Artikel
Normal pressure hydrocephalus in identical twins: A case report
Normaldruckhydrozephalus bei eineiigen Zwillingen: eine Falldarstellung
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Autoren
Veröffentlicht: | 23. April 2004 |
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Gliederung
Text
Objective
Normal pressure hydrocephalus (NPH) is a disease of the elderly patient generally without a family history. A familial association has only once been described in a man and his sister (Portenoy K, 1984). We report on identical twins, who became symptomatic at the same time in different towns.
Methods
Patient 1 was seen by a neurologist at the age of 72 after a 1 year's history of progressive hypokinetic gait disorder and memory deficits. Urinary function was not impaired. Parkisonism was suspected but the patient did not respond to Levodopa. MRI demonstrated a communicating hydrocephalus. One year later the identical twin, a 73 year old male, presented with a 2 years´ history of progressive gait disturbance, memory deficits, and urinary incontinence. MRI again showed an enlargement of all ventricles. Mental capability was assessed by the mini-mental state examination.
Results
Both patients received one or multiple spinal tap tests resulting in a temporary improvement of the gait ataxia. Diagnosis of NPH was further confirmed by a pathologic lumbar infusion test in case 2. Ventricular peritoneal shunts were implanted using a medium pressure valve (case 1) and a programmable valve with an opening pressure of 120 mm H2O (case 2). In the uneventful postoperative course, gait ataxia (both cases) and urinary function (case 2) improved. Mini mental state performance remained unchanged (case 2). On 3/12 months follow-up urinary incontinence and gait ataxia had almost completely resolved while memory deficits persisted.
Conclusions
NPH in identical twins indicates a genetic determination that may be due to congenital arrested or to late-onset hydrocephalus. Only in pediatric syndromes have X-linked recessive, autosomal recessive and dominant inheritance been reported.