Understanding neuromuscular disorders.

coming soon.......

Primary Progressive Aphasia

Primary progressive aphasia (PPA) involves changes in the ability to communicate—to use language to speak, read, write, and understand what others are saying. Problems with memory, reasoning, and judgment are not apparent at first but can develop over time. In addition, some people with PPA may experience significant behavioral changes, similar to those seen in bvFTD, as the disease progresses. As symptoms get worse, people with PPA cannot live alone safely.

Currently, there are three types of PPA, categorized by the kind of language problems seen at first.

In semantic PPA, also called semantic dementia, a person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

In agrammatic PPA, also called progressive nonfluent aphasia, a person has trouble saying words that link nouns and verbs together—for example, "of," "from," and "for." Eventually, the person may no longer be able to speak at all. He or she may also have difficulty swallowing and develop movement symptoms similar to those seen in corticobasal syndrome.

In logopenic PPA, a person has trouble finding the right words during conversation but can understand words and sentences. The person does not have problems with grammar.

Behavioral Problems in Dementia.

This is a good paper which discusses behavioral problems related with dementia and their management.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC181170/

Clinical Manifestations of the Serotonin Syndrome

Cognitive and behavioral features:
confusion/disorientation, agitation/irritability, coma/unresponsiveness, anxiety,
hallucinations (visual and auditory)
Autonomic features:
hyperthermia, diaphoresis, sinus tachycardia, hypertension, dilated pupils, nausea,
flushing
Neuromuscular:
myoclonus (legs especially), hyperreflexia, muscle rigidity, restlessness/hyperactivity,
tremor, ataxia, extensor plantar responses

Clinical Features of Neuroleptic Malignant Syndrome

Administration of neuroleptics (especially high-potency conventional agents)
Hyperthermia >38.0C
Muscle rigidity (may have cogwheel rigidity)
Tremor, myoclonus
Stupor, mutism, delirium
Autonomic instability – tachycardia, labile BP
Tachypnea, dyspnea
Sweating, sialorrhea, incontinent
Dysarthria, dysphagia
Raised CPK & WCC, metabolic acidosis, hypoxia, low serum iron, raised
catecholamines, theta or delta waves on EEG
Complications: rhabdomyolysis, ARF, aspiration pneumonia, pulmonary embolus,
cardiac arrest, DIC, limb contractures, ischemic brain injury
Exclude other central or systemic or toxic causes of hyperthermia

Mitochondrial cytopathy

Mitochondrial mutations - Mitochondrial DNA acquire mutations about 6-7 times the rate of nuclear DNA.

Reasons-
1. Lacks protective Histones
2. Close proximity to electron transport chain(exposure to high conc of free radicals)
3. Lacks DNA repair mechanism

(Ref : Cohen and Gold )

Aphasia - Disorder of language

Aphasia is not simply a sensory or motor deficit. Language has 6 broad components. Fluency,Naming,Comprehension,Repetition,Reading and Writing. Fluency,comprehension and repetition has some localizing values.

1. Broca`s aphasia - fluency and repetition impaired. Comprehension intact. Of note, broca`s aphasia can have some impaired comprehension, particularly syntactically dependent sentences.For eg ." The lion was killed by the tiger" . They often incorrectly choose tiger as the dead animal. Prosody is also lost.

2.Wernicke`s aphasia - Compehension and repetiton impaired. Fluency intact.Lots of paraphasic errors. Neologism.

In Broca`s aphasia the patient is frustated, in Wernicke`s aphasia the examiner is frustated.


3.Conduction aphasia - Repetition only impaired. Comprehension and fluency intact.

4. Transcortical aphasia - resembles Broca`s, Wernicke`s but repetition is spared. Seen in watershed infarcts. Can be subdivided into Transcortical sensory aphasia if comprehension is impaired or Transcortical motor if fluency is impaired but comprehension is intact.

5. Global aphasia - Repetition,comphresion and fluency impaired.Seen in large Left MCA infarct.

Ref : Neuroanatomy - Hal Blumenfeld