Trochlear-oculomotor synkinesis: another congenital cranial dysinnervation disorder? / Sincinesia troclear-oculomotora: outro distúrbio congênito de denervação craniana?

ABSTRACT This report documents an unusual phenomenon. A 6-year-old girl with trochlear-oculomotor synkinesis presented with superior oblique and palpebral levator co-contraction. The literature was reviewed and the possibility of classifying this entity as a congenital cranial dysinnervation disorder was speculated.

RESUMO Este relato descreve um fenômeno incomum. Uma menina de 6 anos com sincinesia troclear-oculomotora apresentou co-contração do oblíquo superior e do levantador da pálpebra. A literatura foi revisada e especulou-se a possibilidade de classificar essa desordem como um distúrbio da congenital cranial dysinnervation disorder.

Atrofia cortical posterior / Posterior cortical atrophy. Report of five cases

Posterior cortical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer's disease. The first symptoms are progressive impairment of visuo spatial (Balint's and Gertsmann's syndromes) or visuo perceptive (visual agnosia, alexia) function. Episodic memory and executive function are spared until later stages. We report two males aged 51 and 55years and three females aged 50, 54 and 56 years, with posterior cortical atrophy. Ophthalmologic study was normal in all. Presenting signs and symptoms were visual ataxia, simultagnosia, agraphia, acalculia, spatial disorientation and unilateral neglect (Balint's and Gerstmann's syndromes). Apperceptive visual agnosia, aphasia, apraxia and alexia were also observed. One female had cortical blindness. Structural images were inconclusive, but PET scan and SPECT disclosed functional impairments in occipitotemporal or occipitoparietal areas.

Double elevator palsy, subtypes and outcomes of surgery

To describe the clinical manifestations of subtypes of double elevator palsy and to report the outcomes of surgery in these patients. This retrospective study was conducted on hospital records of patients with double elevator palsy at Labbafinejad Medical Center over a ten-year period from 1994 to 2004. Patients were classified into three subgroups of primary elevator muscle palsy [9 subjects], primary supranuclear palsy with secondary inferior rectus restriction [4 subjects] and pure inferior rectus restriction [7 subjects] according to forced duction test [FDT], force generation test [FGT] and Bell's reflex. Patients in the first group underwent Knapp procedure, the second group received Knapp procedure and inferior rectus recession simultaneously and in the third group vertical recess-resect or mere inferior rectus recess operation was performed. Success was defined as final residual deviation of 5 PD or less and 25% improvement or more in restriction after all operations. Overall 20 subjects including 10 male and 10 female patients with mean age of 12.6 +/- 9.3 [range 1.5-32] years were operated during the mentioned period which included 9 cases of primary elevator muscle palsy, 4 patients with primary supranuclear palsy and secondary inferior rectus restriction, and 7 subjects with pure inferior rectus restriction. Mean follow-up was 22.0 +/- 20.0 [range 3-63.5] months. Mean pre and post-operative deviation was 32.0 +/- 8.0 PD and 3.8 +/- 8.0 PD [P < 0.001] respectively, and mean restriction before and after the operation[s] was -3.5 +/- 0.7 and -2.3 +/- 1.2 [P < 0.001], respectively. Success rate was 77% for correction of deviation and 80% for improvement in muscle restriction. Surgery for double elevator palsy must be individualized according to FDT, FGT and Bell's reflex. The outcomes are favorable with appropriate surgical planning