White Matter Disorders : Introduction White Matter Disorders in a patient with DM White Matter Disorders in HIV patient with low CD4 counts White Matter Disorder : Introduction Brain consists of
Pathophysiology: Any damage to the white matter can cause white matter disorders
Diagnosis: White matter disorder is a radiological diagnosis, and includes many diseases, and presentation. Few examples are discussed below. Case Based Learning 51 yo F with DM, HTN, HLD presented for AMS. Details of history not known. MRI shows the following. Unclear of diagnosis of MS. LP is done. No inflammatory cells, or oligoclonal band is seen. Working diagnosis is microvascular disease. CPK, Lactic Acid is elevated on admission. HIV is negative. B12 level is normal. MMA and Homocystein is not checked. What is the DDx.      Further work up reveals absence of proteinuria, and retinopathy. ANA, RPR, MMA and Homocysteine, Discussion: MS: including the relatively rapid onset of symptoms, a relapsing–remitting course, fatigue, depression, HA, ocular symptoms, autonomic dysfunction, evidence of brainstem or cerebellar symptoms (including slurring of speech), seizure-like activity. Higher cortical features may be spared early in the disease, but may be affected later in the days; female predominance; On MRI, lesions primarily affecting the white matter of the brain and spinal cord are found; corpus callosum is commonly affected; lesions may or may not enhance with contrast; CSF is normal mostly except for mild elevation of protein, Oligoclonal Bands, and leucocytes, mostly Monocytes; Visual or auditory evoked potential; Other Demylinating disease B12 Deficiency SLE Lyme Disease: Affects CNS in 15 % of the time; this diagnosis should always be considered if white mater lesions are present; B/L CN VII is the key feature; Lymphocytic meningitis can occur; painful radiculitis can occur; rarely focal encephalomyelitis or myelopathy can occur. HIV: progressive apathy, depression, psychomotor retardation, and memory loss can occur; There is often focal subcortical involvement, with infection of macrophages in the subcortical white matter and the basal ganglia. Maligancy: glioma (Gliomas can in- filtrate the corpus callosum) or a lymphoma (Lymphoma of the central nervous system tend to affect the deep structures, including the corpus callosum) MELAS: typically begin before 40 yr but can occur after that; encephalomyopathy, seizures, and dementia often occur with headaches and vomiting; DM is frequently seen; Stroke affect cortical and sub-cortical white matter, but do not confer the vascular area;  Migraine: White-matter abnormalities called “unidentified bright objects” are associated with migraine; The number of white-matter abnormalities in- creases with an increasing frequency of migraines; Cerebral Vasculopathies Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy: Subcortical ischemic strokes, particularly in the anterior temporal lobes, begin in the fifth or sixth decade, often in the absence of traditional vascular risk factors; Psychiatric symptoms, dementia, and migraine headaches are common; MRI shows multiple subcortical infarcts, with diffuse leukoencephalopathy; eiosinophilic material accumulates between the smooth-muscle cells Cerebral Vasculitis: Susac’s syndrome, also known as retinoco- chleocerebral vasculopathy, is a microangiopathy that affects the brain, retinal arteries, and cochlea, causing headaches, personality changes, dementia, bilateral branch retinal artery occlusions, and deaf- ness. It occurs predominantly in women 20 to 40 years of age and is thought to be immune-medi- ated. Chronic lesions, described as “snowball le- sions,” can be confluent and can affect large areas of the corpus callosum; Patients with primary angiitis of the central nervous system have an elevated erythrocyte sedimenta- tion rate, a characteristic angiographic appearance, and evidence of inflammation in the cerebrospinal fluid; Binswanger’s disease, or subcortical leukoencephalopathy : progressive subcortical white-matter disease associated with progressive cognitive and motor decline; 4th - 7th decade; HTN; small discrete infarcts and larger areas of diffuse and incomplete infarction and demyelination with thickening and hyalinization of arterioles Lacunar infarcts are deep white-matter infarcts, typically less than 15 mm in diameter, that are due to occlusion of a penetrating artery; The occlusion may be caused by multiple processes, most commonly hypertension, diabetes, advanced age, ischemic heart disease, and cigarette smoking; White Matter Disorders in HIV with low CD4 counts Clinical Question 26 yo AAM with 1 mth history of worsening of mental status. HIV of ??? duration. MRI shows global volume loss with white matter disease. LP is normal. CD 4 count is 7. Viral load is very high. DDx. of white matter diseases in a HIV patient includes the following DDx of white matter disease in this patient (with low CD4 counts) includes the following Delirium
HIV-associated Dementia
Infection and Malignancy in HIV host
Metabolic
Treatment
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