Tongue immobile and cannot be prolruded Emotional lability Spontaneous outbursts of laughing and crying Primary lateral sclerosis

immobile and cannot be prolruded Emotional lability Spontaneous outbursts of laughing and crying Primary lateral sclerosis - refers to slowly progressive spasticity and weakness of the limbs, more marked in the legs, which may be associated with pseudobulbar paley It has a better prognosis Investigations - No specific test Investigations are performed to exclude other condtions -fa) MR I or myelogram of sp.cord -lo exclude compressive lesion in neck Cranial MRI in patients with buibar onset to exclude brain stem lesions. On T2-welghted Images, high Intensity lesions are visible in motor cortex, internal capsule and brain stem (a) EMG and nerve conduction studies - reveal anlerior horn cell damage but do not specify the cause Creaiine phosphokinase may be slighthy raised (c) CSF is normal TREATMENT - Is symptomatic Other Clinical paiiems o! MND - 1. Familial MND - Chromosome 21 2 Madras MND - Non-farrillalp onset 10-30 years, slow progression Often asymmetric - deafness, buibar or other cranial n involvement 3 MND with demenlia - Amyotrophic Oakob-CreutzfeJdi syndrome. MND with Alzheimer's disease, familial MND with demenlia. Related disorders I KENNEDY'S SYNDROME (Buibar and spinal muscular airophy) - A rare of MND with near normal life expectancy Presents in males between ages of 20 and 50 Features. Mild oysarthria, wasied fasciculatlng tongue, tremor, proximal weakness with wasting and fasclculations Spinal muscular airophy 2 MULTIFOCAL MOTOR NEUROPATHY (MMN) - occurs in men under 45. with asymmetrical, progressive, mainly distal weakness with onset in the arms Signs include fasclculalions, wasting and reduced reliexes EMC shows multlfocal conduction block. High serum titres of antibodies lo GM1 ganglioside alien detectable May respond to treatment with iv. human immunoglobulln, plasmaphsresis or cyclophosphamide. 3 SPINAL MUSCULAR ATROPHIES (SMAs) - Diseases that usually present in infancy or chllftiood and allect only lower motor neurones (a) Type I SMA (Werding-Hoffman disease) presents al birth or in early inlancy with hypotonia, reduced movements, proximal weakness, wasting