Search results for “cerebral atrophy”

180 results found
Article

Visual agnosia

Visual agnosia is a special type of agnosia where patients are unable to recognize objects despite having knowledge about them and being able to visually perceive them. It typically occurs when there is damage to the ventral occipitotemporal pathway, such as in posterior cerebral atrophy (Benso...
Article

Hyperostosis frontalis interna

Hyperostosis frontalis interna is characterized by benign overgrowth of the inner table of the frontal bone. The etiology is unknown. The condition is generally an incidental finding of no clinical significance. It is typically bilateral and symmetrical and may extend to involve the parietal bon...
Article

Subdural hygroma

Subdural hygromas (alternative plural: hygromata 9) refer to the accumulation of fluid in the subdural space. In many cases, it is considered an epiphenomenon of head injury when it is called a traumatic subdural hygroma.  Epidemiology Subdural hygromas are encountered in all age-groups but ar...
Article

Cella media index

The cella media index (also known as Schiersmann's index) is one of the methods used to assess ventricle size with respect to brain tissue and cerebral atrophy. It is the ratio of biparietal diameter of skull to maximum external diameter of bodies of the lateral ventricles (cella media) 1. A no...
Article

Dementia

Dementia, relabelled major neurocognitive disorder in the Diagnostic and Statistical Manual of Mental Disorders (DSM-5), is a clinical syndrome defined by progressive decline in one or more cognitive domains relative to a person's premorbid functional level. To fulfill the criteria for dementia,...
Article

Kearns-Sayre syndrome

Kearns-Sayre syndrome (KSS), also known as oculocraniosomatic disorder, is a rare multisystem mitochondrial disorder.  Clinical presentation The patient often presents with progressive external ophthalmoplegia 1. Neurologic symptoms develop in childhood or adolescence, usually before 20 years ...
Article

Menkes disease

Menkes disease, also known as trichopoliodystrophy or kinky hair kinky vessel syndrome, is an X-linked recessive disorder that results in a derangement in copper handling. It results in low copper levels and subsequently, deficiency in copper-dependent mitochondrial enzymes.  Epidemiology Menk...
Article

Fucosidosis

Fucosidosis is a rare inherited autosomal recessive lysosomal storage disorder, hypomyelinating disorder, and mucopolysaccharidosis-like disorder, characterized by multiorgan accumulation of fucose-containing products. Epidemiology It is considered very rare, with approximately only 100 cases ...
Article

Congenital cerebral toxoplasmosis

Congenital cerebral toxoplasmosis is a manifestation of congenital toxoplasmosis and refers to development of cerebral toxoplasmosis in the fetus through maternal transmission. Please refer on congenital toxoplasmosis to a broad discussion on epidemiology and pathology.  Radiographic features ...
Article

Encephalocraniocutaneous lipomatosis

Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland syndrome, is a rare congenital neurocutaneous syndrome characterized by unilateral lipomas of the cranium, face, and neck, ipsilateral lipodermoids of the eye, and ipsilateral brain anomalies. The clinical features of ECCL ove...
Article

Parry-Romberg syndrome

Parry-Romberg syndrome (PRS), also known as progressive facial hemiatrophy (PFH), is a rare progressive craniofacial disorder (phakomatosis). It is classically characterized by a slowly progressive degeneration (atrophy) of the soft tissues of half of the face (hemifacial atrophy). This involve...
Article

Cerebrotendinous xanthomatosis

Cerebrotendinous xanthomatosis is an autosomal recessive lipid storage disorder caused by defects in sterol-27-hydroxylase enzyme in bile acid synthesis. Clinical presentation The clinical presentation is highly variable with many potential neurological and other/systemic clinical features 6. ...
Article

Sturge-Weber syndrome (mnemonic)

Some key findings pertaining to Sturge-Weber syndrome may be recalled using the following mnemonics: STURGE CAPS 8 Cs Mnemonics STURGE CAPS S: seizures, sporadic T: tram-track gyriform calcification; trigeminal territory port-wine stain U: unilateral weakness (hemiparesis contralateral to...
Article

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a rare inherited autosomal recessive leukodystrophy characterized by slowly progressive pyramidal, cerebellar, and dorsal column dysfunction. Epidemiology Although considered rare, the exact prevalen...
Article

Biotinidase deficiency

Biotinidase deficiency is a rare autosomal recessive condition in which the body is unable to break down the conjugated form of biotin (vitamin B7), resulting in low levels of bioavailable biotin, and clinical biotin deficiency. Epidemiology Profound deficiency (<10% of normal level) is estima...
Article

Rasmussen encephalitis

Rasmussen encephalitis, also known as chronic focal encephalitis, is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. It is not to be confused with a Rasmussen aneurysm. Epidemiology Most cases (85% of cases) occur in children under the age of 10 years...
Article

Friedreich ataxia

Friedreich ataxia is the most common hereditary progressive ataxia. Epidemiology Thought to have an estimated prevalence of ~1:50,000. There is no recognized gender predilection. Typically present in childhood to adolescence 11. Those with a higher number of trinucleotide repeats (>500) are t...
Case

Normal pressure hydrocephalus

  Diagnosis certain
Andrei Tsoriev
Published 13 Feb 2015
95% complete
Annotated image MRI
Case

Neurosyphilis

  Diagnosis almost certain
Damien Charles
Published 18 Mar 2024
77% complete
MRI
Case

Intracranial hypotension: iatrogenic

  Diagnosis certain
Daniel Chow
Published 14 Feb 2016
100% complete
MRI CT

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