Susac's syndrome

Susac syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. The encephalopathy manifests with headache, confusion, memory loss, behavioral changes, dysarthria, and occasional mutism. The BRAO may be extensive or subtle; if the posterior pole of the retina is involved, patients may complain
of impaired vision. These BRAO are usually bilateral and may be the presenting features of the illness, or occur later in the clinical The MR scans in SS show a rather distinctive pattern of supratentorial white matter lesions that always involve the corpus callosum. There is often deep gray matter, posterior fossa involvement, and frequent parenchymal with occasional leptomeningeal enhancement. The central callosal lesions in Susac’s differ from multiple sclerosis as the ependymal undersurface of the corpus callosum is usually involved and callosal atrophy is usually seen in demyelinating disaese. Central involvement and an appropriate clinical picture should support the diagnosis of SS in patients with at least two of the three features of the clinical triad. NEUROLOGY 2003;61:1783–1787

See Abid's Susac DWI for another nice view

CADASIL MRI

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited, autosomal dominant condition caused by mutations of the Notch3 gene. Affected individuals have migraine, mood disturbance, and recurrent strokes, often progressing to subcortical dementia and premature death. MRI findings include focal lacunar infarcts and diffuse T2-weighted hyperintensity, or leukoaraiosis. Temporal pole hyperintensity is a radiologic marker of CADASIL. Involvement of the external capsule and corpus callosum are also characteristic findings that may help to distinguish the disease. Neurology March 13, 2001 vol. 56 no. 5 628-634

Status epilepticus MRI findings

Hotter than a cup of coffee! Note: DWI imaging increased signal in thalamus, insula and cortex. Note profound hippocampal involvement on axial FLAIR and DWI images. On FLAIR images there is significant hippocampal and temporal lobe involvement. No cells on LP. EEG shows ongoing right temporal seizures. We think she was having three seizures without loss of consciousness or convulsions every 20 minutes for a week before this scan



DWI with temporal lobe involvement

DWI with insula and thalamic involvement

FLAIR showing significant hippocampal and temporal love involvement. All this injury is felt secondary to the ongoing uncontrolled seizure activity.

Convulsive syncope

Syncopal episodes may be followed by myoclonus or synchronous or asynchronous jerking. In this video a young woman has a syncopal event elicited by hyperventilation and rapid standing in a controlled environment. Not relatively short duration of the spell and lack of a tonic phase and a clonic phase. The video shows somewhat of an exaggerated event than is usually seen.

Eastchester Clapping sign

Hand clapping is a motor program mastered in infancy. A patients with hemispatial neglect who, when asked to clap, repeatedly performed one-handed motions stopping abruptly at the midline of the visual hemispace, as if pantomiming slapping an invisible board. In contrast, hemiplegic patients without neglect will reach across and clap against their plegic hands. This phenomenon provides an easy, rapid, and unambiguous test for neglect, applicable to patients of any ethnicity or age.

Internuclear ophthalmoplegia

In this video Daroff explains the INO completely. Do check it out, if only to see Daroff's completely 1960's OKN strip. We gotta get one of those for Paul Lee.

Bilateral Internuclear ophthalmoplegia

The disorder is caused by injury or dysfunction in the medial longitudinal fasciculus(MLF), a heavily-myelinated tract that allows conjugate eye movement by connecting the paramedian pontine reticular formation (PPRF) -abducens nucleuscomplex of contralateral side to the oculomotor nucleus of the ipsilateral side.

In young patients with bilateral INO, multiple sclerosis is often the cause. In older patients with one-sided lesions a stroke is a distinct possibility. Other causes are possible. (Wiki)

Doll's eye sign in comatose patient

This show the normal Doll's eyes response in a patient who has coma from bicortical dysfunction

Absent Doll's eyes in patient with massive brainstem dysfunction

Absent Doll's eye movements to VOR. Does not rule out absence to Cold calorics though.

