Imaging diagnosis of Lhermitte-Duclos disease and Cowden’s syndrome
Keywords:
Lhermitte-Duclos disease, dysplastic gangliocytoma of the cerebellum, Cowden's syndromeAbstract
Objective: To describe the clinical and imaging characteristics of an adult patient with Cowden syndrome and Lhermitte-Duclos disease.
Clinical case report: A 53-year-old male patient, with history of macrocranial, borderline mental retardation and IQ of 75 with the Weil test, debuted with moderate holocranial headache of subacute and progressive onset, lateralization when walking and papilledema. Given the diagnosis of obstructive hydrocephalus, a ventriculoperitoneal shunt was performed with total reversal of the initial clinical condition. The patient has received conservative treatment, before refusal of surgery, with no worsening or appearance of neoplasms.
Conclusions: Lhermitte-Duclos disease is a very rare autosomal dominant disease. The “tiger stripe” pattern on magnetic resonance imaging of a unilateral cerebellar lesion is a pathognomonic sign of the disease and a diagnostic criterion for Cowden syndrome. Despite the fact that surgical treatment is ideal, given this patient's refusal, it has been managed conservatively, with favorable evolution.
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References
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