BackgroundCryptococcus neoformans is saprophytic encapsulated yeast. Infection is acquired by inhalation of the organism and could be asymptomatic or limited to the lungs, specially in the immunocompetent host. Cryptococcal meningitis is a serious opportunistic infection among post transplant recipients. Cranial nerve palsies and ophthalmoplegia are well known complications of this disease, but bilateral complete ophthalmoplegia is a very rare presentation.Case PresentationA Sri Lankan young male, who is a post kidney transplant recipient, presented with bilateral complete ophthalmoplegia and subsequently was diagnosed to have cryptococcal meningitis based on Indian ink stain and culture of cerebrospinal fluid (CSF). His magnetic resonance imaging (MRI) showed bilateral multiple nodular lesions in both basal ganglia and thalami. Brainstem imaging was normal.ConclusionsCryptococcal meningitis is a serious fungal infection in post transplant patients. It should be suspected in any immunocompromised patient with fever, headache and focal neurological signs. Bilateral thalamic lesions, inflammation and invasion of the cranial nerves and raised intracranial pressure were thought to be possible mechanisms resulting in bilateral complete ophthalmoplegia in this patient.

BackgroundSpinal muscular atrophies (SMAs) are a group of disorders characterized by degeneration of the anterior horn cells in the spinal cord and motor nuclei in the lower brainstem. It is transmitted by autosomal recessive inheritance and most of these conditions are linked to SMN gene. Even if the clinical picture is mainly dominated by the diffuse muscular atrophy, some patients can also show atypical clinical features such as myoclonic epilepsy (“SMA plus”), which may be related to other genes. In particular, the association of SMA and progressive myoclonic epilepsy (PME) has been previously described.Case presentationWe present a case of two brothers with late onset SMA associated with a unique form of non progressive myoclonic epilepsy without cognitive impairment or ataxia. They had identical clinical and electrophysiological features.ConclusionsThe association of SMA with myoclonic epilepsy may constitute a separate and genetically independent syndrome with unique clinical and electrophysiological findings. Collection of similar cases with genetic studies is needed to define the phenotype clearly and to identify new genes and molecular pathogenetic mechanisms involved in this condition.

Pearls & Oy-sters: Ocular motor abnormalities in bilateral paramedian thalamic stroke PEARLS• The thalamus is supplied by 4 bilateral arterial systems: 3 derived from the vertebrobasilar system and 1 derived from the posterior communicating artery (PCA), each supplying the ipsilateral thalamus.• Top of the basilar syndrome caused by occlusion of the rostral portion of the basilar artery can present with an array of visual, oculomotor, and behavioral abnormalities as a result of ischemia to midbrain, thalami, and temporal and occipital lobes. OY-STERS• The artery of Percheron (AOP) is an anatomical variant arising from a single PCA and supplies the bilateral medial thalamus.• Bilateral paramedian thalamic strokes due to AOP occlusion are characterized by altered mental status, vertical gaze palsy, and memory impairment, and are similar in presentation to top of the basilar syndrome. The absence of posterior cortical visual disturbances favors AOP occlusion.CASE 1 A 67-year-old right-handed man with hypertension was admitted to a rural hospital with sudden onset of confusion, double vision, and bilateral drooping of the eyelids. He had difficulty walking and altered behavior. He was afebrile and rest of his clinical examination had normal results. He was admitted to the intensive care unit and managed as a suspected krait bite (due to its high prevalence in the area), although there was no history of a snakebite. Forty-eight hours later, the patient was fully oriented. His eye signs were persistent and he was referred to our institution for further evaluation. Neurologic examination of the eyes (figure 1) revealed bilateral asymmetric ptosis and skew eye deviation (vertical misalignment of the eyes where the eyes move upward, but in opposite directions) with an exotropia (divergent squint). Adduction of the eyes was impaired bilaterally. There was no nystagmus. Vertical saccadic and pursuit eye movements were minimal. On oculocephalic maneuvers, the eye movements were restored. Pupillary examination, convergence, and the rest of the cranial nerves were normal. The rest of the cerebellar, motor, and sensory systems were normal. His brain MRI, which was done 1 week after the onset of symptoms, revealed bilateral thalamic T1 low signal intensities and T2 high signal intensities with minimal gadolinium enhancement suggestive of bilateral thalamic infarctions. Diffusion-weighted imaging demonstrated restricted diffusion in the same region. There was no concurrent midbrain lesion. Magnetic resonance angiogram and venogram had normal results. A diagnosis of bilateral thalamic infarction due to occlusion of the AOP was made. A source of cardiothromboembolism was not elicited. The patient was managed with antiplatelets. Lid crutches were prescribed for his ptosis.CASE 2 A 47-year-old woman presented with 3 days history of acute onset of dizziness, transient loss of consciousness, and binocular diplopia with difficulty in walking unassisted. She was confused and amnesic after she regained consciousness in the hospital. Her ...

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Behcet’s disease is a multisystem vasculitis of unknown aetiology, in which neurologic involvement has been reported in the range of 5% to 10%. We report a young patient, who presented with orogenital ulcers, brainstem dysfunction and positive pathergy reaction where the diagnosis of Neuro Behcet’s disease (parenchymal type) was made. His magnetic resonance imaging (MRI) showed bilateral lesions, mainly in brainstem, thalami and periventricular regions. He was successfully treated with immunosuppressive agents.