Neurological manifestation of Whipple's disease is:
Ans. is 'a' i.e., Seizures o Whipple's disease is a rare disease caused by the bacterium Tropheryma whippeli.o It is a systemic condition that may involve any organ of the body but principally affects the# Intestine# Central nervous system# JointsClinical features-o The onset of Whipple s disease is insiduous and it is characterized by# Diarrhoea# Steatorrhea# Abdominal pain# Weight loss# Migratory large joint arthropathy# Fever# Ophthalmologic and central nervous system symptoms.# Dementia is a relatively late symptom and is an extremely poor prognostic sign.o CNS symptoms have been reported in 10% to 43% of patients with intestinal whipples. (more recent series reporting lower ratio).# CNS manifestation can occur concurrently with intistinal manifestation at the time of diagnosis, but is more common at the time of clinical relapses which can occur during or after the treatment.o 2 recives summarized the CNS manfestation of whipples's disease in 84 & 122 published cases suspectivity: -o Common finding are : -i) Supranuclear ophthalmoplegia (32 to 51%).ii) Progressive dementia & cognition changes (28 to 71%).iii) Altered level of conciousness (27 to 50%).o Less common finding are:-# Psychiatric symptoms, hypothalmic manifestions (i.g., polydipsia, hyperphagia, insomnia), cranial nerve abnormalities, nystagmus, seizures & Ataxia.Two signs that are characteristic of C.N.S. whipples disease are:# Occulomasticatory myorhythmia# Oculofacial skeletal myorhythmia.o Each consisting of slow rhythm & synchronised contraction (~l/sec) of ocular, facial or other muscles.o They occur in < 20 % of cases.o They have not been documented in other CNS diseases.Diagnosiso The diagnosis of Whipple's disease is suggested by a multisystem disease in a man with diarrhoea and steatorrhoea.o The primary approach to establish diagnosis in these cases is to obtain tissue biopsies from small intestine.o On biopsy the hallmark of Whipple's disease is the presence of distended macrophages within the small intestine mucosa. These macrophages contains PAS positive diastase resistant granules (which are lysosomes stuffed with partially digested microorganisms) and rod shaped micro organism.o Earlier the diagnosis was entirely based on demonstration of these PAS positive macrophages with organisms but it is now recognized that PAS positive macrophages may also be seen in AIDS patients with mycobacterium avium complex.# So, now PCR testing of infected tissue is the optimal way to confirm the diagnosis and monitor the t/t.
Seizures
Cerebellar ataxia
Focal neurological deficits
Encephalopathy
Intestine