapy has not been reported in the series reviewed [2, 3, 6, 71. The fact that it disappeared promptly on dis- continuation of bromocriptine suggests that the drug was the causative factor. Our observation in this case is in dis-

agreement with reports of a beneficial effect of bromocrip- tine in detrusor dysfunction and favors observations made in later studies [l, 4, 51. This patient probably developed ongoing detrusor instability with long-term bromocriptine therapy. If a patient taking bromocriptine develops incon- tinence of urine, it may be worthwhile stopping the drug before embarking on an elaborate work-up.

Experimental Therapeutics Branch National Institute of Neurological

National Institutes of Health Bethesda, M D 20205

azd Communicative Disorders and Stroke

References 1.

2.

3.

4.

5.

6.

7.

Abrams PH, Dunn M: A double blind trial of bromocriptine in the treatment of bladder instability. Br J Urol5 124-27, 1979 Calne DB, Williams AC, Neophyrides A: Long term treatment of parkinsonism with bromocriptine. Lancet 1:735-737, 1978 Fahn S, Cole LJ, Snider SR: Role of bromocriptine in the treatment of parkinsonism. Neurology 29: 1077-1083, 1979 Farrar DJ, Osborne JC: Use of bromocriptine in the treatment of unstable bladder. Br J Urol 48235-238, 1976 Farrar DJ, Pryor JS: Effects of bromocriptine in patients with benign prostatic hypertrophy. Br J Urol 48:73-75, 1976 Lieberman AN, Kupersmith M, Gopinathan G, et al: Bromo- criptine in Parkinson disease: further studies. Neurology 29:363-369, 1979 Parkes D: Drug therapy-bromocriptine. N Engl J Med 301:873-878, 1979

Hepatic Cirrhosis and Intracranial Hemorrhage : Significance of the Association in 53 Pathological Cases GPrard Boudouresques,* MD, Jean-Jacques Hauw, MD, Vincent Meininger, MD, Raymond Escourolle, MD, Bernard Pertuiset, MD, AndrC Buge, MD, Francois Lhermitte, MD, and Paul Castaigne, MD

In a recent survey of 43 1 neuropathological observations of intracranial hemorrhage with pathological examination of the liver collected from 1962 to 1977 at the Laboratoire de Neuropathologie Charles Foix [l] , we found 53 (12%) examples of hepatic cirrhosis (Table). Forty-four cases were associated with intracerebral hemorrhage. This high inci- dence of intracerebral hemorrhage in hepatic cirrhosis was surprising, as it has not previously been reported in the literature [2-41. To check the significance of these data, we compared the 44 cases of intracerebral hemorrhage with a control group of 100 sex- and age-matched cases from the same laboratory collected during the same period and

204 Annals of Neurology Vol 8 No 2 August 1980