MRI features of CSF Hypotension

Pretreatment (upper row) and post-treatment (lower row) axial fluid-attenuated inversion recovery magnetic resonance imaging (MRI) (A) showing resolution of bilateral subdural haematomas (arrowheads) and engorgement of the superior sagittal sinus (arrow) in a 35-year-old man; axial T1-weighted MRIs after intravenous administration of gadolinium (B) showing resolution of enhancement of the pachymeninges (arrowheads) in a 39-year-old woman; and sagittal T1-weighted MRIs showing resolution of sagging of the brain [flattening of the pons with obliteration of the prepontine cistern (arrowheads) and downward displacement of the cerebellar tonsils mimicking a Chiari malformation (arrowhead)] and hyperaemia/enlargement of the pituitary gland (arrow) in a 41-year-old woman. Also note restoration of ventricular size post treatment in all panels.

Schievink W Cephalalgia 2008;28:1345-1356

Hot Crossed Buns sign in Multisystem Atrophy-Cerebellar type

The hot cross bun appearance is seen in patients with MSA-c. The sign is due to a selective loss of myelinated transverse pontocerebellar fibers and neurons in the pontine raphe with preservation of the pontine tegmentum and corticospinal tracts (1). Other investigators (2) have demonstrated a similar pattern of neuronal loss in a patient with parkinsonism, the neuronal loss being secondary to presumed vasculitis, and proposed that the sign may reflect wallerian degeneration of transverse pontocere- bellar fibers secondary to vasculitic infarction. Regardless of the mechanism, this pattern of selective neuronal depletion results in a cross-shaped hyperintensity in the pons on T2-weighted MR images. Radiology: Volume 245: Number 2—November 2007

Marchiafava-Bignami disease

MARCHIAFAVA-Bignami disease (MBD) is the symmetrical demyelination of the middle portion of the corpus callosum that can be observed in people with chronic alcoholism. Its evolution may be fatal or lead to various degrees of slowly evolving cognitive impairment. Patients with a benign course have been reported. Scattered reports exisit that it may respond to thiamine injections and therefore may be a variant of Wernicke's encephalopathy.ARCHNEUROL/VOL56, JAN1999

Concentric ring of Balo

Balo's Concentric Sclerosis, is considered a rare variant of multiple sclerosis (MS), is characterized by a lesion consisting of rings of demyelination alternating with rings of intact myelin. Lesions have been observed in the spinal cord, cerebellum, brain stem, optic chiasm, and cerebral hemispheres.2 BCS is thought to be slightly more prevalent among males than among females, and to be particularly prevalent among younger men.3 Traditionally, BCS has been considered to be a rapidly fatal disease. With the advent of MRI, patients like the individual described in this Case Study have been diagnosed early, received treatment, and survived for months or years. These results imply that some cases of BCS might have a mild course, and cases that could have been effectively treated but previously remained unrecognized are now being discovered. A classification scheme for BCS has been proposed that defines the disease into the following three subtypes: a self-limited, monophasic illness; relapsing–remitting demyelination; and the classically described, primary rapidly progressive course. Nature Clinical Practice Neurology (2007) 3, 349-354

Wernicke's Encephalopathy

Basal ganglia and dentate nucleus damage in Carbon Monoxide poisoning

Caudate atrophy in Wilson's disease

Face of Panda sign in Wilson's disease

Brain MRI showed bilateral T2 hyperintensity involving putamen, thalami, and brainstem. The midbrain “panda sign” is due to high signal in the tegmentum, normal signals in the red nuclei and lateral portion of the pars reticulata of the substantia nigra, and hypointensity of the superior colliculus.

Dina A. Jacobs, Clyde E. Markowitz, David S. Liebeskind, et al. Neurology 2003;61;969

Scattered vs String of pearls signs in MCA/ICA stenosis

Humming bird sign of PSP

Susac Syndrome

Note the "streaks" and "snowballs" at the corpus callosum